136 - American Neurological Association

136th Annual Meeting of the 

American Neurological Association 

September 25–27, 2011 

Manchester Grand Hyatt, San Diego, CA 

Poster Listings 

Derek Denny-Brown New Member 

Symposium Abstracts 1–3 

Poster Session Abstracts 4–18 

Works in Progress Abstracts 19–21 

Career Development Abstracts 22 

Author Index 112–123 

Subject Index 124–126

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Poster Listings 

Sunday, September 25, 2011 

Topic Posters Poster Listing Pages Abstract Pages 

Cerebrovascular Disease 101–144 3–5; 19 23–32; 99–100 

Movement Disorders 201–240 5–7; 19 32–41; 100–101 

Sleep Disorders and Circadian Rhythm 301–303 7 42 

Education 401–408 7–8 42–44 

Career Development 515–531 22 111 

Monday, September 26, 2011 


Behavioral Neurology 601–622 8–9; 19 44–49; 102 

Epilepsy 701–732 9–10; 19–20 49–55; 102–104 

Neuromuscular Disease 801–841 10–11; 20 55–63; 104–105 

Neurogenetics 1001–1013 11–12; 20 63–65; 105–106 

Trauma/Injury 1101–1107 12 65–66 

Neurology Critical Care 1201–1206 12; 20 66–67; 106 

Pediatric Neurology 1301–1304 12–13 67–68 

Rehabilitation and Regeneration 1401–1403 13 68–69 

Tuesday, September 27, 2011 


Dementia and Aging 1501–1542 13–15; 20 69–79; 107–108 

Headache and Pain 1601–1623 15–16; 21 79–84; 108 

Neuroimmunology and Demyelinating Disease 1701–1747 16–28; 21 84–94; 108–110 

Neurooncology 1801–1809 18 94–96 

Neurovirology 1901–1908 18 96–98 

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Poster Listings

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136 th Annual Meeting Tuesday, 

September 27, 2011 Derek 

Denny-Brown New Member 

Symposium Abstracts 

Derek Denny-Brown Neurological Scholar Award: Can 

We Detect Alzheimer’s Disease a Decade Before 

Dementia, and Why Would We Want To? 

Reisa Sperling, M.D. Center for Alzheimer Research and 

Treatment, Brigham and Women’s Hospital, Massachusetts 

General Hospital, Harvard Medical School 

The pathophysiological process of Alzheimer’s disease (AD) 

is thought to begin years, if not decades, prior to the onset 

of clinical dementia. Converging data from PET amyloid 

imaging, cerebrospinal fluid studies, and large autopsy series 

suggest that approximately one-third of clinically normal 

older individuals harbor a substantial burden of cerebral 

amyloid-b deposition. Recent multi-modality imaging studies, 

using PET amyloid imaging and functional MRI, have 

demonstrated that amyloid-b deposition in normal elderly is 

associated with aberrant fMRI activity in the default network, 

in a pattern strongly resembling that seen in AD dementia. 

AD-like patterns of atrophy have also been detected 

in amyloid-positive normal older individuals, and recent evidence 

suggests that specific patterns of cortical thinning are 

predictive of who will develop dementia a decade later. A 

small number of studies have reported an association 

between higher amyloid burden and lower memory performance 

even among the range of clinically normal older 

individuals, particularly those studies employing more challenging 

neuropsychological measures. These findings provide 

support for the hypothesis that amyloid-b accumulation is 

linked to synaptic dysfunction in the networks supporting 

memory processes, and that brain dysfunction is detectable 

prior to the emergence of significant cognitive impairment. 

It is likely, however, that many additional factors modulate 

the likelihood of subsequent clinical decline. Longitudinal 

studies are ongoing to determine if these amyloid-positive 

older individuals are indeed in the preclinical stages of AD, 

and to elucidate the endophenotype that best predicts those 

who will progress to AD dementia. In parallel with these 

natural history studies, we must begin secondary prevention 

trials to determine if altering amyloid burden can delay the 

emergence of clinical symptoms. It is likely that, similar to 

cancer, diabetes, cardiovascular and most other chronic diseases, 

many therapies for AD will be most efficacious in the 

early stages of the pathophysiological process. 

Derek Denny-Brown Neurological Scholar Award: The 

Human Sense of Smell at Millisecond Speed 

Jay A. Gottfried, MD, PhD, Northwestern University, 

Chicago, IL 

Very little is known about the basic anatomy, connectivity, 

and physiology of the human olfactory system. What we do 

know is largely inferential, derived from non-human animal 

studies that may hold scant relevance for the human sense 

of smell. Although functional imaging studies have 

expanded our general understanding of human olfactory 

neurobiology, these techniques have poor temporal resolution 

and provide only correlative information. Thus even 

the most basic assumptions about human olfaction have not 

been systematically tested. In this presentation I will discuss 

1 

new work using olfactory intracranial EEG techniques in 

patients with medically refractory epilepsy. By recording 

directly from olfactory limbic brain regions, we have begun to 

gain direct insights into the electrophysiological organization of 

the human olfactory system. Preliminary results suggest that 

the emotional content of a smell modulates piriform activity 

in the gamma-band range, and that cortical stimulation at piriform 

cortex elicits short-latency (


Results: Forty-three of 45 heterotopia (96%) showed 

functional correlation with discrete regions of overlying cortex 

(mean peak coefficient 0.61). Nodules also demonstrated 

correlation with contralateral cortex (62%), other nodules 

(51%), ipsilateral nonoverlying cortex (42%), and basal ganglia/cerebellum 

(13%). The peak degree of connectivity 

between heterotopia and other gray matter regions was significantly 

related to epilepsy duration. 

Interpretation: Nearly all heterotopia in PNH are functionally 

connected to overlying cortex, and the strength of 

aberrant connectivity increases with duration of epilepsy. 

Along with prior evidence that cortico-cortical tract defects 

underlie dyslexia in this disorder, these results suggest that 

altered connections are a critical substrate for neurological 

dysfunction in brain malformations. 

Study supported by: NIH/NINDS, Epilepsy Foundation, 

William F. Milton Fund 

M810. Evaluating Mechanoreceptors in Glabrous Skin 

in Diabetic Neuropathy 

Iliza Myers, Kay Artibee, Jun Li and Amanda C. Peltier; 

Nashville, TN 

Meissner corpuscles (MCs) and their myelinated afferents 

are found only in glabrous skin. We hypothesized that alterations 

in MCs occur in diabetic neuropathy and correlate 

with other measures of axonal loss. Immunohistochemistry 

was performed on 2 and 3 mm skin punches from the index 

finger and distal leg, respectively. Four diabetic patients 

(ages 45–75), with neuropathy confirmed by exam and 

nerve conduction studies and four control patients (aged 

35–50), were studied. Average Meissner corpuscle density 

(MCD) in control patients was 15.3 6 7.1 MCs/ mm 2 with 

significant reductions observed in two patients (3.3, 6.2 MCs/ 

mm 2 ) with near normal or higher density in two other 

patients (11.1, 28.1 MCs/mm 2 ), suggestive of proliferation. 

MCs in diabetics often displayed abnormal morphology. 

Density of intrapapillary myelinated endings (IME) correlated 

with MCD in all patients (r ¼ 0.97). Diabetic 

patients’ intraepidermal nerve fiber density (IENFD) in the 

distal leg was 4.2 6 5.9 fibers/mm, compared to 13.7 6 

1.3 in controls. IME density did not correlate with IENFD, 

suggesting that these populations of fibers are independently 

affected by hyperglycemia. Glabrous skin biopsies afford 

evaluation of mechanoreceptors which are important in 

studying pathophysiology of diabetic neuropathy. 

Study supported by: Supported by NINDS K23 

NS056009 (A.C. P.), NINDS R01NS066927-01(J.L.), Vanderbilt 

CTSA grant 1 UL1 RR024975. 

T1501. Amyloid-Beta Dynamics and Prevention Trials 

in Dominantly Inherited Alzheimer’s Disease 

Randall J. Bateman and on behalf of the Dominantly 

Inherited Alzheimer Network; St. Louis, MO 

Amyloid-beta is a key pathologic protein in Alzheimer’s disease(AD) 

and extensive research has started an era of clinical 

trials targeting amyloid-beta. Understanding the dynamics 

of amyloid-beta formation and clearance in the human 

CNS and the processes that lead to clinical disease are essential 

to design better clinical trials. Developing efforts to prevent 

AD in autosomal dominant mutation carriers may test 

the amyloid hypothesis, determine the timing of treatment, 

and lead the way to AD prevention. 

Amyloid-beta production and clearance rates were measured 

with stable isotope labeling kinetics. The Dominantly 

Inherited Alzheimer’s Network interim findings of clinical, 

cognitive, MRI, PET, CSF, and blood biomarkers were analyzed 

with respect to the expected age of onset. 

2 

Amyloid-beta clearance rate is decreased by 30% in AD 

compared to controls. Changes in clinical, cognitive, MRI, 

PET, CSF, and blood biomarkers in autosomal dominant 

AD indicate the pathophysiologic cascade begins up to 20 

years before the expected age of onset. 

The pathophysiologic changes of AD can be specifically 

measured and targeted for clinical trials. Autosomal dominant 

AD prevention trials offer a unique opportunity to 

lead the way to effective treatments for all AD. 

Study supported by: NIH K-23-AG03094601, NIH R-01- 

NS065667, NIH 3P-01-AG02627603S1 (FACS), NIH 1U- 

01-AG03243801 (DIAN), ADRC (P50 AG05681-22), 

HASD (P01 AG03991-22), WU CTSA award (UL1 

RR024992), Mass Spectrometry Resource (NIH RR000954), 

Eli Lilly research collaboration 

R.J.B. is a co-founder of a company (C2N Diagnostics) 

that has licensed a Washington University patent on some 

of the technology described in this abstract. 

T1505. Components of Blood Pressure and Progression 

of Cerebral Leukoaraiosis: The ARIC Study 

Rebecca F. Gottesman, Diane J. Catellier, Laura H. Coker, 

Josef Coresh, Clifford R. Jack, Jr., David S. Knopman, 

Kathryn M. Rose, A. Richey Sharrett, Dean K. Shibata and 

Thomas H. Mosley; Baltimore, MD; Chapel Hill, NC; 

Winston-Salem, NC; Rochester, MN; Durham, NC; Seattle, 

WA and Jackson, MS 

Background The contribution of blood pressure (BP) components 

to the burden and progression of cerebral white 

matter hyperintensity (WMH) is poorly understood. We 

evaluated these associations in the population-based Atherosclerosis 

Risk in Communities (ARIC) cohort. 

Methods 983 participants each underwent 2 brain MRIs 

10 years apart. Systolic (SBP) and diastolic BP (DBP) were 

measured at 4 study visits. Four BP components were examined 

as predictors of WMH progression: 1) mean arterial 

pressure (MAP); 2) pulse pressure (PP); 3) orthostatic hypotension 

(OH); and 4) 10-year change in BP. 

Results Baseline (preceding MRI #1) MAP value predicted 

WMH progression (OR 1.39 (1.20–1.62), per 10 

mm Hg increase), but PP did not. Presence of OH did not 

predict WMH progression, but OH severity did (OR 1.21 

(1.02–1.42) per 10 mm Hg SBP decrease). Change in DBP 

over 10 years had a U-shaped relationship with WMH progression: 

extreme increases and decreases, independent of 

antihypertensive use, were both associated with greater 

WMH progression (p¼0.007). 

Discussion WMH progression is significantly predicted 

by MAP and extent of orthostatic SBP reduction. Significant 

changes over time in DBP, whether positive or negative, predict 

WMH progression, cautioning against simplified interpretations 

of blood pressure associations. 

Study supported by: The Atherosclerosis Risk in Communities 

Study is carried out as a collaborative study supported by National 

Heart, Lung, and Blood Institute contracts (HHSN26820 

1100005C, HHSN268201100006C, HHSN268201100007C, 

HHSN268201100008C, HHSN268201100009C, HHSN26820 

11000010C, HHSN2682011000011C, HHSN2682011000012C). 

Works in Progress Abstracts 

M839. Virtual Demyelination in pmp22 Deficiency 

Jiasong Guo, Qing Yan, Gina Sosinsky, Mark Elisman, 

Cameron McIntyre, Lily Wang, Ueli Suter and Jun Li; 

Nashville; San Diego; Cleveland; Zurich, Swaziland and 

Nashville, TN 

Safety factor for action potential propagation in pmp22þ/ 

nerves appears impaired (Bai et al, J Neurosci 2010). The 

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present study investigates mechanisms responsible for the 

impairment. Fluorescent dyes with different molecular sizes 

were injected into sciatic nerves. After 4-hour incubation, 

sciatic nerves were teased into individual nerve fibers, and 

examined under fluorescence microscopy. Fluorescence was 

of strong intensity in about a half of paranodal tomacula of 

pmp22þ/ nerves (15 mice), but absent or minimal in the 

paranodes of wild-type nerves (11 mice). This finding suggests 

that myelin is abnormaly leaky, and may result in excessive outward 

current. Application of potassium channel blocker, 4AP, to 

reduce outward current improved the amplitude of motor 

response during nerve stimulation. Western blot and immunohistochemistry 

revealed alterations of tight junction protein assembly, 

a potential molecular mechanism for the myelin leakage. 

Conclusions: Our results show excessive leakage in 

pmp22þ/ myelin in the absence of demyelination. This 

leakage is functionally similar to demyelination. These findings 

not only reveal novel mechanism for conduction block 

but also establish new therapeutic approach for this disease. 

Study supported by: NIH 

M1012. Exome Sequencing Identifies a Rare Variant in 

the CYP27B1 Gene Associated with Multiple Sclerosis 

George C. Ebers and Sreeram V. Ramagopalan; Oxford, 

Oxfordshire, United Kingdom 

Background: Multiple sclerosis (MS) is a complex neurological 

disease. We previously described the ascertainment of 

3 

43 Canadian families with 4 or more individuals with MS. 

Genetic linkage analysis and genotyping of candidate genes 

in these families has not fully explained familial disease 

clustering although alleles of GWAS genes are 

overrepresented. 

Methods: Whole exome sequencing was performed to 

further understand heightened prevalence of MS in these 

families. 

Findings: Forty-three individuals with MS (one/family) 

were sequenced. On average over 58000 variants were identified 

in each individual. Searching for rare variants in 

known or candidate MS susceptibility genes led to identification 

of a rare loss-of- function variant in the CYP27B1 

gene. This variant in 2716 parent-affected child trios 

showed significant association to MS P¼6 10 5 . Further 

genotyping of other variants in over 11,000 individuals 

showed that rare CYP27B1 variants conferred significant 

risk of MS, Peto odds ratio ¼ 4.1 (95% confidence interval 

1.5–11.1). 

Interpretation: Causative role for CYP27B1 in MS risk 

is supported. CYP27B1 encodes the vitamin D activating 1alpha 

hydroxylase enzyme. A role for vitamin D in MS 

pathogenesis is strongly implicated. We show the utility of 

using extreme multicase families to identify rare variants. 

Study supported by: This study was supported by grant 

funding from the MS Society of the United Kingdom and 

the Scientific Foundation of the Canadian MS Society. 


136 th Annual Meeting Sunday, 

September 25, 2011 

Poster Session 

Posters will be displayed in Elizabeth A-E of the Manchester 

Grand Hyatt from 10:00 am – 7:00 pm, with 

authors present from 6:00 pm – 7:00 pm. 

NOTE: An asterisk designates a resident/fellow travel award 

winner. Two asterisks represent a medical student travel award 

winner. 

Cerebrovascular Disease 


S101 Duration of Diabetes and Ischemic Stroke Risk: 

The Northern Manhattan Study 

Julio R. Vieira*, Chirantan Banerjee, Yeseon P. Moon, 

Myunghee C. Paik, Tatjana Rundek, Ralph L. Sacco and 

Mitchell S.V. Elkind; New York, NY and Miami, FL 

S102 Functional Outcomes in CREST among Patients 

with Periprocedural Stroke and Myocardial Infarction 

Bart M. Demaerschalk, Robert J. Hye, O.W. Brown, Donald 

V. Heck, Irfan Altafullah, Jenifer H. Voeks, George Howard, 

James F. Meschia and Thomas G. Brott; Phoenix, AZ; San 

Diego, CA; Royal Oak, MI; Winston Salem, NC; Robbinsdale, 

MN; Birmingham, AL and Jacksonville, FL 

S103 Inhibition and Scavenging of Complement as 

Therapeutic Targets in the Mouse Model of Acute 

Ischemic Stroke 

Xinzhi Chen, Mark P. Mattson and Milan Basta; Baltimore, 

MD and Potomac, MD 

S104 Does ACE (rs4646994) and a ADDUCIN (rs4961) 

Gene Polymorphisms Predicts the Recurrence of 

Hypertensive Intracerebral Hemorrhage 

Usha K. Misra, Jayantee Kalita, Bindu I. Somarajan, 

Bishwanath Kumar, Moromi Das and Balraj Mittal; 

Lucknow, Uttar Pradesh, India 

S105 Risk of Intracerebral Hemorrhage in t-PA Treated 

Patients with Elevated INR 

William P. Neil, Rema Raman, Ernstrom Karin and Thomas 

M. Hemmen; San Diego, CA 

S106 Toward a Further Clinical Physiological 

Elucidation: Immediate Regression of Leukoaraiosis after 

Carotid Artery Revascularization 

and Yu-Ming Chuang; New Taipei City, Taiwan 

S107 Ischemic Stroke Exome Pilot Study 

John W. Cole, Xinyeu Liu, Luke J. Tallon, Lisa K. Sadzewicz, 

Oscar C. Stine, Nicole Dueker, Yuching Cheng, Marcella A. 

Wozniak, Barney J. Stern, James F. Mitchell, Braxton D. 

Mitchell, Steven J. Kittner and Jeffrey R. O’Connell; 

Baltimore, MD and Jacksonville, FL 

S108 Phosphodiesterase Inhibitors Modulate Human 

Brain Microvascular Endothelial Cell Barrier Properties 

and Response to Injury 

Shuo Liu, Fan Yang, Chuanhui Yu, Annlia Paganini-Hill and 

Mark Fisher; Irvine, CA 

4 

S109 Incidence of Nocturnal Blood Pressure Dipping 

during Hospital Admission and Discharge among 

African American Stroke Patients 

Lien Diep, John Kwagyan, Joseph Kurantsin-Mills, Janaki 

Kalyanam, Amy Wong, Kermit Crowder, Bonnie Davis, Leia 

Harbour, Jean Edson and Annapurni Jayam-Trouth; 

Washington, DC and Baltimore, MD 

S110 Preoperative Factors Associated with In-Hospital 

Mortality Following Minimally Invasive Hematoma 

Aspiration and Thrombolysis for Intracerebral 

Hemorrhage 

Feng Xu, Zhouping Tang, Huicong Kang, Dengji Pan, 

Suiqiang Zhu and Wei Wang; Wuhan, China 

S111 Does Incidental Micro-Hemorrhage, Detected by 

Gradient Echo Sequence MRI, Predict Hemorrhagic 

Transformation of an Ischemic Stroke? 

Konark Malhotra and Yousef M. Mohammad; Chicago, IL 

S112 Younger Patients Have Lower Quality of Life after 

Intracranial Aneurysm Diagnosis 

Nerissa Ko, Richard Hornung, Charles Moomaw, Laura 

Sauerbeck and Joseph Broderick; San Francisco, CA and 

Cincinnati, OH 

S113 Aggressive Medical Management of Primary 

Intracerebral Hemorrhage: Cost/Benefit Analysis 

Luis Cava, Diane C. Andress-Rothrock and John F. Rothrock; 

Birmingham, AL 

S114 Withdrawn. 

S115 Genetic Analysis of Strain-Specific Stroke 

Sesceptibility in Mice: How To Classify C57BL/6? 

Amy K. Guzik, Sean S. Li, Ira M. Hall, Charles R. Farber, 

Brian H. Annex and Bradford B. Worrall; Charlottesville, VA 

S116 Risk Factor Control in a Phase 3 Carotid 

Revascularization Trial 

James F. Meschia, Pierre P. Leimgruber, Vito A. Mantese, 

Carlos H. Timaran, David Chiu, Bart M. Demaerschalk, 

Mary E. Longbottom, Jenifer H. Voeks, George Howard and 

Thomas G. Brott; Jacksonville, FL; Spokane, WA; St. Louis, 

MO; Dallas, TX; Houston, TX; Phoenix, AZ and 


S117 Radiologic Analysis of Thrombolysis-Induced 

Intracerebral Hemorrhage and the Role of Early Blood 

Pressure Management 

Maxim Mokin, Tareq Kass-Hout, Omar Kass-Hout, Robert 

Zivadinov and Bijal Mehta; Buffalo, NY 

S118 Anterior Circulation Stroke Causing Dizziness or 

Vertigo: A Systematic Review 

Yun Zhou, Seung-hun Lee, Ali S. Saber Tehrani, Karen A. 

Robinson and David E. Newman-Toker; Baltimore, MD and 

Gwangju, Korea 

S119 The Presence of Intracranial Vascular Calcification 

May Protect Against Vasospasm Following Subarachnoid 

Hemorrhage 

Joya Paul, Mohamed Zghouzi, Yousef Mohammad, Shyam 

Prabhakaran, Sudeep Bhabad and Bichun Ouyang; Chicago, IL 

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S120 Stroke Knowledge: A Nation-Wide, Internet-Based 

Survey of 11,121 Inhabitants in Japan 

Hisanao Akiyama, Kanako Shimizu, Yoshiaki Tokuyama and 

Yasuhiro Hasegawa; Kawasaki, Kanagawa, Japan 

S121 Is Anti Epileptic Drug Necessary in Cortical 

Venous Thrombosis? 

Velmurugendran Cannigaiper Uthamaroyan, Kaushik Sundar, 

Meenakshisundaram Umaiorubahan and Shankar 

Venkatasubramaniam; Chennai, Tamilnadu, India 

S122 Race and Insurance Disparities in Cardiovascular 

Risk Factors Control in Patients with Acute Stroke 

Anna M. Cervantes, Jose R. Romero, Helena Lau, Feliks 

Koyfman, Aleksandra Pikula, Thanh N. Nguyen, Carlos S. 

Kase and Viken L. Babikian; Boston, MA 

S123 A Population-Based Verbal Autopsy Study of 1250 

Stroke Deaths in Bangladesh 

Farrah J. Mateen, Nurul Alam, Marco Carone and Robert E. 

Black; Baltimore, MD; Dhaka, Bangladesh and Berkeley, CA 

S124 Heparin-Induced Thrombocytopenia Associated 

with Cerebral Venous Sinus Thrombosis Following 

Postoperative Enoxaparin Administration in a 64 Year- 

Old Female 

Robert A. Fishman and Ashis Tayal; Pittsburgh, PA 

S125 Renal Failure Increases Risk for Intracranial 

Hemorrhage Following Stroke 

Elisabeth B. Marsh, Argye E. Hillis, Rafael H. Llinas and 

Rebecca F. Gottesman; Baltimore, MD 

S126 Dominant Vertebral Artery Occlusion during 

Ipsilateral Head Tilt 

In Soo Moon, Jae-Hwan Choi and Kwang-Dong Choi; Busan, 

Korea 

S127 A Novel Presentation of Mesodiencephalic 

Ischemic Stroke: Case Report and Literature Review 

Khalid S. Alqadi, Tariq Alfahad and Kathleen Burger; 

Washington, DC 

S128 Fatal Postpartum Cerebral Vasoconstriction 

Syndrome 

Jennifer E. Fugate, Eelco F.M. Wijdicks, Kelly D. Flemming 

and Alejandro A. Rabinstein; Rochester, MN 

S129 Racial Disparities in Secondary Stroke Prevention 

Practices 

Pratik Bhattacharya, Seemant Chaturvedi, Flicia Mada, Leeza 

Salowich-Palm, Sabrina Hinton, Scott Millis, Sam Watson 

and Kumar Rajamani; Detroit, MI and Lansing, MI 

S130 Predicting Ischemic Stroke Outcomes Based on 

Volume of Lesion 

Shazia Z. Choudhary, Jay-Ming Wang, Zahid Choudary, 

Michael Sloan, Harry R. Van Loveren, Karen R. Wilson, 

Morgan Wang and David A. Decker; Tampa, FL and 

Orlando, FL 

S131 Treatment Outside the NINDS Stroke Study 

Exclusion Criteria: A Case Report 

Nhu T. Bruce and Brett C. Meyer; San Diego, CA 

5 

S132 Size Matters: Predictors of Intracranial 

Hemorrhage in Stroke Patients on Anticoagulation 



S133 A Rare Presentation of Anterior and Posterior 

Spinal Arteries Ischemia during Dilaysis 

Noor Yono, Adrian Marchidann and Rabih Kashouty; 

Manhasset, NY and New York, NY 

S134 Neurosarcoidosis: A Case Presentation 

Amtul Farheen, Nancy Gadallah, Rony Dekermenjian, 

Michael Rosenberg and Sushanth Bhat; Edison, NJ 

S135 Stroke Outcomes Based on MERCI/PENUMBRA 

Intervention and Hemodynamics 

Jay-Ming Wang**, Zahid I. Choudary, Shazia Z. Choudhary, 

Michael Sloan, Harry R. Van Loveren, Karen R. Wilson and 

David A. Decker; Tampa, FL 

S136 Migraine-Related ICH – A Case Study and 

Review 

Shivani Ghoshal**, Sankalp Gokhale and Louis Caplan; 

Boston, MA 

S137 Stroke as a Complication of Tuberculous 

Meningitis 

Amtul Farheen, Sumaiya Salim, Zoha Fasih, Rony 

Dekermenjian and Sushanth Bhat; Edison, NJ 

S138 Patterns and Mechanisms of Head-Shaking 

Nystagmus in Anterior Inferior Cerebellar Artery 

Infarction 

Young Eun Huh and Ji Soo Kim; Seongnam-si, 

Gyeonggi-do, Korea 

S139 Co-Complaints Influence Odds of Stroke 

Diagnosis in ED Dizziness 

Ali S. Saber Tehrani, Yu-Hsiang Hsieh, Jonathan A. Edlow, 

Carlos A. Camargo and David E. Newman-Toker; Baltimore, 

MD and Boston, MA 

S140 Eclamptic Versus Non-Eclamptic PRES (Posterior 

Reversible Encephalopathy Syndrome): Comparison of 

Clinical Features and Response to Treatment 

Pavan Bhargava, Fazeel Siddiqui, Vivek Patel, Rodger J. Elble 

and Srikanth Vallurupalli; Springfield, IL 

Movement Disorders 

S201 Allele Specific RNAi Against Triplet Repeat 

Disease-Causing Alleles in Huntington Disease 

Hirokazu Furuya, Masaki Takahashi, Shoko Watanabe, 

Miho Murata, Ichiro Kanazawa, Keiji Wada and Hirohiko 

Hohjoh; Omuta, Fukuoka, Japan and Kodaira, Tokyo, 

Japan 

S202 Antigen-Sensitized Dendritic Cell Vaccine Against 

Human a-Synuclein: A Potential Cell-Based Therapy 

Against Parkinson’s Disease 

Chuanhai Cao, Xiaoyang Lin, Wang Li, Cai Jianfeng, Li 

Kunyu, Song Shijie and Juan Sanchez-Ramos; Tampa 



S203 Depressive Symptoms and Neurodegeneration in 

the Locus Coeruleus: The Honolulu-Asia Aging Study 

Ross Webster, Robert D. Abbott, Helen Petrovitch, Kamal H. 

Masaki, Caroline M. Tanner, Jane H. Uyehara-Lock and Lon 

R. White; Honolulu, HI; Hiroshima, Japan and Sunnyvale, 

CA 

S204 MRI in HDLS Shows a Unique Mechanism of 

Neuroaxonal Degeneration 

C. Sundal*, C. Wider, J.A. Van Gerpen, J. Lash, J.Y. 

Garbern, K.J. Schweitzer, J. Aasly, B. Goodman, B.K. 

Woodruff, C.W. Christine, M. Baker, J.E. Parisi, S. Roeber, S. 

DeArmond, R. Rademakers, D.W. Dickson, D.F. Broderick, O. 

Andersen and Z.K. Wszolek; Gothenburg, Sweden; Jacksonville, 

FL; Rochester, NY; Trondheim, Norway; Scottsdale, AZ; San 

Francisco, CA; Rochester, MN and Munchen, Germany 

S205 Appropriate Outcome Measures for Cognitive 

Trials in Huntington’s Disease 

Jody Corey-Bloom, Jody Goldstein, Shea Gluhm, Charles Van 

Liew, Stephanie Lessig, Jagan Pillai and Steven D. Edland; La 

Jolla, CA 

S206 Distinctive Neurocognitive Profiles Associated with 

Right and Left Motor Symptom Onset in Parkinson’s 

Disease 

Paul J. Eslinger, Daymond Wagner, Suman Sen, Mechelle M. 

Lewis, Guangwei Du, Michele L. Shaffer and Xuemei Huang; 

Hershey, PA 

S207 The Clinical and Pathological Features of Familial 

Parkinsonism with EIF4G1Gene Mutation 

Shinsuke Fujioka, Owen A. Ross, Rosa Rademakers, Matthew 

J. Farrer, Dennis W. Dickson and Zbigniew K. Wszolek; 

Jacksonville, FL and Vancouver, BC, Canada 

S208 Alcohol Consumption and Risk of Parkinson 

Disease 

Rui Liu, Yikyung Park, Xuemei Huang, Xuguang Guo, Albert 

Hollenbeck, Aaron Blair and Honglei Chen; Research Triangle 

Park, NC; Rockville, MD; Hershey, PA and Washington, DC 

S209 Hereditary Diffuse Leukoencephalopathy with 

Spheroids: An Under-Diagnosed Disease 

C. Sundal, C. Wider, J.A. Van Gerpen, J. Lash, J.Y. Garbern, 

E.A. Shuster, K.J. Schweitzer, J. Aasly, B. Goodman, B.K. 

Woodruff, W. Kupsky, A. Tselis, C.W. Christine, M. Baker, 

J.E. Parisi, S. Roeber, S. DeArmond, R. Rademakers, D.W. 

Dickson, O. Andersen and Z.K. Wszolek; Gothenburg, Sweden; 

Jacksonville, FL; Rochester, NY; Trondheim, Norway; 

Scottsdale, AZ; Detroit, MI; San Francisco, CA; Rochester, 

MN and Munchen, Germany 

S210 Co-Existing HTT and ATXN8OS Repeat 

Expansions and a ‘Face of the Giant Panda’ Sign on 

MRI in a Patient with a Complex Movement Disorder 

Shin C. Beh, Cherian A. Karunapuzha and Shilpa Chitnis; 

Dallas, TX 

S211 Clinical Differences among PD-MCI Subtypes 

Jennifer G. Goldman, Glenn T. Stebbins, Bryan Bernard and 

Christopher G. Goetz; Chicago, IL 

6 

S212 Loss of Cortical Gray Matter in Parkinson’s 

Disease 

Suman Sen, Paul J. Eslinger, Daymond Wagner, Guangwei 

Du, Mechelle M. Lewis, Michel L. Shaffer and Xuemei 

Huang; Hershey, PA 

S213 Association of PSP with Chemical Occupational 

Exposure Factors 

Irene Litvan, Christopher R. Cunningham, Peter Lees, Leila 

Jackson, Jorge Juncos, David Riley, Yvette Bordelon, David 

Standaert, Stephen Reich, Alex C. Cambon, Joseph Jankovic, 

Deborah A. Hall, Richard Dubinsky, Cassandra Shepherd, 

Claire Henchcliffe, Ryan Uitti, Benzi Kluger, David Shprecher, 

Connie Marras, Eliza Gallin, James Leverenz and Shesh N. 

Rai; Louisville, KY; Baltimore, MD; Cleveland, OH; Atlanta, 

GA; Los Angeles, CA; Birmingham, AL; Houston, TX; Chicago, 

IL; Kansas City, KS; New York, NY; Jacksonville, Fl; Boulder, 

CO; Salt Lake City, UT; Toronto, Canada and Seattle, WA 

S214 Psychiatric Co-Morbidities and Mortality among 

Hospitalized Parkinson Disease Patients 

Nicte Mejia and Zeina Chemali; Boston, MA 

S215 Comparison of Subthalamic (STN) and Pallidal 

(GPi) Deep Brain Stimulation (DBS) on Gait and 

Balance in Patients with Parkinson’s Disease (PD) 

Jyhgong Gabriel Hou, Minn Thant, Aliya Sarwar, Linda 

Fincher, Monthaporn S. Bryant, Farrah Atassi and Eugene C. 

Lai; Houston, TX and Galveston, TX 

S216 Reliability of Self-Reported Parkinson’s Disease 

(PD) Features 

Caroline M. Tanner, Cheryl Meng, Ira Shoulson, Anthony E. 

Lang, David Oakes, Roger Kurlan, Alberto Ascherio, Flint Beal, 

Emily Flagg, Jennifer Harman, Michael Schwarzchild, James 

Beck, Bernard Ravina, Kenneth Marek, Shirley Eberly, Karen 

Marder and Robin Elliott; Sunnyvale, CA; Washington, DC; 

Toronto, ON, Canada; Rochester, NJ; Summit, NJ; Boston, 

MA; New York, NY; Cambridge, MA and New Haven, CT 

S217 First Neurological Examination Can Predict 

Course of Parkinson’s Disease (PD) 

Ali H. Rajput, Michele L. Rajput and Alex H. Rajput; 

Saskatoon, SK, Canada 

S218 Difficulty with Balance Occurs Early in PD: It 

Isn’t Appreciated Because It’s Not Asked about Nor 

Tested For 

Abraham N. Lieberman, Samea Husain, Naomi Salins and 

Anthony Santiago; Phoenix, AZ 

S219 Poor Dementia Screening with Mattis Dementia 

Rating Scale Cutoffs in Highly Educated Parkinson’s 

Disease Patients 

Travis H. Turner and Vanessa Hinson; Charleston, SC 

S220 Impaired Social Problem-Solving in Huntington’s 

Disease 

Charles Van Liew, Jody Goldstein, Shea Gluhm, Jagan Pillai 

and Jody Corey-Bloom; San Diego, CA 

S221 Association of Psychological Symptoms with 

Impulse-Control Behaviors after Dopamine Agonist 

Therapy for Parkinson’s Disease: A Longitudinal Study 



Jennifer S. Hui, Steven Cen, Megan Gomez and Lauice Yang; 

Los Angeles, CA and Pasadena, CA 

S222 Balance Difficulty in PD Correlates with Step 

Length and Velocity 

Abraham Lieberman, Naomi Salins, Tiki Hussain, Di Pan 

and Anthony Santiago; Phoenix, AZ 

S223 Motor Asymmetry in SCAs 

Mitra Assadi, Bhavpreet Dham, Gazelle Zerafati, Jon Veloski 

and Paola Leone; Camden, NJ and Philadelphia, PA 

S224 Neurotoxin Injection for Treatment of Cervical 

Dystonia 

Chandler E. Gill, Anna L. Molinari, Neil D. Manus, Michael 

W. Pelster, Jason A. Cook, Wallace Title and David Charles; 

Maywood, IL; Nashville, TN; Minneapolis, MN and New 

Orleans, LA 

S225 The Association between Mediterranean-Type Diet 

Adherence and Parkinson’s Disease 

Roy N. Alcalay, Yian Gu, Helen Mehia-Santana, Lucien Cote, 

Karen S. Marder and Nicholas Scarmeas; New York, NY 

S226 Autonomic Dysfunction in Early Parkinson’s 

Disease 

Anna L. Molinari, Fenna T. Phibbs, Lily Wang, Yanna Song 

and P. David Charles; Nashville, TN 

S227 Balance Difficulty in PD Correlates with the BNI 

Balance Scale 



S228 STEADY-PD. Safety and Tolerability of Isradipine 

CR in Early Parkinson’s Disease. Interim Tolerability 

Data Analysis 

Tanya Simuni, Kevin Biglan, David Oakes, Cheryl Deeley, 

Irenita Gardiner, Parkinson Study Group and STEADY PD 

Investigators; Chicago, IL and Rochester, NY 

S229 Nigella sativa Oil Controls Astrogliosis and 

Reduces Haloperidol-Induced Deficit in Rats 

Tafheem Malik**, Darakhshan Jabeen Haleem, Shema Husan, 

Shahid Pervez and Tasneem Fatima; Karachi, Sind, Pakistan 

and Karachi, Pakistan 

S230 Cost-Benefit Assessment of Two Forms of 

Botulinum Toxin Type-A in Different Pathologies 

Humberto Juarez, Santamaria Salvador, Leticia Hernandez 

and Enrique Molina; Mexico City, Mexico, Mexico 

S231 Weight and Height Distribution in Children with 

Tourette Syndrome 

Katie Kompoliti, Glenn T. Stebbins and Christopher G. Goetz; 

Chicago, IL 

S232 Motor Deterioration after Medication Withdrawal 

in Early Parkinson’s Disease 

Chandler E. Gill**, Anna L. Molinari, Thomas L. Davis, 

Mark Bliton, Stuart G. Finder, Michael G. Tramontana, Peter 

E. Konrad and David Charles; Maywood, IL; Nashville, TN 

and Los Angeles, CA 

7 

S233 Gender Differences in the Interleukin-6 G-174C 

Polymorphism and the Risk of Parkinson’s Disease 

Marta San Luciano, Laurie J. Ozelius, Richard B.. Lipton, 

Deborah Raymond, Kaili M. Stanley, Susan B. Bressman and 

Rachel Saunders-Pullman; New York, NY and Bronx, NY 

S234 Serum Cholesterol Is Linked with Nigrostriatal 

Iron Deposition in Parkinson’s Disease 

Guangwei Du, Mechelle M. Lewis, Michele L. Shaffer, 

Honglei Chen, Richard B. Mailman and Xuemei Huang; 

Hershey, PA and Research Triangle Park, NC 

S235 Balance Difficulty Differs from Gait Difficulty in PD 



S236 Adult Onset Dopamine Responsive Dystonia 

(DRD): Is There a New Gene? 

Hossein Ansari, Ludwig Gutmann and Laurie Gutmann; 

Morgantown, WV 

S237 Dystonia Induced Mechanical Stress as a Cause of 

DBS Extension Fracture and Expulsion 

Massimo Mondani, Elisa Petacchi, Roberto Eleopra, Silvia 

Molteni, Sabrina Gualdi, Miran Skrap and Andrea 

Martinuzzi; Udine, UD, Italy; Conegliano, TV, Italy and 

Sesto San Giovanni, MI, Italy 

Sleep Disorders and Circadian Rhythm 

S301 Endogenous GABA A Receptor Enhancement 

Modulates Vigilance in the Primary Hypersomnias 

David B. Rye, Kathy Parker, Lynn Marie Trotti, Paul S. 

Garcia, James C. Ritchie, Michael J. Owens, Leslie Morrow, 

Donald L. Bliwise and Andrew Jenkins; Atlanta, GA; 

Rochester, NY and Chapel Hill, NC 

S302 Frequency of Parsaomnias in Patients with Non- 

Epileptic Seizures 

Mitchell G. Miglis, Michael Boffa, Sujata Thawani, Alcibiades 

Rodriguez and Anuradha Singh; New York, NY 

S303 Endothelial Function in Patients with Obstructive 

Sleep Apnea 

Kanika Bagai, James Muldowney, Yanna Song, Lily Wang, 

Douglas E. Vaughan and Beth A. Malow; Nashville, TN and 

Chicago, IL 

Education 

S401 The Effectiveness of Education Intervention on 

Health Knowledge among Neurological Patients 

Mercedes Jacobson and Polina Pomerants; Philadelphia, PA 

S402 Teleneurology in Leading U.S. Medical Institutions 

Benjamin P. George**, Nicholas J. Scoglio, Jason I. Reminick, 

Kevin M. Biglan and E. Ray Dorsey; Rochester, NY and 

Baltimore, MD 

S403 Standardized Sign-Out Improves Communication 

Skills 

Brian D. Moseley, Jonathan H. Smith, Gloria E. Diaz- 

Medina, Mateo Paz Soldan, Meredith Wicklund, Radhika 

Dhamija, Haatem Reda, Michael F. Presti and Jeffrey W. 

Britton; Rochester, MN 


S404 Improved Scores on the AAN Resident Inservice 

Training Examination (RITE) after Lecture Curriculum 

Intervention 

L. John Greenfield, Vicki Ramsey-Williams, Theresa Marshall 

and Boyd Koffmann; Toledo, OH and Little Rock, AR 

S405 Evidence Based Medicine(EBM) in the 2 nd Year 

Neurosciences Curriculum 

Michael J. Schneck and Edward Neafsey; Maywood, IL 

S406 Adapting a Teaching Hospital Inpatient Neurology 

Service to New Duty Hour Requirements: The 

Washington University Adult Neurology Experience 

Robert Bucelli and Barbara J. Snider; Saint Louis, MO 

S407 Translational Research in Neuro-AIDS and Mental 

Health 

Amanda Brown, Valerie Wojna, Bruce Shiramizu, Avindra 

Nath and Justin C. McArthur; Baltimore, MD; San Juan, PR 

and Honolulu, HI 

S408 Relationship between Medical Student Feedback 

and Grading 

and James M. Stankiewicz; Boston, MA 

136 th Annual Meeting Monday, 








winner. 

Behavioral Neurology 


M601 Behaviorally-Driven Anatomical Mapping of 

Hemispatial Neglect 

Alex R. Carter, Mark P. Mcavoy, Serguei V. Astafiev, Jennifer 

Rengachary, Daniel L.W. Pope, Abraham Z. Snyder, Kristi 

Zinn, Nick Metcalf, Gordon L. Shulman and Maurizio 

Corbetta; Saint Louis, MO 

M602 Markers of Celiac Disease and Gluten Sensitivity 

in Patients with Cerebellar Ataxia 

Caroline Tan, Peter H. Green, Khalaf O. Bushara, Donald D. 

Kasarda, Ejaz Shamim, Norman Latov, Mark Hallett and 

Armin Alaedini; New York, NY; Minneapolis, MN; Albany, 

CA and Bethesda, MD 

M603 Early Signs of Cognitive Impairment among 

Multiple Sclerosis Patients with Clinically Isolated 

Syndrome 

Theodora Panou, Vasileios Mastorodemos, Efrosyni Papadaki, 

Panagiotis G. Simos and Andreas Plaitakis; Heraklion, Greece 

and Rethymnon, Greece 

M604 Motor Chunking Is Correlated with Sensorimotor 

Cortex and Striatum Activation 

Nicholas Wymbs and Scott T. Grafton; Santa Barbara, CA 

8 

M605 Areas of Ischemia Associated with ‘‘Frontal Lobe’’ 

Task Failure 

Yessenia Gomez and Argye E. Hillis; Baltimore, MD 

M606 Thalamic Atrophy in Gastric Bypass Patients with 

Cognitive Complaints 

Jonathan Graff-Radford, Jennifer Whitwell, Max R. Trenerry, 

J.E. Ahlskog, Michael D. Jensen, Clifford R. Jack, Jr. and 

Keith A. Josephs; Rochester, MN 

M607 Parietal Lobe Lesions Affect the Generation of 

Antisaccades 

James A. Sharpe, Ping Cheng and Moshe Eizenamn; Toronto, 

ON, Canada 

M608 Cognitive Ability Correlates of Psychiatric and 

Social Behaviors in Williams Syndrome 

Rowena Ng, Anna Järvinen-Pasley and Ursula Bellugi; 

San Diego, CA 

M609 Human Brain Mapping at the Single Cellular 

Level: Neuronal and Area Specific Differences in Health 

and Diseases 

Ryan P. Moore, Irisa Mahaparn, Eliezer Masliah and 

Pavel V. Belichenko; La Jolla, CA 

M610 Cognitive and Behavioral Differences between 

ADHD Populations (Inattentive Type Versus ADHD 

Plus) Using Neuropsychological Testing and 

Self-Reported Symptoms in Diagnosed Population 

from Years 1991–2008 

Barbara C. Fisher, Danielle M. Garges and Stephany Fulda; 

Shelby Township, MI and Munich, Germany 

M611 The Use of Quetiapine in Agitated Patients 

with Acquired Brain Injury: A Case Control 

Study 

Sara Piccoli, Gabriella Paparella, Alec Vestri and Andrea 

Martinuzzi; Pieve di Soligo, TV, Italy 

M612 On-Line Lexical-Semantics in the Semantic 

Variant of Primary Progressive Aphasia 

David S. Race and Argye E. Hillis; Baltimore, MD 

M613 Differences in Time Interval Distributions 

Reveal Controlled and Automatic Contributions to 

Cued Word Retrieval 

Kyongje Sung, Erin J. Pickett, Kerry Ledoux, Tracy D. 

Vannorsdall, Mohammad Elsayed, Nia M. Billings, 

Jessica Silva, David J. Schretlen and Barry Gordon; 

Baltimore, MD 

M614 Gender Differences across the Lifespan in 

Neuropsychological Testing Performance in ADHD 

Population from the Years 1991–2008 

Barbara C. Fisher, Danielle M. Garges and Stephany Fulda; 

Shelby Township, MI and Munich, Germany 

M615 Hazard Perception in Cognitively Impaired Older 

Drivers 

Nazan Aksan, Monica Lees, John D. Lee, Shaun Vecera and 

Matthew Rizzo; Mountain View, CA; Madison, WI and Iowa 

City, IA 


M616 Differentiation of Alzheimer’s Disease and 

Depression with Standard Cognitive Measures 

Meredith C. Frederick**, Stefan Sillau, David B. Arciniegas, 

C. Alan Anderson, Katherine L. Howard and Christopher M. 

Filley; Aurora, CO and Denver, CO 

M617 Attention Deficit Hyperactivity Disorder in 

Depressed Adults 

Ildefonso Rodríguez Leyva, Rubén Haro SIlva and Ana A. 

Rentería Palomo; San Luis Potosi, San Luis Potosi, Mexico 

M618 Cognition and EEG Abnormalities in Non- 

Epileptic AD(H)D/LD Patients with and without Anti- 

Epileptic Drug/Stimulant Therapy 

Brittany M. DiVito, Heather A. Koch, Spencer A. Leblang, 

Erik C. Bakken and Drake D. Duane; Scottsdale, AZ and 

Tempe, AZ 

M619 Evolution of EEG Abnormalities in Non-Epileptic 

AD(H)D/LD Patients with and without Anti-Epileptic 

Drug/Stimulant Therapy 

Heather A. Koch, Brittany M. DiVito, Spencer A. Leblang, 

Erik C Bakken and Drake D. Duane; Scottsdale, AZ and 

Tempe, AZ 

M620 Transection of CA3 Does Not Affect Memory in 

Rats 

Mohamad Z. Koubeissi, Saifur Rashid, Gemma Casadesus, 

Joseph LaManna, Kui Xu, Hans Luders and Dominique 

Durand; Cleveland, OH 

M621 Cruetzfeldt-Jakob Disease Presenting as a Rapidly 

Progressing Dementia with Non-Convulsive Status 

Epilepticus 

Natasha Tilluckdharry, Megan McGarry and Dipak P. 

Pandya; Paterson, NJ 

Epilepsy 


M701 Controlled Cortical Impact in Adult Rats and 

Posttraumatic Seizures and Epilepsy 

Kevin M. Kelly, Elena A. Kharlamov, Eric R. Miller, Bo Lu 

and Zakaria Mtchedlishvili; Pittsburgh, PA and 

Philadelphia, PA 

M702 Serotonin 1A Receptors and Memory in Temporal 

Lobe Epilepsy 

William H. Theodore, Edythe Wiggs, Ashley Martinez, Irene 

Dustin, Omar Khan, Shmuel Appel, Patricia Reeves-Tyer and 

Susumu Sato; Bethesda 

M703 Ictal Hypoxemia Is Associated with Cardiac 

Repolarization Abnormalities 

Lisa M. Bateman, Franchette Pascual, Michael Lee, Chin- 

Shang Li and Masud Seyal; Sacramento, CA and Los Angeles, 

CA 

M704 Gray Matter Heterotopia in an Epileptic Brain 

Malformation Are Functionally Connected to Overlying 

Cortex 

Joanna A. Christodoulou, Linsey M. Walker, Stephanie N. Del 

Tufo, Tami Katzir, Susan Whitfield-Gabrieli, John D.E. 

Gabrieli and Bernard S. Chang; Cambridge, MA; Boston, MA 

and Haifa, Israel 

9 

M705 JAK/STAT Inhibition Slows the Progression of 

Temporal Lobe Epilepsy in an Animal Model 

Heidi L. Grabenstatter, Yasmin Cruz Del Angel, Marco I. 

Gonzalez, Yogendra H. Raol, Shelley J. Russek and Amy R. 

Brooks-Kayal; Aurora, CO and Boston, MA 

M706 Long-Term Changes in mGluR-Mediated Long- 

Term Depression Following a Single Episode of Early 

Life Seizures in Rats 

Paul B. Bernard, Anna Castano and Tim A. Benke; Aurora, 

CO 

M707 Heterozygous Loss of the Epilepsy-Associated 

GABA A Receptor a1 Subunit Causes Spontaneous EEG 

Spike Discharges in Two Mouse Strains 

Fazal M. Arain and Martin J. Gallagher; Nashville, TN 

M708 Regional Network Disruption in Temporal Lobe 

Epilepsy 

Luigi Maccotta* and Edward Hogan; St. Louis, MO 

M709 Mice Deficient in SNAREs/SNARE Regulators 

Predict Kindling Phenotype 

John T. Slevin, Elena E. Matveeva, Sidney W. Whiteheart, 

Greg A. Gerhardt and Thomas C. Vanaman; Lexington, KY 

M710 Pharmacokinetic Equivalence between Immediate- 

Release and Extended-Release Topiramate 

Lawrence J. Lambrecht, Wesley M. Todd and Mark B. 

Halvorsen; Maple Grove, MN 

M711 Cingulate Epilepsy, Reporting 3 Clinical and 

Electrophysiologic Subtypes with Surgical Outcomes 

Mhd Rafeed Alkawadri, Norman K. So, Paul C. Van Ness 

and Andreas V. Alexopoulos; Cleveland, OH and Dallas, TX 

M712 GABAB Receptor Antagonists Reduce Pro- 

Epileptic Activity in Hippocampal Slices of Ts65Dn 

Mice, a Genetic Model of Down Syndrome 

Alexander M. Kleschevnikov, Brett Rasmuss, Pavel V. 

Belichenko and William Mobley; La Jolla, CA 

M713 Lacosamide: Long-Term Safety and Efficacy in 

Partial-Onset Seizures 

William Rosenfeld, Nathan B. Fountain, Gintaras Kaubrys, 

Elinor Ben-Menachem, Cindy McShea, Jouko Isojarvi and 

Pamela Doty; St. Louis; Charlottesville; Vilnius, Lithuania; 

Göteborg, Sweden and Raleigh 

M714 Lacosamide: Long-Term Safety in Partial-Onset 

Seizures 

Robert F. LeRoy, Gregory Krauss, Nathan B.. Fountain, 

Deanne Dilley, O’Neill D’Cruz and Pamela Doty; Dallas, TX; 

Baltimore, MD; Charlottesville, VA and Raleigh, NC 

M715 Withdrawn. 

M716 Computational Models of Ligand-Gated Receptor 

Function Characterize Anticonvulsant Drug Actions at 

GABAergic and Glutamatergic Synapses 

and David E. Naylor; Torrance, CA 

M717 Status Epilepticus: An Independent Predictor of 

Poor Survival after Anoxic Brain Injury 


Jennie Luna, Nelly Awkar, Megan McGarry, Deepthi 

Karanam and Dipak P. Pandya; Paterson, NJ 

M718 Periodic Lateralized Epileptiform Discharges 

(PLEDs)-Rhythmic Discharges (RDs) in Anoxic 

Encephalopathy 

Sushanth Bhat, Sombabu Maganti, Eli S. Neiman, Divya 

Gupta and Sudhansu Chokroverty; Edison, NJ 

M719 Periodic Lateralizing Epileptiform Discharges 

(PLEDs) Causing Persistent Magnetic Resonance 

Imaging (MRI) Changes in Ipsilateral Thalamus 

Umang Shah, Umer Akber and Chunyang Wang; Camden, NJ 

M720 Ammoniacal Encephalopathy Presenting as 

Complex Partial Seizure-Like Episodes: A Case Series 

Darine Kassar and Stanley Iyadurai; Saint Louis, MO 

M721 Status Epilepticus as an Initial Manifestation of 

Sneddon’s Syndrome 

Anish Shah, Saurav Sen, Jennie Luna and Dipak P. Pandya; 

Paterson, NJ 

M722 Status Epilepticus Secondary to Milk Alkali 

Syndrome Induced by Hypercalcemia (Oral Antacids) 

Rabih Kashouty, Noor Yono and Mershed Al Samara; 

Manhattan, NY; Manhasset, NY and Southfield, MI 

M723 A Case of Magnesium-Responsive Paraneoplastic 

Non-Convulsive Status Epilepticus 

Robert K. Shin, Anna V. Rosenbaum and Nicholas Frost; 

Baltimore, MD 


Neuromuscular Disease 


M801 Nicotinamide Mononucleotide (NMN) Treatment 

of Diabetic Neuropathy 

Ankit Sura, Mitch Onken, Krish Chandrasekaran, Helen Chen 

and James W. Russell; Baltimore, MD 

M802 Defining Outcome Measures in Sporadic IBM for 

a Follistatin Gene Transfer Clinical Trial 

Linda P. Lowes, Lindsay N. Alfano, Laurence Viollet- 

Callendret, Xiomara Q. Rosales, Brian Kaspar, K. Reed Clark, 

Zarife Sahenk, Kevin M. Flanigan and Jerry R. Mendell; 

Columbus, OH 

M803 Tomaculous Formation in HNPP 

Jun Li, Zahara M. Jaffer, Xuebao Zhang, Qing Yan, Michael 

E. Shy, Ueli Suter, Jonathan Chernoff and Jiasong Guo; 

Nashville, TN; Philadelphia; Nashville; Detroit and Zurich, 

Swaziland 

M804 Zebrafish Models of Amyotrophic Lateral 

Sclerosis 

Stacey A. Sakowski, J. Simon Lunn, Angela S. Busta, Carey 

Backus, Sang Su Oh, James J. Dowling and Eva L. Feldman; 

Ann Arbor, MI 

M805 ALS-Like Spinal Cord Pathology in Transgenic Mice 

with a Mutation in the Valosin-Containing Protein Gene 

Hong Z. Yin, Tahseen Mozaffar, Virginia E. Kimonis and 

John H. Weiss; Irvine, CA 

10 

M806 Reevaluating Disease Progression in 

Facioscapulohumeral Dystrophy 

Jeffrey M. Statland*, William B. Martens, Kathryn R. Wagner, 

John Kissel, Shree Pandya, Michael P. McDermott and Rabi 

Tawil; Rochester, NY; Baltimore, MD and Columbus, OH 

M807 Dysferlin (DYSF) Is Absent from the Muscle-Fiber 

Sarcolemma in Various Neuromuscular Diseases, and in 

Sporadic Inclusion-Body Myositis (s-IBM) It Forms 

Cytoplasmic Inclusions Colocalizing with Amyloid-b42 

(Ab42) 

Mafalda Cacciottolo, Anna Nogalska, Carla D’Agostino, W 

King Engel and Valerie Askanas; Los Angeles, CA 

M808 Novel Demonstration of Conformationally- 

Modified Tau in Sporadic Inclusion-Body Myositis 

(s-IBM) Muscle Fibers. Possible Importance to s-IBM 

Pathogenesis 

Anna Nogalska, Carla D’Agostino, King W. Engel and Valerie 

Askanas; Los Angeles, CA 

M809 Anti-Ganglioside Antibodies Mimic CNS 

Inhibitors of Axon Regeneration 

Kazim Sheikh and Gang Zhang; Houston, TX 

M810 Evaluating Mechanoreceptors in Glabrous Skin in 

Diabetic Neuropathy 


Nashville, TN 

M811 Amiodarone Associated Myopathy. A Report of 4 

Cases 

Eoin P. Flanagan, Charles M. Harper, Erik K. St. Louis, 

Michael H. Silber, Ronald C. Petersen and Keith A. Josephs; 

Rochester, MN 

M812 Cervical Cord 1 H-Magnetic Resonance Spectroscopy 

( 1 H-MRS) in Amyotrophic Lateral Sclerosis (ALS): 

Relationship to Clinoco- Electrophysiological Dysfunction 

Ken Ikeda, Yasuhiro Yoshii, Kiyoko Murata, Riya Nagata, 

Kiyokazu Kawabe, Osamu Kano and Yasuo Iwasaki; Tokyo, Japan 

M813 Localization of FIG4 in the Nervous System 

Jiasong Guo, Qing Yan and Jun Li; Nashville, TN 

M814 High Frequency Chest Wall Oscillation 

(HFCWO) in Amyotrophic Lateral Sclerosis (ALS) 

Patients Decreases Respiratory Infections Requiring 

Antibiotics and/or Hospitalization: A Pre-Post 

Observational Study 

Benjamin R. Brooks, Velma L. Langford, Amber L. Ward, 

Nicole M. Williams, Mindy S. Nichols, Elena Bravver and 

Scott C. Lindblom; Charlotte, NC 

M815 Dominant Cardiomyopathy and Very Distal 

Myopathy with Rod, Myofibrillar and AVSF 

Myopathology 

Stanley J. Iyadurai, Chris Weihl, Bob Baloh and Alan 

Pestronk; St. Louis, MO 

M816 Adult-Onset Rod Myopathy Syndrome (AORMS): 

Sustained Benefit from IVIG Plus Rituximab 

Shalini Mahajan, King W. Engel, Valerie Askanas, 

Indermohan Luthra and Varun Gupta; Los Angeles, CA and 

Rancho Mirage, CA 



M817 Geographic Trends of ALS in Minnesota 

Eric J. Sorenson and Lisa Kronk; Rochester, MN and 

Minneapolis, MN 

M818 Clinical Features Associated with Fine Specificity 

of IgG Anti-GQ1b Antibodies 

Susumu Kusunoki, Seiko Suzuki, Masami Ueda and Motoi 

Kuwahara; Osaka-Sayama, Osaka, Japan 

M819 Amyotrophic Lateral Sclerosis [ALS] Dashboard: 

Cognitive, Behavioral, Bulbar, Respiratory, Arm, Leg 

Domain-Specific Disease Staging – Statistically 

Significant Larger Proportion of Stage 3 Behavioral, 

Bulbar, Arm and Leg, but Not Cognitive or Re 

Benjamin R. Brooks, Mohammed S. Sanjak, Elena Bravver, 

William L. Bockenek, Urvi G. Desai, Scott C. Lindblom, 

Thomas J. Paccico, Nicole M. Williams, Mindy S. Nichols, 

Amber L. Ward, Kathryn A. Wright, Mifflin O’Neill, Velma 

L. Langford, Kristy Walgren, Priscilla Russo, Anne Blythe and 

Heather Oplinger; Charlotte, NC 

M820 A New Phenotype in Neurodegeneration: 

Trigeminal Sensory Deficits Preceding Rostro-Caudal 

Progressive Motor and Sensory Neuronopathy, Chorea 

and Dementia 

Camilo Toro, Justin Y. Kwan, Tanya J. Lehky, Bryan J. 

Traynor, Catherine A. Groden, Rena A. Godfrey, Michele E. 

Nehrebecky, Wiggs A. Edythe and Gahl William; Bethesda, MD 

M821 Update on a Phase 1 Study of ISIS 333611 in 

Familial ALS Due to SOD1 Mutations 

Timothy M. Miller, Richard Smith, Swati Aggarwal, Alan 

Pestronk, William David, Jeffrey Rothstein, Ericka Simpson, 

Benjamin Brooks, Isaac Bakst, Patricia Andres, Peggy Allred, 

Katie Alexander, Kathie Bishop, C.F. Bennett and Merit 

Cudkowicz; St. Louis, MO; La Jolla, CA; Boston, MA; 

Baltimore, MD; Houston, TX; Charlotte, NC and 

Carlsbad, CA 

M822 Retrospective Analysis of a Cohort of Non 

Systemic Vasculitic Neuropathy 

Raghav Govindarajan, Jagadish B. Agadi, Anita Mahadevan 

and S.K. Shankar; Weston, FL and Bangalore, India 

M823 Optimizing a Hospital Discharge Database for 

Passive Surveillance of Guillain-Barre Syndrome (GBS) 

Christopher D. Lee and Timothy F. Jones; Nashville, TN 

M824 Gene Expression Analysis in Patients with 

Amyotrophic Lateral Sclerosis and Multifocal Motor 

Neuropathy 

Alexander Shtilbans, Soon Gang Choi, Mary E. Fowkes, Greg 

Khitrov, Mona Shahbazi, Jess Ting, Stuart C. Sealfon and 

Dale J. Lange; New York, NY 

M825 Small-Fiber Polyneuropathy (SFPN) Can Cause 

Chronic Pain and Somatic Complaints in Youth 

and Anne Louise Oaklander; Boston, MA 

M826 Median Nerve Ultrasound in Diabetic PN with 

and without Carpal Tunnel Syndrome 

Anhar Hassan, Andrea Leep Hunderford, James Watson, 

Andrea Boon and Eric Sorenson; Rochester, MN 

11 

M827 Subacute Paraneoplastic Motor Neuronopathy 

with Ri Immunoeactivity and Breast Cancer: A 

Clinicopathologically Studied Patient 

and David S. Younger; New York, NY 

M828 Magnetic Resonance Neurography Versus 

Electromyography for the Diagnosis of 

Radigulopathy 

Youssef A. Dakka, Andrew Biondo, John Corrigan, 

Shehanaz Ellik and Ximena Arcilla-Londono; 

Detroit, MI 

M829 Denervation Causes Lower Expression of Heat 

Shock Protein 27 in Regenerating Skeletal Muscle 

Takahiro Jimi, Yoshihiro Wakayama and Hajime Hara; 

Yokohama, Japan 

M830 Rapid Magnetic Stimulation Versus Conventional 

Physiotherapy in Bell’s Palsy 

Devathasan Gobinathan and Lea Dosado; Singapore, 

Singapore 

M831 Establishing a Rare Disease Center in China: The 

Periodic Paralysis Program 

Qing Ke, Benyan Luo, Ming Ming, Michael Hanna, Jacob 

Levitt, Barbara Herr and Robert C. Griggs; Hang Zhou, Zhe 

Jiang, China; Rochester, NY; London, United Kingdom and 

New York, NY 

M832 Effect of Fatigue on Pulmonary Function Studies 

in ALS Patients 

Kathleen S. Alfuth, Ericka P. Simpson and Aparajitha K. 

Verma; Houston, TX 

M833 Monomelic Amyotrophy – A Rare Case 

Presentation 

Amtul Farheen, Manpreet Multani, Aiesha Ahmed, Raji 

Grewal and Raji Grewal; Edison, NJ 

M834 Botulism: A Case Report 

Peter Struck, Amtul Farheen and Sushanth Bhat; New 

Brunswick and Edison, NJ 

M835 A Novel Pentameric Thiophene Derivative 

(p-FTAA) Strongly Highlights Clusters of Paired Helical 

Filaments Containing Phosphorylated Tau in Sporadic 

Inclusion-Body Myositis (s-IBM) Muscle Fibers 

Therése Klingstedt, Anna Nogalska, Cristiane Blechschmidt, 

Stefan Prokop, Frank L. Heppner, King W. Engel, Valerie 

Askanas and Peter K.R. Nilsson; Linköping, Sweden; Los 

Angeles and Berlin, Germany 

Neurogenetics 


M1002 Clinico-Genetic Characterization of a 

Large Italian Cohort with Primary Spastic 

Paraplegia 

Andrea Martinuzzi, Mariateresa Bassi, Grazia D’Angelo, 

Sara Bonato, Gabriella Paparella, Olimpia Musumeci, 

Mariagiovanna Rossetto, Marianna Fantin, Francesca Peruch, 

Alessia Arnoldi, Claudia Crimella, Erika Tenderini, Paolo 


Bonanni, Vanessa Casanova, Giovanni Meola, Giacomo Comi, 

Antonio Toscano and Nereo Bresolin; Conegliano, TV, 

Italy; Bosisio Parini, LC, Italy; Messina, Italy and 

Milano, Italy 

M1003 A Study of ACE and ADD1 Gene Polymorphism 

in Extra and Intracranial Atherosclerosis in Patients with 

Ischemic Stroke 

Jayantee Kalita, Usha K. Misra, Sunil Kumar, Bishwanath 

Kumar, Bindu I. Somarajan and Balraj Mittal; Lucknow, 

Uttar Pradesh, India 

M1004 Generation and Characterization of MeCP2_270 

Mutant Mice 

Chiranjeevi Bodda, Karolina Can, Liakath Ali Kifayathulla, 

Hope Yao Agbemenyah and Ashraf U. Mannan; Goettingen, 

Germany 

M1005 Identification of Epigenomic Modifications as 

Biomarkers for Amyotrophic Lateral Sclerosis 

Claudia Figueroa-Romero, Junguk Hur, Yu Hong, John S. 

Lunn, Crystal Pacut, Colin E. Delaney, Raymond Yung, 

Brian Callaghan and Eva L. Feldman; 

Ann Arbor, MI 

M1006 A Drosophila Model of Williams Syndrome 

Ralph J. Greenspan and Jenee Wagner; La Jolla, CA 

M1007 Clinical and Pathological Features of Progressive 

Supranuclear Palsy with Family History 

Shinsuke Fujioka, Avi Algom, Audrey Strongosky, Dennis W. 

Dickson and Zbigniew K. Wszolek; Jacksonville, FL 

M1008 Aberrant Methylation by Mutations of DNA 

Methyltransferase 1 Cause Peripheral and Central 

Axonal Degeneration 

Christopher J. Klein, Maria V. Botuyan, Yanhong Wu, 

Christopher J. Ward, Garth A. Nicholson, Simon Hammans, 

Hiromitch Yamanishi, Adam R. Karpf, Douglas C. Wallace, 

Mariella Simon, Cecilie Lander, Julie M. Cunningham, 

Glenn E. Smith, William J. Litchy, Benjamin Boes, 

Elizabeth J. Atkinson, Sumit Middha, P. James Dyck, 

Joseph E. Parisi, Georges Mer, David I. Smith and Peter J. 

Dyck; Rochester, MN; Sydney, Australia; Southampton, 

United Kingdom; Hyogo, Japan; Buffalo, NY; Irvine, CA; 

Herston, Australia and Indianapolis, IN 

M1009 Common and Distinct Associations of 

HLA-DRB1 and -DPB1 Alleles with Neuromyelitis 

Optica and Multiple Sclerosis in Japanese 

Satoshi Yoshimura, Noriko Isobe, Takuya Matsushita, 

Tomomi Yonekawa, Katsuhisa Masaki and Jun-ichi Kira; 

Fukuoka, Japan 

M1010 Computer Simulations of Striatal Atrophy and 

Age at Onset of Huntington’s Disease 

Steven D. Edland and Jagan Pillai; La Jolla, CA 

Trauma/Injury 


M1101 A Human Natural IgM Drives Axon Outgrowth 

Xiaohua Xu, Arthur Warrington, Brent Wright, Allan Bieber, 

Virginia Van Keulen, Larry Pease and Moses Rodriguez; 

Rochester, MN 

12 

M1102 Computed Tomography Characteristics of 

Pediatric Traumatic Brain Injury 

Korak Sarkar and Kia Shahlaie; Sacramento 

M1103 Neurological Outcome Scale for Traumatic Brain 

Injury: Predictive Validity and Sensitivity to Change 

Paolo Moretti, Stephen R. McCauley, Elisabeth A. Wilde, 

James N. Scott and Guy L. Clifton; Houston, TX and Calgary, 

AB, Canada 

M1104 Determination of Awareness in Patients with 

Severe Brain Injury Using EEG Spectral Analysis 

Andrew M. Goldfine, Jonathan D. Victor, Mary M. Conte, 

Jonathan C. Bardin and Nicholas C. Schiff; New York, NY 

and White Plains, NY 

M1105 Orthopedic Injuries in Multiple Sclerosis 

Patients: Analysis of the Incidence of Injury in This 

Vulnerable Population 

Daniel Mandell** and William Tosches; Worcester, MA 

M1106 Raising the Dead: Barriers to Therapeutic 

Hypothermia Post Cardiac Arrest 

and Mark Andrews; Palo Alto, CA 

M1107 Abnormal Oculomotor Function among Blast- 

Injured Combat Veterans 

Bruce P. Capehart, Adam Mehlenbacher, Carol Smith- 

Hammond, Dale Bass and James Burke; Durham, NC 

Neurology Critical Care 


M1202 Predictors of Outcome in Prolonged Refractory 

Status Epilepticus 

Sara Hocker, Jeffrey W. Britton, Jay Mandrekar, Eelco F.M. 

Wijdicks and Alejandro A. Rabinstein; Rochester, MN 

M1203 Refractory Status Epilepticus and Heart Damage 

– A Warning for Neurologists 

Sara Hocker, Jeffrey W. Britton, Jay Mandrekar, Eelco F.M. 

Wijdicks and Alejandro A. Rabinstein; Rochester, MN 

M1204 Safety and Feasibility of Intrathecal Nicardipine 

for Vasospasm after Subarachnoid Hemorrhage 

William D. Freeman, Sothear Luke, Christina Campbell, Dan 

Jackson and James F. Meschia; Jacksonville, FL 

M1205 Acute Bacterial Meningitis as a Possible Cause of 

Severe Dysautonomia Leading to Death 

Yadira Velazquez-Rodriguez, Umer Akbar and Evren 

Burakgazi-Dalkili; Camden, NJ 

Pediatric Neurology 

M1301 Diffusion Tensor MRI Tractography Reveals 

Altered Brainstem Fiber Connections Accompanying 

Agenesis of the Corpus Callosum 

Juebin Huang, Jian Chen, Haipeng Cai, Robert P. Friedland, 

Mohamad Z. Koubeissi, David H. Laidlaw and Alexander P. 

Auchus; Jackson, MS; Hattiesburg, MS; Louisville, KY; 

Cleveland, OH and Providence, RI 


M1302 Neurofibromatosis 1 (NF1) Vasculopathy in 

Children – An Emerging Entity 

Partha S. Ghosh, A.D. Rothner and Manikum Moodley; 

Cleveland, OH 

M1303 Macro CK-1 a Cause of Spuriously 

Elevated CK Associated with Leukoencephalopathy 

in an Infant 

and John B. Bodensteiner; Phoenix, AZ 

M1304 Pharmaceuticals in the Environment: A 

Focus on Neurological Medications 

and Ilene Ruhoy; Seattle, WA 

Rehabilitation and Regeneration 

M1401 Human Induced Pluripotent Stem 

Cell-Derived Neural Progenitor Grafting into 

Rat Hippomcapus 

Alison L. Althaus, Yu Liu, Duriel Hardy and Jack M. Parent; 

Ann Arbor, MI 

M1402 Local Molecular Manipulation and Peripheral 

Nerve Regeneration 

Douglas W. Zochodne, Kimberly J. Christie, Christine A. 

Webber, Chu Cheng and Jose A. Martinez; Calgary, AB, 

Canada and Edmonton, AB, Canada 

M1403 Very Early Gait Training after Acute Stroke; a 

Dose-Escalation Study 

Randolph S. Marshall, Ying K. Cheung, Clare Bassile, Laura 

A. Evensen, Roujie Chen, Veronica Perez, Ronald M. Lazar 

and Bernadette Boden-Albala; New York 




Posters will be displayed in Elizabeth A-E of the 

Manchester Grand Hyatt from 10:00 am – 7:00 pm, 

with authors present from 6:00 pm – 7:00 pm. 



winner. 

Dementia and Aging 


T1501 Amyloid-Beta Dynamics and Prevention Trials in 

Dominantly Inherited Alzheimer’s Disease 



T1502 Integrating Genome-Wide Association and 

Functional Validation To Understand Susceptibility for 

Alzheimer’s Pathology 

Joshua M. Shulman, Portia I. Chipendo, Lori B. Chibnik, 

Brendan T. Keenan, Dong Tran, Matthew A. Huentelman, 

Julie A. Schneider, Eric M. Reiman, Denis A. Evans, David 

A. Bennett, Mel B. Feany and Philip L. De Jager; Boston, 

MA; Chicago, IL and Phoenix, AZ 

T1503 Brain Expression Genome-Wide Association 

Study (eGWAS) and Alzheimer’s Disease 

Nilufer Ertekin-Taner, Fanggeng Zou, High Seng Chai, 

Curtis S. Younkin, Julia Crook, V Shane Pankratz, 

13 

Mariet Allen, Minerva M. Carrasquillo, Christopher N. 

Rowley, Otto Pedraza, Morad Ansari, Caroline Hayward, 

Igor Rudan, Harry Campbell, Ozren Polasek, Nicholas D. 

Hastie, Asha A. Nair, Sumit Middha, Sooraj Maharjan, 

Thuy Nguyen, Li Ma, Kimberly G. Malphrus, Ryan Palusak, 

Sarah Lincoln, Gina Bisceglio, Constantin Georgescu, 

Christopher P. Kolbert, Jin Jen, Zbigniew Wszolek, Maria 

Barcikowska, Sigrid B. Sando, Jan Aasly, Kevin Morgan, 

Clifford Jack, Ronald C. Petersen, Neill R. Graff-Radford, 

Alan Wright, Dennis W. Dickson and Steven G. Younkin; 

Jacksonville, FL; Rochester, MN; Edinburgh, United 

Kingdom; Split, Croatia; Zagreb, Croatia; Warsaw, 

Poland; Trondheim, Norway and Nottingham, 

United Kingdom 

T1504 Insulin-Like Growth Factor 1 (IGF-1) and Risk 

of Alzheimer’s Disease: The Framingham Study 

Andrew J. Westwood*, Alexa S. Baiser, Ramachandran S. 

Vasan, Tamara B. Harris, Ronenn Roubenoff, Aleksandra 

Pikula, Rhoda Au, Charles DeCarli, Philip A. Wolf and 

Sudha Seshadri; Boston, MA; Framingham, MA; Bethesda, 

MD and Sacremento, CA 

T1505 Components of Blood Pressure and Progression 








T1506 Therapeutic and Preventive Effects of a Novel 

AD Vaccine 

Carmen Vigo, Ivan Cuevas, Lucia Fernandez, Valter 

Lombardi, Richard Manivanh and Ramon Cacabelos; 

Sunnyvale, CA and Bergondo, La Coruna, Spain 

T1507 MoCA vs. MMSE: Patterns of Cognitive 

Performance across Adult Lifespan in a Non-Clinical 

Sample 

Shea Gluhm, Charles Van Liew, Jody Goldstein, Guerry Peavy, 

Mark Jacobson, Stephanie Lessig and Jody Corey-Bloom; La 

Jolla, CA and San Diego, CA 

T1508 Amyloid Imaging with Florbetapir-PET 

Correlates with Cognitive Performance in Non- 

Demented Oldest-Old 

Maria M. Corrada, Dana E. Greenia, Chris M. Clark, Carrie 

B. Peltz, Mark A. Mintun, Michael J. Pontecorvo, Abhinay D. 

Joshi and Claudia H. Kawas; Irvine, CA; Philadelphia, PA 

and St. Louis, MI 

T1509 FRET Measurements of Ab-Induced Glutamate 

Release from Astrocytes 

Sara Sanz-Blasco, Juan C. Piña-Crespo, Maria V. Talantova 

and Stuart A. Lipton; La Jolla, CA 

T1510 Is Poststroke Dementia Related to Amyloid 

Deposition and Microglia Activation? 

Wolf-Dieter Heiss, Basia Radlinska, Jean-Paul Soucy, Ralf 

Schirrmacher, Alexander Thiel and Vladimir Hachinski; 

Cologne, Germany; Montreal, QC, Canada and London, ON, 

Canada 



T1511 Cycad Methylazoxymethanol Linked to DNA 

Damage, Cancer and Neurodegeneration 

Glen E. Kisby, Rebecca C. Fry, Michael R. Lasarev, Theodor 

K. Bammler, Richard P. Beyer, Mona I. Churchwell, Daniel R. 

Doerge, Lisiane B. Meira, Valerie S. Palmer, Ana-Luisa 

Ramos-Crawford, Xuefeng Ren, Robert S. Sullivan, Terrance J. 

Kavanagh, Leona D. Samson, Helmut Zarbl and Peter S. 

Spencer; Portland, OR; Seattle, WA; Cambridge, MA; 

Jefferson, AR; Guildford, Kent, United Kingdom; Buffalo, NY; 

Piscataway, NJ and Chapel Hill, NC 

T1512 Genetic Associations between VPS10 Receptor 

Genes and Late-Onset Alzheimer’s Disease 

Christiane Reitz, Joseph Lee, Lindsay Farrer, Margaret Pericak- 

Vance, Jonathan Haines, Ekaterina Rogaeva, Peter St. George- 

Hyslop and Richard Mayeux; New York; Boston; Miami; 

Nashville and Toronto, Canada 

T1513 Cardiac Ejection Fraction, Cognitive Function 

and Leukoaraiosis in an Elderly Cohort: The 

Cardiovascular Health Study 

Rebecca F. Gottesman, Salvador Cruz-Flores, Annette 

Fitzpatrick, John Gottdiener, Traci Bartz, Richard Kronmal 

and W.T. Longstreth, Jr.; Baltimore, MD; St. Louis, MO and 

Seattle, WA 

T1514 Predicting MCI Outcome with Clinically 

Available MRI and CSF Biomarkers 

David S. Heister, James B. Brewer, Sebastian Magda, Kaj 

Blennow and Linda K. McEvoy; La Jolla, CA and Mölndal, 

Sweden 

T1515 Light and Electron Microscopic Analysis of FUS 

Immunoreactivity in 3 Variants of Tau and TDP-43 

Negative Frontotemporal Lobar Degeneration 

Keith A. Josephs, Wen-Lang Lin, Joseph E. Parisi, Neil Graff- 

Radford, Ronald C. Petersen and Dennis W. Dickson; 

Rochester, MN and Jacksonville, FL 

T1516 Effects of Once-Daily, Extended-Release 

Memantine (28 mg/day) on Cognitive Domains in 

Patients with Moderate to Severe Alzheimer’s Disease 

Michael Tocco, Suzanne Hendrix, Michael L. Miller, Vojislav 

Pejovic and Stephen M. Graham; Jersey City, NJ; Salt Lake 

City, UT and Chicago, IL 

T1517 Efficacy of Memantine by Baseline Disease 

Severity: A Pooled Post-Hoc Analysis of Trials in Mild to 

Moderate Alzheimer’s Disease 




T1518 Clinical Gait Abnormalities and Hippocampal 

Morphometry in MCI: Preliminary Results from the 

ADNI Study 

Vincent S. DeOrchis and Joe Verghese; Bronx, NY 

T1519 Topography of Cortical Thinning in 

PIB-Negative Subcortical Vascular Dementia 

Versus Alzheimer’s Disease 

Chi Hun Kim, Sang Won Seo, Sung Tae Kim, Jae-Hong Lee, 

Jae Seung Kim, Seung Jun Oh, Suk-Hui Kim, Hae Kwan 

Cheong, Jong-Min Lee, Seun Jeon and Duk L. Na; Seoul, 

Korea and Suwon, Korea 

14 

T1520 The First Nationwide Survey of Bardet-Biedl 

Syndrome in Japan 

Makito Hirano, Toshihide Yamashita, Yasushi Ikuno, Hiromi 

Iwahashi, Mitsuru Ohishi, Toshiyuki Mano, Ryu Ishihara, Ichiro 

Tanaka, Keiko Yanagihara, Yusaku Nakamura and Susumu 

Kusunoki; Sakai, Osaka, Japan; Suita, Japan; Izumi, Japan; 

Osaka, Japan; Kashihara, Japan and Osaka Sayama, Japan 

T1521 P600 Word Repetition Effect Amplitude 

Correlates with Left Hippocampal Volume 

John M. Olichney, Rawi Nanakul, Alireza K. Javan, Patrick 

E. Adams, Andrea Schneider, Andreea Seritan, Randi J. 

Hagerman, Paul J. Hagerman and Susan M. Rivera; Davis, 

CA and Sacramento, CA 

T1522 Visuospatial Construction Measures and Their 

Utility in Identifying Dementia of the Alzheimer’s Type 

Bonnie M. Scott, G. Duncan, H. Carlson, Matthew Nance, 

Michele K. York, Angela Larery, Josephine Stouter and Adriana 

M. Strutt; Houston, TX 

T1523 Responder Analysis in a Trial of Once-Daily, 

Extended-Release Memantine (28 mg) in Patients with 

Moderate to Severe Alzheimer’s Disease 

Stephen M. Graham, Suzanne Hendrix, Michael L. Miller, 

Vojislav Pejovic and Michael Tocco; Jersey City, NJ; Salt Lake 


T1524 Rates of Cognitive Decline and Alzheimer’s 

Disease (AD) Neuropathology in Oldest-Old 

Archana B. Balasubramanian, Claudia H. Kawas, Daniel J. 

Berlau, Carrie B. Peltz and María M. Corrada; Irvine, CA 

T1525 Cortical Thickness on MR Imaging: Relation to 

Cognitive Reserve in Normal Aging and Mild Cognitive 

Impairment 

Jagan A. Pillai, Linda K. McEvoy, Donald J. Hagler, Jr, 

Dominic Holland, Anders M. Dale, David P. Salmon, Douglas 

Galasko and Christine Fennema-Notestine; San Diego, CA 

T1526 Effects of Once-Daily, Extended-Release 

Memantine on Individual Activities of Daily Living in 





T1527 Neurophysiologic Markers of Aging-Related 

Muscle Weakness 

Ela B. Plow, Dina Gohar, Mehmed B. Bayram, Jing Hou, 

Alexandria Wyant, Yin Fang, Vlodek Siemionow and Guang 

H. Yue; Cleveland, OH 

T1528 The Purkinje Cell of the Cerebellar Cortex in 

Alzheimer’s Disease 

and Stavros J. Baloyannis; Thessaloniki, Greece 

T1529 A Translational Program of BDNF Gene Delivery 

for Alzheimer’s Disease 

Mark H. Tuszynski, Alan Nagahara, Adrian P. Kells, J. 

Bringas, John Forsayeth and Krystopf S. Bankiewicz; La Jolla, 

CA and San Francisco, CA 


T1530 Imaging Signatures of Pathology in Behavioral 

Variant Frontotemporal Dementia 

Jennifer L. Whitwell, Clifford R. Jack, David S. Knopman, 

Bradley F. Boeve, Ronald C. Petersen, Joseph E. Parisi, Dennis 

W. Dickson and Keith A. Josephs; Rochester, MN and 

Jacksonville, FL 

T1531 Improved Statistical Power To Detect Treatment 

Effects on Functional Outcomes in Alzheimer’s Disease 

(AD) Clinical Trials by Item-Response Theory (IRT) 

M. Colin Ard, Douglas R. Galasko and Steven D. Edland; La 

Jolla, CA 

T1532 Neuropathologic Basis of Age-Associated Brain 

Atrophy 

Deniz Erten-Lyons, Randy Woltjer, Hiroko Dodge, Lisa Silbert 

and Kaye Jeffrey; Portland, OR 

T1533 A Long-Term, Open-Label Extension Study 

Evaluating the Safety of Extended-Release Memantine 

(28 mg) in Patients with Moderate to Severe Alzheimer’s 

Disease 

Stephen M. Graham and James Perhach; Jersey City, NJ 

T1534 A Retrospective Analysis Using Data-Monitoring 

Algorithms: What Are the Logical Relationships between 

the ADAS-Cog and MMSE? 

Christian Yavorsky, Guillermo DiClemente, Mark Opler, Sofija 

Jovic, Brian Rothman and Ashleigh DeFries; New York, NY 

T1535 Withdrawn. 

T1536 Case of Bimodal Charles Bonnet Syndrome and 

Dementia 

Mirret El-Hagrassy and Gokhan Akfirat; New York, NY 

T1537 Lead Exposure Up-Regulated Autophagy 

Response in Neuroblastoma SH-SY5Y Cells Via mTOR 

Kinase Signaling Pathway 

Shun-Sheng Chen, Chueh-Tan Chen and Jiin-Tsuey Cheng; 

Kaohsiung, Taiwan 

Headache and Pain 



T1602 OnbotulinumtoxinA for the Treatment of 

Chronic Migraine: Long-Term Outcome 

Hanlon T. Christopher, Silvia M. Weibelt, 

Diane C. Andress-Rothrock and John F. Rothrock; 


T1603 Utility of Orally-Inhaled Dihydroergotamine 

When Early Intervention Is Impractical 

Shashidhar Kori, Stewart Tepper, Peter J. Goadsby, 

Paul Winner, Min Wang and Stephen Silberstein; 

Mountain View; Cleveland; San Francisco; 

West Palm Beach and Philadelphia 

T1604 Characterization of Intraepidermal Nerve 

Fiber Morphology in Pain Associated with Diabetic 

Neuropathy and Impaired Glucose Tolerance 

Hsinlin T. Cheng, Jacqueline R. Dauch, Gordon A. Smith, 

Robinson J. Singleton, Brandon M. Yanik and Eva L. 

Feldman; Ann Arbor, MI and Salt Lake City, UT 

15 

T1605 Heavily T2-Weighted Magnetic Resonance 

Myelography for Post-Lumbar Puncture Headache: 

A Pilot Study 

Yen-Feng Wang, Jong-Ling Fuh, Jiing-Feng Lirng and 

Shuu-Jiun Wang; Taipei, Taiwan 

T1606 Angioplasty and Stenting for the Treatment of 

Idiopathic Intracranial Hypertension Associated with 

Dural Venous Sinus Stenosis 

Parisa P. Javedani**, Jeremy D. Fields, Kenneth C. Liu, 

Stanley L. Barnwell and Bryan Petersen; Portland, OR and 

Charlottesville, VA 

T1607 Adrenal Insufficiency Presenting as Postural 

Tachycardia Syndrome 


T1608 Prevalence of Chronic Migraine (CM), Headache- 

Related Disability and Sociodemographic Factors in the 

US Population: Results from the American Migraine 

Prevalence and Prevention (AMPP) Study 

Dawn C. Buse, Michael L. Reed, Kristina Fanning, 

Aubrey N. Manack, Catherine C. Turkel and 

Richard B. Lipton; Bronx, NY; Chapel Hill, NC and 

Irvine, CA 

T1609 Chronic Low Dose Methadone for the 

Suppression of Treatment-Refractory Chronic Migraine 

Keyvani Madjid and John F. Rothrock; San Diego, CA and 


T1610 Relationship between High Frequency Nausea 

and Treatment Satisfaction in Episodic Migraine (EM): 

Results of the American Migraine Prevalence and 

Prevention (AMPP) Study 

Richard B. Lipton, Michael L. Reed, Kristina M. Fanning and 

Dawn C. Buse; Bronx, NY and Chapel Hill, NC 

T1611 Frequent Nausea in Episodic Migraine (EM) Is 

Common and Associated with Increased Burden: Results 

from the American Migraine Prevalence and Prevention 

(AMPP) Study 

Dawn C. Buse, Michael L. Reed, Kristina M. Fanning and 

Richard B. Lipton; Bronx, NY and Chapel Hill, NC 

T1612 Medical Consultation and Headache-Impact 

among Persons with Chronic Migraine (CM) and 

Episodic Migraine (EM): Results from the American 

Migraine Prevalence and Prevention (AMPP) Study 

Aubrey N. Manack, Dawn C. Buse, Daniel Serrano, Sepideh 

F. Varon, Catherine C. Turkel and Richard B. Lipton; Irvine, 

CA; Bronx, NY and Chapel Hill, NC 

T1613 Unmet Treatment Needs among Episodic 

Migraineurs (EM): Results of the American Migraine 

Prevalence and Prevention Study (AMPP) 

Richard B. Lipton, Dawn C. Buse, Daniel Serrano, Daisy S. 

Ng-Mak, Starr H. Pearlman and Michael Reed; Bronx, NY; 

Chapel Hill, NC; West Point, PA and Savannah, GA 

T1614 Association between Triptan Use and Cardiac 

Contraindications in Migraine 

Daisy S. Ng-Mak, Valerie P. Pracilio, Stephen Silberstein, 

Joseph Couto, Cary Sennett, Mary Hopkins, Jon Bumbaugh 



and Neil Goldfarb; West Point, PA; Philadelphia, PA and 

Bowie, MD 

T1615 ‘‘Let-Down Headache’’: Reductions in Stress and 

Improvement in Mood Predict Headaches in Persons 

with Migraine 

Dawn C. Buse, Sheryl R. Haut, Charles Hall, Howard 

Tennen, Tiffani DeFreitas, Thomas M. Borkowski and 

Richard B. Lipton; Bronx, NY and Storrs, CT 

T1616 Assessing the Consistency of LEVADEX TM 

(MAP0004, Orally Inhaled Dihydroergotamine) 

Pharmacokinetic Parameters in Healthy Volunteers: 

Results from 3 Clinical Studies 

Amy Forst, Julie Iwashita, Don Kellerman, Shashidhar Kori, 

Tracy Thomas and Glyn Taylor; Mountain View; 

Merthyr Tydfil, United Kingdom and Radyr, 

United Kingdom 

T1617 Migraine Recurrence Rates with Acute Treatment: 

Case for Standardizing the Definition 

Stewart Tepper, Shashidhar Kori, Peter J. Goadsby, Michel 

Ferrari, Richard Lipton, Scott Borland, Min Wang and David 

Dodick; Cleveland; Mountain View; San Francisco; Leiden, 

Netherlands; Bronx and Scottsdale 

T1618 Transdermal Sumatriptan for Acute Treatment of 

Migraine 

and Jerome Goldstein; San Francisco, CA 

T1619 Dejerine Roussy Syndrome in a Patient with 

Sneddon’s Syndrome as an Initial Manifestation 

Jennie Luna, Anish Shah, Sourav Sen and Dipak P. Pandya; 

Paterson, NJ 

T1620 Valproate-Responsive Subclinical Rhythmic 

Electrographic Disharges (SREDA) in a Migraineur 

Umer Akbar, Bhavpreet Dham, Evren Burakgazi and John 

Kelly; Camden, NJ and Washington 

T1621 Utriculo-Ocular Counterroll Reflex Disruption in 

Skew Deviation 

James A. Sharpe, Manokaraananthan Chandrakumar, Alan 

Blakeman, Herbert C. Goltz and Agnes M. Wong; Toronto, 

ON, Canada 

T1622 Superior Semicircular Canal Dehiscence (SSCD) 

and Osteoporosis in Elderly Asian Women 

Alexander Yu, Douglas L. Teich and Eric T. Wong; Boston, 

MA 

Neuroimmunology and Demyelinating Disease 

T1701 Antibodies to Metabotropic Glutamate Receptors 

in Ophelia Syndrome and Cerebellitis 

Eric Lancaster, Eugenia Martinez-Hernandez, Maarten J. 

Titulaer, Sarah Wong, Jian Xu, Anis Contractor, Rita Balice- 

Gordon and Josep Dalmau; Philadelphia, PA and Chicago, IL 

T1702 Mortality Outcomes for Interferon Beta-1b 

Versus Placebo 21 Years Following Randomization 

Douglas S. Goodin, Anthony T. Reder, George Ebers, Gary 

Cutter, Marcelo Kremenchutzky, Joel Oger, Mark Rametta, 

Karola Beckmann and Volker Knappertz; San Francisco, CA; 

16 

Chicago, IL; Oxford, United Kingdom; Birmingham, AL; 

London, ON, Canada; Vancouver, BC, Canada; Wayne, NJ; 

Berlin, Germany and Montville, NJ 

T1703 A Recombinant Human Neuron-Binding IgM 

Protects Spinal Cord Axons and Improves Motor 

Function in a Murine Model of Multiple Sclerosis 

Aleksandar Denic*, Slobodan Macura, Arthur E. Warrington, 

Istvan Pirko, Brandon R. Grossardt, Larry R. Pease and Moses 

Rodriguez; Rochester, MN 

T1704 Neuroprotection Mediated through Estrogen 

Receptor Alpha in Astrocytes 

Rory Spence, Mary Hamby, Elizabeth Umeda, Noriko Itoh, 

Sienmi Du, Galyna Bondar, Michael Sofroniew and Rhonda 

Voskuhl; Los Angeles, CA 

T1705 Genesis of Astrogliosis in an Autoimmune Model 

of Multiple Sclerosis 

Fuzheng Guo, Yoshiko Maeda, Joyce Ma, Monica Delgado and 

David Pleasure; Sacramento, CA 

T1706 Distinct Features of Neuromyelitis Optica 

According to Anti-Aquaporin-4 Antibody IgG Subclass 

Noriko Isobe, Tomomi Yonekawa, Takuya Matsushita, Yuji 

Kawano, Katsuhisa Masaki, Satoshi Yoshimura and Jun-ichi 

Kira; Fukuoka, Japan and Matsuyama, Japan 

T1707 Effect of Fingolimod on Relapse Rate by Prior 

Treatment Status and Reason for Discontinuation: 

FREEDOMS Subgroup Analyses 

Marcelo Kremenchutzky, Paul O’Connor, Reinhard Hohlfeld, 

Ernst-Wilhelm Radue, Ludwig Kappos, Lixin Zhang-Auberson, 

Dieter A. Häring, Philipp von Rosenstiel, Xiangyi Meng and 

Augusto Grinspan; London, ON, Canada; Toronto, ON, 

Canada; Munich, Germany; Basel, Switzerland and East 

Hanover, NJ 

T1708 Effect of Fingolimod on Relapse Rate by Prior 


TRANSFORMS Subgroup Analyses 

Bhupendra O. Khatri, Jean Pelletier, Ludwig Kappos, Hans- 

Peters Hartung, Giancarlo Comi, Frederik Barkhof, Jeffrey 

Cohen, Tracy Stites, Xiangyi Meng and Augusto Grinspan; 

Milwaukee, WI; Marseille, France; Basel, Switzerland; 

Dusseldorf, Germany; San Raffaele, Milan, Italy; Amsterdam, 

Netherlands; Cleveland, OH and East Hanover, NJ 

T1709 Safety Overview of Fingolimod in Relapsing 

Multiple Sclerosis: Phase 2 and 3 Studies 

Jeffrey A. Cohen, Ludwig Kappos, Gordon Francis, William 

Collins, Lixin Zhang-Auberson and Dejun Tang; Cleveland, 

OH; Basel, Switzerland and East Hanover, NJ 

T1710 Fingolimod Mechanism of Action (MOA) in 

Multiple Sclerosis (MS) 

Jerold Chun and Jeffery A. Cohen; La Jolla, CA and 

Cleveland, OH 

T1711 Antibodies to the VGKC-Complex Proteins LGI1 

and CASPR2 in Acquired Neuromyotonia 

Camilla Buckley, Philippa Pettingill, Rosie Pettingill, Matthew 

Kiernan, Osamu Watanabe, Sarosh Irani, Patrick Waters and 

Angela Vincent; Oxford, United Kingdom; Sydney, Australia 

and Kagoshima, Japan 



T1713 Association of MS Susceptibility Variants and 

Early Attack Location 

Ellen M. Mowry, Jean Pelletier, Maria R. Blasco, Pierre Duquette, 

Pablo Villoslada, Pierre-Antoine Gourraud, Irina Malikova, 

ChristophePicard,JamieMcDonald,ElaineRoger,StacyCaillier 

and Emmanuelle Waubant; San Francisco; Marseille, France; 

Madrid, Spain; Montreal, Canada and Pamplona, Spain 

T1714 Osmotic Demyelination Syndrome: Lack of 

Association between Outcome and Severity of 

Hyponatremia 

Jennifer E. Fugate*, Jonathan Graff-Radford and Alejandro A. 

Rabinstein; Rochester, MN 

T1715 Correlation of Brain Atrophy, Disability and 

Spinal Cord Atrophy in a Murine Model of MS 

M Mateo Paz Soldan, Jeffrey D. Gamez, Aaron J. Johnson, 

Anne K. Lohrey, Yi Chen and Istvan Pirko; Rochester, MN 

and Cincinnati, OH 

T1716 Anti-NMDA-Receptor Encephalitis: Clinical 

Analysis of 457 Patients 

Maarten J. Titulaer, Eugenia Martinez-Hernandez, Lindsey 

McCracken, Rita Balice-Gordon and Josep Dalmau; 

Philadelphia, PA and Barcelona, Spain 

T1717 Cigarette Smoke Induces Inflammation and 

Oxidative Stress in Brains of Lewis Rats 

Ashwani Khanna and Walter Royal, III; Baltimore, MD 


T1719 Differential Sensitivity of Human PBMC Subsets 

to Alemtuzumab-Mediated Cytotoxicity 

William Siders, Sambasiva Rao, Juanita Campos-Rivera, Jose 

Sancho, Paula Boutin, Peter Severy, Johanne Kaplan, Bruce 

Roberts and Srinivas Shankara; Framingham, MA 

T1720 Transcriptional Profiles Uncover Population 

Structure among Multiple Sclerosis Patients 

Linda Ottoboni, David Hafler, Howard Weiner and 

Philip De Jager; Boston, MA and New Haven, CT 

T1721 Analysis of Immune Competence Following 

Alemtuzumab Treatment in huCD52transgenic Mice 

William Siders, Nathalie Chretien, Michael LaMorte, Bruce 

Roberts and Johanne Kaplan; Framingham, MA 




T1724 A Single Dose Escalation Study of the Safety, 

Pharmacokinetics, and Pharmacodynamics of 

Subcutaneous Pegylated Interferon-Beta in Healthy 

Volunteers 

Kenneth Grabstein, Aijun Wang, Natalie Winblade Nairn and 

Daniel J. Burge; Seattle, WA 

17 

T1725 Regulation of IL-12 by IL-23 in Bone Marrow 

Dendritic Cells 

Farinaz Safavi, Patricia Gonnella, Bogojub Ciric, 

Abdolmohamad Rostami and Farinaz Safavi; Philadelphia, PA 

T1726 Clinically Apparent MRI Activity Predicts 2-Year 

Outcomes in Patients with RRMS 

Thomas Scott, Xiaojun You and Pamela Foulds; Pittsburgh, 

PA and Weston, MA 

T1727 The Correlation of Brain MRI Lesion Load with 

Functional Outcome in Animal Models of MS 

Istvan Pirko, Jeffrey Gamez, Moses Rodriguez, Mihajlo 

Babovic and Slobodan I. Macura; Rochester, MN and 

Northfield, MN 

T1728 Comparison of CIDP Patients with Normal and 

Elevated CSF Protein 

YuanYuan Xue and Ericka P. Simpson; Houston, TX 

T1729 Are Anti-TAG-1 Autoantibodies Markers in 

Autoimmune Demyelinating Disorders of the PNS and 

CNS? 

Harry Alexopoulos, Pantelis P. Pavlakis, Domna Karagogeos, 

Clementine E. Karageorgiou and Marinos C. Dalakas; Athens, 

Greece and Heraklion, Greece 

T1730 Introduction and Diffusion of Multiple Sclerosis 

in the United States 

Mitchell T. Wallin and John F. Kurtzke; Washington, DC 



T1733 The Relationship between Conduction Block and 

Clinical Characteristics in Guillain-Barré Syndrome with 

Anti-GM1/GalNAc-GD1a Antibodies 

Go Ogawa, Ken-ichi Kaida, Susumu Kusunoki, 

Motoi Kuwabara, Fumihiko Kimura and Keiko Kamakura; 

Tokorozawa, Saitama, Japan and Osaka-Sayama, 

Osaka, Japan 


T1735 Randomized, Open-Label Study To Evaluate 

Patient-Reported Outcomes (PRO) with Fingolimod 

after Changing from Prior Disease-Modifying Therapy 

(DMT) for Relapsing Multiple Sclerosis (MS): EPOC 

Study Rationale and Design 

Luigi M. Barbato, Lesley Schofield, Kevin McCague, Linda 

Pestreich, Kathy Tobias and Manoj Malhotra; East Hanover, NJ 

T1736 Novel Diagnostic Tool for MS 

Nancy L. Monson, Ann J. Ligocki, William H. Rounds, 

Diane Xiang, Lindsay G. Cowell, Doug Bigwood, Eric 

Eastman, Jeffrey L. Bennett, Scott D. Boyd, Andrew Z. 

Fire, Elliot M. Frohman and Benjamin M. Greenberg; 

Dallas, TX; Gaithersburg, MD; Denver, CO and 

Palo Alto, CA 

T1737 Efficacy of Combination Therapy in Marburg 

Variant Type of Multiple Sclerosis 

Jennifer Gabbard, Yasmin Bilal and Dipak Pandya; Paterson, 

NJ 


T1738 Coma as an Inital Manifestation of Acute 

Disseminated Encephalomyelitis 

Megan McGarry, Natasha Tilluckdharry and Dipak P. 


T1739 NMO-IgG in a Patient with Neurosarcoidosis 

Lena Derani** and Elham Bayat; Washington, DC 

T1740 Correlates of Dietary Intake in Individuals with 

Multiple Sclerosis 

Matthew A. Plow, Marcia Finlayson and Chi Cho; Cleveland, 

OH and Chicago, IL 

Neurooncology 


T1801 Comparative Uptake and Cytotoxicity of Anti-Hu 

and Anti-Ri Antibodies in Rat Cerebellar Slice Cultures 

John E. Greenlee, Susan A. Clawson, Blair Wood, Kenneth E. 

Hill and Noel G. Carlson; Salt Lake City, UT 

T1802 Cerebrospinal Fluid Chemokine/Cytokine 

Biomarkers for Melanoma Brain Metastasis 

Edwin Lok, Amy S. Chung, Szexian Lee, Tamar Melman, 

Kenneth D. Swanson and Eric T. Wong; Boston, MA 

T1803 Role of p75NTR and Its Signaling Pathways in 

Fenretinide (4-hydroxyphenyl Retinamide – 4HPR) 

Induced Apoptosis in Neuroblastoma Cells 

Veena R. Ganeshan and Nina F. Schor; Rochester, NY 

T1804 Relationship between Brain MRI Imaging 

Parameters and Molecular Biomarkers as Prognostic 

Indicators in Glioblastoma Multiforme 

Xiao-Tang Kong, Senxi Du, Beverly D. Fu, Mark E. Linskey 

and Daniela Bota; Orange, CA and Irvine, CA 

T1805 Secondary Intramedullary Spinal Cord Non- 

Hodgkin’s Lymphoma 

Eoin P. Flanagan, Brian P. O’Neill, Thomas M. Habermann 

and B. Mark Keegan; Rochester, MN 

T1806 Case Report: Optic Neuropathy in a Patient with 

Glioblastoma Receiving Bevacizumab 

Robert A. Fishman, Lara Kunschner and Erik Happ; 

Pittsburgh, PA 

T1807 Anti-Ri-Associated Paraneoplastic Brainstem 

Cerebellar Syndrome with Medically-Intractable Nausea 

in a Patient with Large Cell Neuroendocrine Lung 

Carcinoma: A Case Report 

Amber N. Mitchell, Jessica Levesque and Earl Zimmerman; 

Albany, NY 

T1808 Glioblastoma Multiforme: A Rare Presentation of 

Pineal Tumor with Leptomeningeal Seeding and Future 

Directions 

Noor Yono, Tulika Ranjan and Rabih Kashouty; Manhasset, 

NY, United States Minor Outlying Islands and New York, NY 

18 

T1809 Benign-Histology Meningioma with Extracranial 

Metastasis 

Umer Akbar, Bhavpreet Dham and Melissa Carran; Camden, NJ 

Neurovirology 

T1901 HIV Associated Neurocognitive Disorder 

(HAND) Is Not Associated with Increased Fibrillar 

Amyloid Deposits Using 11 C-PiB in Middle-Aged HIVþ 

Participants 

Beau Ances, Jewell Thomas, Tammie Benzinger, Jon Christensen, 

Mengesha Teshome, Patricia Aldea, Anne Fagan, David 

Holtzman, John Morris and David Clifford; Saint Louis, MO 

T1902 Modulation of HIV-Tat Neurotoxicity by 

Potassium Channel Blockers 

Srikant Rangaraju* and Jeffrey A. Rumbaugh; Atlanta, GA 

T1903 Varicella Zoster Encephalitis – Relationship 

between Viral Load, Time and Outcome 

Benedict D. Michael, Mike Griffiths, Anna Stewart, Charlotte 

Buckley, Mark Hopkins, Ian J. Hart, Alistair Miller, Sareen Galbraith 

and Tom Solomon; Liverpool, Merseyside, United Kingdom 

T1904 Acute Varicella Zoster Virus Encephalitis in 

Adults – Relationship between Viral Load, Time, 

Clinical Features and Outcome 

Benedict D. Michael, Michael Griffiths, Anna Stewart, Charlotte 

Buckley, Mark Hopkins, Ian J. Hart, Alistair Miller, Sareen E. 

Galbraith and Tom Solomon; Liverpool, United Kingdom 

T1905 TLR4 Expression Is Upregulated in 

HIV-Associated Neurocognitive Disorder (HAND) 

Amir H. Sabouri, Gursharan Chana, Amol Shah, Critian L. 

Achim, Ronald J. Ellis, Ian P. Everall and HNRC Group; 

La Jolla, CA; Melbourne, Australia and San Diego, CA 

T1906 Revisiting Reactivation of Calcified 

Neurocysticercosis: Report of Three Recent Cases 

Sanjna M. John** and Maria del Pilar Cortes Nino; 

Kingston, ON, Canada and Montreal, QC, 

Canada 

T1907 Clinical Features at Admission in Patients with 

Meningeal Cryptococcosis in a Third Level Hospital in 

Mexico 

Jesus F. Mendoza, Gilberto Vazquez, Jeronimo Rodriguez 

and Ildefonso Rodriguez; San Luis Potosí, San Luis Potosí, 

Mexico 

T1908 The Diagnosis of Tuberculous Meningitis: 

A Current Review of the Clinical and Laboratory 

Methods 

Brian Chisulo, Shan H. Jiang and Yue S. Pan; Guangzhou, 

GZ, China 



September 25, 2011 Works in 

Progress Poster Session 

WIP posters will be displayed in Elizabeth A-E of the 



The Works in Progress category emphasizes ongoing 

clinical or basic research of an extraordinary nature, which 

warrants expediated presentation. These abstracts were 

selected based on scientific merit, timeliness, and anticipated 

interest to the membership. Key aspects of research 

must have been conducted after the regular abstract 

deadline. 


S141 Polymorphism of Ninjurin2 in Korean 

Atherothrombotic Stroke Patients 

Dae-il Chang, Sung Hyuk Heo and Hye Ok Kim; Seoul, 

Korea 

S142 NMDA Receptor Biomarker for Acute Stroke 

Kerstin Bettermann and Svetlana Dambinova; Hershey, PA 

and Kennesaw, GA 

S143 Initial Transcranial Doppler Velocity Predicts 

Development of Symptomatic Vasospasm in Aneurysmal 

Subarachnoid Hemorrhage 

Shyam Prabhakaran, Konark Malhotra, Rajeev Garg, Sayona 

John, Richard Temes and Viven Lee; Chicago, IL 

S144 Relative Change in Transcranial Doppler Velocities 

Is Inferior to Absolute Thresholds in Prediction of 

Vasospasm after Subarachnoid Hemorrhage 

Konark Malhotra, James Connors, Viven Lee and Shyam 

Prabhakaran; Chicago, IL 

Movement Disorder 


S238 Dopamine Transporter Imaging Predicts Long 

Term Outcomes in Parkinson’s Disease 

Bernard M. Ravina, Kenneth Marek, Shirley Eberly, David 

Oakes and Ira Shoulson; Rochester and New Haven 

S239 Time to First Levodopa-Induced Motor 

Complication: Results from the STRIDE-PD Study 

C. Warren Olanow, Karl Kieburtz, Olivier Rascol, 

Werner Poewe, Anthony Schapira, Helena Nissinen, 

Mika Leinonen and Fabrizio Stocchi; New York, NY; 

Rochester, NY; Toulouse, France; Innsbruck, Austria; 

London, United Kingdom; Espoo, Finland; Kista, 

Sweden and Rome, Italy 

S240 Acute Effects of Preladenant, a Selective Adenosine 

A2A Antagonist, on Dyskinesia and Parkinsonism in 

Levodopa-Treated Subjects 

Penelope Hogarth, Matthew D. Troyer, Byung S. Park, 

Igor D. Grachev, Tatanisha Laguerre, Fengjuan Xuan, Amol 

Tendolkar and John G Nutt; Portland, OR and Whitehouse 

Station, NJ 

19 







The Works in Progress category emphasizes ongoing clinical 

or basic research of an extraordinary nature, which warrants 

expediated presentation. These abstracts were selected 

based on scientific merit, timeliness, and anticipated interest 

to the membership. Key aspects of research must have been 

conducted after the regular abstract deadline. 


M622 New Approaches to Unraveling the Multi-Scale 

Neuroanatomical Changes in Williams Syndrome 

Ann Lam and Elan L. Ohayon; La Jolla 

Epilepsy 

M725 HLA-B*1502 Genotyping in Carbamazepine and 

Phenytoin Induced Stevens-Johnson Syndrome 

Sivakumar M. Rajappa and Srinivasan A. Venkatesan; 

Chennai, Tamil Nadu, India 

M726 Filamin A Regulates Neural Progenitor 

Proliferation and Brain Size through wee1-Dependent 

cdk1 Phosphorylation 

Gewei Lian and Volney Sheen; Boston, MA 

M727 A Moderate-Throughput Screen for 

Antiepileptogenic Compounds 

Yevgeny Berdichevsky, Yero Saponjian, Michelle Mail and 

Kevin J. Staley; Boston, MA 

M728 Network Structure and Sensitivity to the 

Geometry of Stimuli in Epilepsy and Cognition 

Elan L. Ohayon, Ann Lam and Terrence J. Sejnowski; La 

Jolla, CA 

M729 Hypoglycemia Induced NMDA 

Receptor-Dependent Epileptiform Activity 

in the Hippocampal CA3 Area Causes Damage 

in CA1 

Carlos M. Florez, Jane Zhang, Peter Abdemalik, 

Liang Zhang and Peter L. Carlen; Toronto, ON, Canada 

M730 Alzheimer Disease in Lafora Epilepsy 

Jesus Machado-Salas, Maria Rosa Avila-Costa, Patricia 

Guevara, Jorge Guevara, Reyna M. Duron, Dongsheng Bai, 

Miyabi Tanaka and Antonio V. Delgado-Escueta; Los Angeles, 

CA and Mexico, Mexico 

M731 Antiepileptic Activity of Intrapulmonary 

Midazolam 

Ashish Dhir, Dorota Zolkowska and Michael A. Rogawski; 

Sacramento and Davis 

ID: kannanb I Black Lining: [ON] I Time: 16:18 I Path: N:/3b2/ANA#/Vol00000/110242/APPFile/JW-ANA#110242

M732 Do Brain Volumes in JME (juvenile myoclonic 

epilepsy) differ from normal controls? 

John T. Spitz, Long Vu, Lydia Su, Mark A. Mandelkern, 

Barbara E. Swartz; Berkely, CA, Irvine, CA, Los Angeles, CA, 

Irvine,CA and Newport Beach, CA 


M836 Disrupted Expression of Myogenin in Inclusion 

Body Myositis 

Akatsuki Kubota, Jun Shimizu, Atsushi Iwata and Shoji Tsuji; 

Tokyo, Japan 

M837 Abnormalities of a Novel Autophagy-Associated 

Protein, NBR1, in Muscle Fibers of Sporadic Inclusion- 

Body Myositis (s-IBM) 

Carla D’Agostino, Anna Nogalska, Mafalda Cacciottolo, 

W.King Engel and Valerie Askanas; Los Angeles, CA 

M838 Atrophy and Autophagy in Limb Girdle Muscular 

Dystrophy and Glycogen Storage Disease Type 2 

Corrado I. Angelini, Annachiara Nascimbeni, Marina Fanin 

and Marco Sandri; Padova, Italy 

M839 Virtual Demyelination in pmp22 Deficiency 




Nashville, TN 

M840 First in Human Phase 1 Trial of Neural 

Progenitor Cells in ALS: Results in the First 12 Patients 

Jonathan D. Glass, Nicholas Boulis, Meraida Polak, Crystal 

Kelly, Thais Federici, Jane Bordeau, Seward Rutkove, Karl 

Johe, Tom Hazel and Eva Feldman; Atlanta, GA; Boston, 

MA; Rockville, MD and Ann Arbor, MI 

M841 Clinical Development of an Antisense Therapy for 

the Treatment of Transthyretin Amyloidosis 

Shuling Guo, Elizabeth Ackermann, Sheri Booten, Luis 

Alvarado andrew Siwkowski, Merrill Benson, Steve Hughes 

and Brett Monia; Carlsbad, CA and Indianapolis, IN 

Neurogenetics 


M1011 EPI-A0001: New Potential Therapy for 

Friedreich Ataxia 

David R. Lynch, Steve M. Willi, Robert B. Wilson, Karlla W. 

Brigatti, Olena Kucheruck, Eric C. Deutsch, William D. 

Shrader, Patrice Rioux, Guy Miller, Amale Hawi and Thomas 

Sciascia; Philadelphia, PA; Patterson, NY and Mountain View, 

CA 

M1012 Exome Sequencing Identifies a Rare Variant in 

the CYP27B1 Gene Associated with Multiple Sclerosis 



M1013 Whole Genome Sequencing in Twins Discordant 

for 27 Diseases 



20 

Neurology Critical Care 

M1206 Imaging Biomarkers of Cerebral Edema in 

Malignant Infarction 

Kevin N. Sheth, Albert J. Yoo, R. G. Gonzalez, W. T. 

Kimberly, Zeshan A. Chaudhry, Jordan J. Elm, Sven Jacobson, 

Stephen M. Davis, Geoffrey A. Donnan, Gregory W. Albers 

and Barney J. Stern; Baltimore, MD; Boston, MA; Charleston, 

SC; New York, NY; Carlton South, Victoria, Australia and 

Stanford, CA 














T1538 Proneurogenic Compound Reduces Synaptic 

Ab42 Oligomer Levels and Shows Cognitive Benefit in 

Alzheimer’s Mouse Model 

Sam Gandy, John W. Steele, Charles Glabe, Kai Treuner, 

Todd Albert, Carrolee Barlow, Michelle E. Ehrlich and 

Soong Ho Kim; New York, NY; Irvine, CA and 

San Diego, CA 

T1539 Alzheimer Risk Variant Clusterin (CLU) and 

Brain Function during Aging 

Madhav Thambisetty, Lori Beason-Held, Michael Kraut, 

Michael Nalls, Andrew Singleton, Luigi Ferrucci, Simon 

Lovestone and Susan Resnick; Baltimore and London, United 

Kingdom 

T1540 Association of High Density Lipoprotein to 

Alzheimer Disease 

Ramin Ebrahimi, Naser Ahmahi, Fereshteh Hajsadeghi and 

Hormoz Babaei; LA, CA 

T1541 Amyloid-b 42:40 Metabolism Is Altered in 

Autosomal Dominant Alzheimer’s Disease (ADAD) 

Rachel E. Potter, Vitaliy Ovid, Tom Kasten, Wendy Sigurdson, 

Kwasi Mawuenyega, Bruce Patterson, Don Elbert, Scot Fague, 

Sumi Chakraverty, Alison Goate, Kevin Yarasheski, John C. 

Morris, Tammie Benzinger and Randall J. Bateman; St. Louis, 

MO 

T1542 Acetylated Tau, a Novel Pathological Signature in 

Alzheimer’s Disease and Other Tauopathies 

David J. Irwin, Todd Cohen, Murray Grossman, Steven E. 

Arnold, Sharon X. Xie, Virginia M.Y. Lee and John Q. 

Trojanowski; Philadelphia, PA 




T1623 Retrospective Analysis of Major Congenital 

Malformations (MCMs) and Oral Clefts (OC) Associated 

with In-Utero Topiramate Exposure 

Mark W. Green and Arun Bhattachuria; New York, NY and 

Yardley, PA 


T1741 Comparison of MRI Techniques for Monitoring 


Manuela Vaneckova, Jan Krasensky, Tomas Kalincik, Dana 

Horakova, Eva Havrdova and Zdenek Seidl; Prague, Czech 

Republic 

T1742 Effects of Rituximab on T-Cells in MS 

Uma Vinayagasundaram, Ellen M. Mowry, Ian R. Matthews, 

Julia Marino and Emmanuelle Waubant; San Francisco, CA 

T1743 Fast Macromolecular Proton Fraction (MPF) 

Mapping in Multiple Sclerosis (MS) 

Vasily L. Yarnykh, James D. Bowen, Bart P. Keogh, Pavle 

Repovic, Angeli Mayadev, Beena Gangadharan, Daniel S. 

21 

Rizzuto, Hunter R. Underhill, Kenneth R. Maravilla and Lily 

K. Jung-Henson; Seattle, WA 

T1744 Skull Is Skull and Not Simply Another Bony 

Structure 

Tsutomu Nakada, Yuji Suzuki, Yukihiro Nakayama, Vincent 

J. Huber and Ingrid L. Kwee; Niigata, Niigata, Japan and 

Martinez, CA 

T1745 Characteristics of the Long Latency Vestibular 

Electrical Evoked Potential in Control Human Subjects 

Benn E. Smith, Michael J. Cevette, Jan Stepanek, Daniela 

Cocco, Gaurav Pradhan, Kenneth H. Brookler, Lindsay 

Wagner, Sarah Oakley, David A. Zapala and Mark A. Ross; 

Scottsdale, AZ and Jacksoville, FL 

T1746 Regulatory T Cells Play Contrasting Roles in a 

Viral Model for Multiple Sclerosis 

Nicholas E. Martinez, Fridrik Karlsson, Fumitaka Sato, 

Seiichi Omura, Alireza Minagar, Mathew B. Grisham, and 

Ikuo Tsunoda; Shreveport, LA 

T1747 Idiopathic Relapsing Conus Myelitis 

Raghav Govindarajan and Efrain Salgado; Weston, FL 



September 25, 2011 Career 

Development Poster Session 




Epilepsy 


CD515 Non-Convulsive Status Epilepticus Is Associated 

with Mortality and Worse Short-Term Outcome in 

Critically Ill Children 

Nicholas S. Abend, Alexis A. Topjian, and Dennis J. Dlugos; 


22 


CD531 TLR9 Processing in Multiple Sclerosis: A New 

Immunomodulatory Effect of Interferon-beta 

Konstantin E. Balashov, Suhayl Dhib-Jalbut, and Latt Aung; 

New Brunswick, NJ 

Neurogenetics 

CD522 Aberrant Channel Subunit Trafficking in the 

Neurodegenerative KCNC3R420H SCA13 Phenotype 

Michael F. Waters; Gainesville, FL 




Poster Session Abstracts 






winner. 



S101. Duration of Diabetes and Ischemic Stroke Risk: 

The Northern Manhattan Study 

Julio R. Vieira*, Chirantan Banerjee, Yeseon P. Moon, 

Myunghee C. Paik, Tatjana Rundek, Ralph L. Sacco and 

Mitchell S.V. Elkind; New York, NY and Miami, FL 

Background: Diabetes increases stroke risk, but whether 

time-dependent analyses improve estimates of effect, and 

whether duration is important, is less clear. We hypothesized 

that diabetes duration independently predicts ischemic 

stroke. 

Methods: Among 3,298 stroke-free participants, baseline 

diabetes and age at diagnosis were determined. Incident diabetes 

was assessed annually (median ¼ 9 years). We fit Cox 

proportional hazards models to examine associations 

between diabetes duration and ischemic stroke (IS) risk. 

Results: Mean age was 69 6 10 years (52% Hispanic, 

21% white, and 24% black); 22% were diabetic at baseline 

and 11% developed new diabetes. Diabetes at baseline was 

associated with IS (adjusted HR ¼ 2.5, 95% CI ¼ 1.9– 

3.3), and the magnitude was similar when analyzed as a 

time-varying covariate (adjusted HR ¼ 2.4, 95% CI ¼ 1.8– 

3.2). Duration of diabetes was associated with IS (adjusted 

HR ¼ 1.03 per year with diabetes, 95% CI ¼ 1.02–1.05). 

Compared to non-diabetics, those with diabetes for 0–5 

years (adjusted HR ¼ 1.7, 95% CI ¼ 1.1–2.7), 5–10 years 

(adjusted HR ¼ 1.8, 95% CI ¼ 1.1–3.0), and 10 years 

(adjusted HR ¼ 3.3, 95% CI ¼ 2.4–4.5) were at increased 

risk. There was a duration threshold effect at 10 years (p 

¼ 0.012). 

Conclusion: Duration of diabetes is independently associated 

with ischemic stroke. The risk triples in those with 

diabetes 10 years. 

Study supported by: This study was supported by NIH/ 

NINDS (#R37 29993). 

Dr. Vieira reports no disclosures. Dr. Banerjee reports no 

disclosures. Ms. Moon reports no disclosures. Dr. Paik 

reports no disclosures. Dr. Rundek reports no disclosures. 

Dr. Sacco discloses receiving research support from the 

NIH/NINDS over the last 12 months. Dr. Elkind serves as 

a consultant to Bristol-Myers Squibb and Tethys Bioscience, 

Inc.; serves on an event adjudication committee for Jarvik 

Heart; and serves on speakers’ bureaus for Boehringer-Ingelheim, 

Inc., Bristol-Myers Squibb/Sanofi Pharmaceuticals 

Partnership, and Genentech; and receives research support 

from diaDexus, Inc., Bristol-Myers Squibb/Sanofi Pharmaceuticals 

Partnership, and the NIH/NINDS [# R01 

NS050724 (PI), #NS048134 (PI), # P50 NS049060 (Project 

PI), # R37 NS029993 (Co-PI), #R01 NS55809 (Co-I) 

and #R01 NS062820 (Co-I)]; and has given expert testimony 

on behalf of Novartis (Zelnorm VR 

and stroke litigation) 

and GlaxoSmithKline (Avandia VR 

and stroke litigation). 

23 

Dr. Elkind serves as Resident and Fellow Section Editor for 

Neurology, for which he receives compensation from the 

AAN. 

S102. Functional Outcomes in CREST among Patients 

with Periprocedural Stroke and Myocardial Infarction 

Bart M. Demaerschalk, Robert J. Hye, O.W. Brown, Donald 

V. Heck, Irfan Altafullah, Jenifer H. Voeks, George Howard, 

James F. Meschia and Thomas G. Brott; Phoenix, AZ; San 

Diego, CA; Royal Oak, MI; Winston Salem, NC; Robbinsdale, 

MN; Birmingham, AL and Jacksonville, FL 

Background: Stroke more frequently complicated stenting, 

and myocardial infarction (MI) more frequently complicated 

endarterectomy in the Carotid Revascularization Endarterectomy 

versus Stenting Trial (CREST). 

Purpose: To inform debate on importance of these components 

of the CREST primary endpoint. 

Methods: The modified Rankin Scale (mRS), a measure 

of function, was assessed at one year, and scales were available 

for 72 (89%) of the stroke patients and 34 (81%) of 

the MI patients. 

Results: There was no evidence of a difference in the distribution 

of mRS (pCMH ¼ 0.61). Complete or nearly complete 

recovery (mRS 0-1) was achieved for 43 (60%) of the 

stroke patients and 23 (68%) of the MI patients. Slight to 

severe disability (mRS 2–5) was achieved for 16 (22%) of 

the stroke patients and 5 (15%) of the MI patients. Death 

occurred in 13 (18%) of the stroke patients and in 6 (18%) 

of the MI patients. Disability and death were more frequent 

for both the stroke and MI patients compared to those with 

neither (p


brain infarction volume and 35% vs. 22% inhibition of 

neurological deficit, respectively). Treatment with human serum 

albumin and vehicles (stabilizing solutions) for IVIG 

and C1-INH did not improve the magnitude of brain 

injury observed in non-treated controls. Our data suggest 

that complement attenuation could be considered as a novel 

interventional therapy for AIS. 

Study supported by: CSL Behring 

Salary and consulting fees. 

S104. Does ACE (rs4646994) and a ADDUCIN 

(rs4961) Gene Polymorphisms Predicts the Recurrence 

of Hypertensive Intracerebral Hemorrhage 

Usha K. Misra, Jayantee Kalita, Bindu I. Somarajan, 

Bishwanath Kumar, Moromi Das and Balraj Mittal; 

Lucknow, Uttar Pradesh, India 

Objectives: To evaluate ACE and aADDUCIN gene polymorphisms 

in patients with nonrecurrent and recurrent HICH. 

Methods: Out of 350 HICH, 33 (9.4%) patients had recurrence. 

ACE (rs4646994) and aADDUCIN (rs4961) gene 

polymorphisms was done in patients and 198 controls. Risk 

factors, clinical, CT, ACE (rs4646994) and aADDUCIN 

(rs4961) findings between recurrent and nonrecurrent 

HICH were compared. 

Results: The stroke risk factors and drug compliance were 

similar between the groups. Ganglionic-ganglionic recurrence 

was commonest(75.6%) and all had at least one ICH in hypertensive 

location. DD genotype (OR6.18,95%CI2.93- 

13.02) and D allele (OR2.43,95%CI1.70-3.47) of ACE was 

associated with nonrecurrent ICH compared to controls. In 

patients with recurrent ICH, DD genotype (OR7.46,95% 

CI2.8-19.4) and D allele (OR3.16, 95%CI1.83-5.46) of ACE 

and GW (OR3.49,95%CI 1.47-8.28) and WW (OR 2.9, 

95%CI 1.40-4.30) genotype and W allele 

(OR7.46,95%CI2.80-19.40) of aADDUCIN were more frequent 

compared to controls. Recurrent ICH also had higher 

frequency of WW genotype (OR9.43,95%CI1.49-59.50) and 

W allele (OR2.19,95%CI1.11-4.03) compared to nonrecurrent 

ICH. Higher frequency of DDþWW (P ¼ 0.008) and 

DD/WWþID/GW (P ¼ 0.0001) genotypes were found in 

recurrent compared to nonrecurrent ICH. 

Conclusion: Variant genotype combinations of ACE and 

aADDUCIN render the hypertensive patient vulnerable for 

recurrent ICH. 

Study supported by: Study is financially suppoerted by 

Indian Council of Medical Research, Government of India. 

S105. Risk of Intracerebral Hemorrhage in t-PA Treated 

Patients with Elevated INR 

William P. Neil, Rema Raman, Ernstrom Karin and Thomas 

M. Hemmen; San Diego, CA 

Use of Intravenous tissue Plasminogen Activator (IV t-PA) 

for stroke is limited to patients with an INR of less than 

1.7. Initial guidelines recommended against use of IV t-PA 

in patients with an INR >1.4. This was later revised 

to >1.7. 

We analyzed our database for safety and outcome in 

patients with acute ischemic stroke treated with IV t-PA 

from 2004–2010. We separated patients into two groups: 

those with INR 0.8–1.3, N ¼ 210 (group 1) and those 

with INR 1.4 - 1.7, N ¼ 12 (group 2). 

Intracerebral hemorrhage occurred in 19/210 (9.05%) in 

group 1 and 2/12 (16.67%) in group 2 (p ¼ 0.316). Symptomatic 

intracerebral hemorrhage (sICH) was found in 4/ 

24 

210 (1.9%) in group 1, and 2/12 (16.67%) in group 2 (p 

¼ 0.036). At 90 days, 44/144 (30.56%) in group 1 had 

mRS 0-1, and in group 2, 1/8 (12.5%, p ¼ 0.437). 

We found no difference in sICH or clinical outcome in 

patients with high INR after IV t-PA. The data is limited 

to few patients. Larger prospective studies are needed to 

prove safety and efficacy of IV t-PA in those with higher 

INR. 

S106. Toward a Further Clinical Physiological 

Elucidation: Immediate Regression of Leukoaraiosis after 

Carotid Artery Revascularization 

and Yu-Ming Chuang; New Taipei City, Taiwan 

Background: To correlate leukoaraiosis, cerebral perfusion 

and circle of Willis (CoW) flow patterns after carotid artery 

revascularization. 

Methods: Leukoaraiosis (LA) on fluid attenuation 

inversion recovery (FLAIR) magnetic resonance (MR) 

images at the levels of the centrum semiovale and those 

of the frontal horns at both cerebral hemispheres were 

scored in 62 contiguous patients (men/women ¼ 38/24, 

mean age ¼ 63.2 þ- 8.4 years, range 44–82) before and 

after unilateral carotid artery revascularization (CAS). The 

pre-and post-stenting difference of LA scores, CoW flow 

pattern on MR angiography and MR perfusion parameters 

was analyzed. 

Results: Post-stenting sum of leukoaraiosis score on 

FLAIR imaging was regressed from 9.87þ 0.65 to 

8.33þ 0.72 (P ¼ 0.03). Subjects were assigned to the 

complete CoW group (N ¼ 21) vs. the incomplete CoW 

group (N ¼ 41). Incomplete CoW group had a higher preoperative 

LA load and higher interhemispheric asymmetry 

index of relative cerebral blood volume (rCBV) which could 

be significantly regressed post-operatively. 

Conclusions: A perfusion related remodeling of the leukoaraiosis, 

regarding the factor of CoW configuration was 

observed. 

Study supported by: CGMH 

S107. Ischemic Stroke Exome Pilot Study 

John W. Cole, Xinyeu Liu, Luke J. Tallon, Lisa K. Sadzewicz, 

Oscar C. Stine, Nicole Dueker, Yuching Cheng, Marcella A. 

Wozniak, Barney J. Stern, James F. Mitchell, Braxton D. 

Mitchell, Steven J. Kittner and Jeffrey R. O’Connell; 

Baltimore, MD and Jacksonville, FL 

Background: The genetic architecture of ischemic stroke is 

complex and likely to include rare or low frequency variants 

with high penetrance and large effect sizes. Because a 

significant portion of human functional variation may 

derive from the protein-coding portion of genes we undertook 

a pilot study to identify variation across the human 

exome. 

Methods: Tenischemicstrokecasesincluding8African- 

Americans and 2 Caucasians, consisting of 2 dissections, 5 

lacunar, and 3 cryptogenic strokes underwent exome capture 

and sequencing implementing Illumina and Agilent 

technology. Whole-genome alignments of the sequence 

reads were performed using Burrows-Wheeler Aligner 

against NCBI build 36.1 with the resultant exome data 

evaluated for capture efficiency and rare variants as associated 

with stroke. 

Results: Sequencing generated an average of 25.5 million 

read pairs (75bp x 2) and 3.8Gbp per sample. After 

passing quality filters, screening the exomes against dbSNP 



demonstrated an average of 2839 novel SNPs among African-Americans 

and 1105 among Caucasians. Nine gene 

isoforms demonstrated compound heterozygosity across all 

ten samples. Additional analyses are ongoing. 

Conclusions: We propose that rare coding variants predispose 

to the risk of ischemic stroke and that exomic-based 

analyses are a viable method to identify such variants. 

Study supported by: Department of Veterans Affairs 

S108. Phosphodiesterase Inhibitors Modulate Human 

Brain Microvascular Endothelial Cell Barrier Properties 

and Response to Injury 

Shuo Liu, Fan Yang, Chuanhui Yu, Annlia Paganini-Hill and 

Mark Fisher; Irvine, CA 

Brain microvascular disorders have high prevalence but few 

treatment options. To develop new strategies for these disorders, 

we analyzed effects of phosphodiesterase inhibitors 

on human brain microvascular endothelial cells (HBEC). 

We modified barrier properties and response to injury of 

HBEC with three phosphodiesterase (PDE) inhibitors: cilostazol 

(PDE-3 inhibitor), rolipram (PDE-4 inhibitor), 

and dipyridamole (non-specific PDE inhibitor). Cilostazol 

and dipyridamole altered distribution of endothelial Factin. 

Cilostazol increased expression of tight junction protein 

claudin-5 by 118 % compared to control (p

In this small sample size study, incidental MH as detected 

by Gradient Echo Sequence MRI was not associated with 

increased risk for HT of ischemic stroke. However, prior 

intake of anti-platelet agents was associated with decreased 

risk for HT of ischemic stroke. 

S112. Younger Patients Have Lower Quality of Life after 

Intracranial Aneurysm Diagnosis 

Nerissa Ko, Richard Hornung, Charles Moomaw, Laura 

Sauerbeck and Joseph Broderick; San Francisco, CA and 

Cincinnati, OH 

Objective: Quality of life (QOL) is an important outcome 

in patients with intracranial aneurysm (IA). EuroQol EQ- 

5D is a validated preference-based survey that incorporates 

functional, physical, and mental status into a single QOL 

value. We measured EQ-5D in subjects with and without 

IA in the Familial Intracranial Aneurysm (FIA) Study. 

Methods: Within FIA families, subjects with IA were 

identified after review of clinical and imaging data. Family 

members free of IA entered the study as non-cases. Subjects 

who completed the EQ-5D survey 365 6 60 days after diagnosis 

(75 cases) or study entry (885 non-cases) were 

included in the present analysis. Multivariable logistic 

regression was performed using the EQ-5D index adjusted 

for variables known to affect QOL. 

Results: EQ-5D index was significantly lower in subjects 

with IA than in those without IA (mean 6 SD 0.84 6 

0.19 vs. 0.90 6 0.15, p


Conclusion: Traditional stroke risk factors were comparably 

controlled for asymptomatic and symptomatic patients, 

with notable success only in smoking cessation. Protocoldriven 

approaches merit further study. 

Study supported by: This study was supported by the 

National Institute of Neurological Disorders and Stroke 

(NINDS) and the National Institutes of Health (NIH) 

(R01 NS 038384) and supplemental funding from Abbott 

Vascular Solutions (formerly Guidant). 

Dr. Carlos Timaran reports receiving Speakers’ fees and 

Honoraria from Abbott Vascular. Dr. George Howard 

reports receiving compensation for activities with Bayer 

Healthcare and research support from Amgen and Bayer 

Healthcare. He is also a consultant to Abbott for preparation 

of FDA materials. 

S117. Radiologic Analysis of Thrombolysis-Induced 

Intracerebral Hemorrhage and the Role of Early Blood 

Pressure Management 

Maxim Mokin, Tareq Kass-Hout, Omar Kass-Hout, Robert 

Zivadinov and Bijal Mehta; Buffalo, NY 

Introduction: The radiographic features of thrombolysisinduced 

intracerebral hemorrhages (ICH) and factors that 

influence early hematoma expansion are not well described. 

Methods: We performed volumetric analysis of hematoma 

volumes in patients who developed ICH from intravenous 

thrombolysis for acute ischemic stroke. Analysis of covariance 

was used to evaluate for the effect of baseline blood 

pressure (BP) on initial hematoma volume and further 

growth. 

Results: We found a positive correlation between systolic 

BP following thrombolysis and initial hematoma volume (r 

¼ 0.46, p ¼ 0.03) but not for the diastolic BP (r ¼ 0.07, 

p ¼ 0.40). There was a significant increase in mean hematoma 

volume expansion when comparing results between 

the first and second (median time 9 hours 22 min) CT 

study (14.9 6 19.6 cm 3 to 26.0 6 26.7 cm 3 ,p¼ 0.04). 

There was also a negative association between the reduction 

of systolic BP and hematoma growth (r ¼ 0.67, p ¼ 

0.02), but no correlation with change in diastolic BP (r ¼ - 

0.22, p ¼ 0.28). 

Conclusion: Thrombolysis-induced ICH undergoes 

significant early expansion in size. Systolic BP plays a 

role in both initial hematoma development and early 

growth. 

Dr. Robert Zivadinov received personal compensation 

from Teva Neuroscience, Biogen Idec, EMD Serono and 

Questcor Pharmaceuticals for speaking and consultant 

fees, financial support for research activities from Biogen 

Idec, Teva Neuroscience, Genzyme, Questcor Pharmaceuticals, 

Greatbatch, EMD Serono and Bracco. Dr. Bijal 

Mehta received lecturing honoraria from Teva and Biogen 

Idec. 

S118. Anterior Circulation Stroke Causing Dizziness or 

Vertigo: A Systematic Review 

Yun Zhou, Seung-hun Lee, Ali S. Saber Tehrani, Karen A. 

Robinson and David E. Newman-Toker; Baltimore, MD and 

Gwangju, Korea 

Background: Determine frequency, localization of anterior 

circulation stroke causing dizziness/vertigo. 

Methods: Systematic review of observational studies. 

Search—electronic (MEDLINE) and manual search for English-language 

studies (1966–2011). Inclusions—dizziness/vertigo/syncope 

due to anterior circulation stroke or vascular 

27 

disease (e.g., carotid stenosis). Two independent reviewers 

selected studies, with differences adjudicated by a third. 

Study characteristics, patient symptoms, and lesion locations 

were abstracted. 

Results: We identified 414 unique citations, examined 

67 full manuscripts, and analyzed 39 studies describing 

1214 patients. Principal reasons for abstract exclusion 

were non-English language, no confirmed cerebrovascular 

diagnosis, and not anterior circulation (73%). 

Principal reasons for manuscript exclusion were no 

reported dizziness and no confirmed cerebrovascular diagnosis 

(53%). Unbiased studies of dizziness prevalence 

in anterior circulation stroke (i.e., dizziness not a mandatory 

symptom) reported a presenting symptom of 

dizziness in 12% (8.4% non-vertiginous, 3.2% vertiginous), 

syncope in 6.6% of n ¼ 683. Non-vertiginous 

symptoms were more commonly reported in right hemispheric 

lesions (73.9% vs. 26.1%, v 2 p


Method: The survey was conducted from November, 

2010 via internet using structured, self-applicable questionnaire 

addressed stroke knowledge. 

Results: We have valid responses from 11,121 people 

aged from 20 to 69 years old throughout all Japanese districts. 

66.2% of subjects were no source of informations 

about stroke. On the other hand, subjects of remaining 

33.8% can get the informations of stroke through mainly 

TV (85.2%). They understood the signs of stroke such as 

speech disturbance (95.5%). 67% of them request the ambulance 

and transport to the hospital promptly at the time 

of stroke onset but only 22.4% on the occasion of TIA. 

Importance of early diagnosis and treatment within 3 

hours of onset was well recognized among all age subjects 

(75.3%) and significantly associated with calling ambulance 

quickly when stroke (OR 4.458, 95%CI, 3.897– 

5.099, p ¼ 0.000) or TIA (OR 2.331, 95%CI, 1.923– 

2.825, p ¼ 0.000) occurred. 

Conclusions: Our survey demonstrates that it is important 

to educate stroke knowledges through TV such as judgment 

of stroke and calling ambulance when stroke or TIA 

occurred. 

S121. Is Anti Epileptic Drug Necessary in Cortical 

Venous Thrombosis? 

Velmurugendran Cannigaiper Uthamaroyan, Kaushik Sundar, 

Meenakshisundaram Umaiorubahan and Shankar 

Venkatasubramaniam; Chennai, Tamilnadu, India 

Introduction: Though CVT is associated with seizures,is it 

necessary to start AEDs in all patients with CVT? 

Aims To study the clinical profile of patients with CVT 

and to find out the incidence and etiology of seizures. 

Materials and methods: Patients who presented with 

signs and symptoms suggestive of CVT were admitted and 

brain imaging was done.If CVT was revealed,requisite investigations 

were done.Patients were then treated and followed 

up for a period of one year. 

Results: 50 (28 males/22 females) patients were diagnosed 

to have CVT.Of these 50 patients 25 of them presented with 

Seizures and all these 25 had an infarct/haemorrhage/ infarct 

with haemorrhagic transformation in their MRI. 

Discussion: We found that 50% of our study patients 

had seizures and all of them had an infarct/haemorrhage/or 

infarct with haemorrhagic transformation.The patients who 

did not have an infarct did not have a seizure and were not 

started on AEDs and remain seizure free till date. 

Conclusion: Patients with CVT who have no evidence of 

parenchymal lesion in the MRI need not be treated with 

AEDs as a prophylaxis and a wait and watch strategy can be 

followed. 

Study supported by: Self 

S122. Race and Insurance Disparities in Cardiovascular 

Risk Factors Control in Patients with Acute Stroke 

Anna M. Cervantes, Jose R. Romero, Helena Lau, Feliks 

Koyfman, Aleksandra Pikula, Thanh N. Nguyen, Carlos S. 

Kase and Viken L. Babikian; Boston, MA 

Objective: To investigate racial and socioeconomic disparities 

in cardiovascular risk factor control at the time of acute 

ischemic stroke at an urban hospital. 

Background: Racial and socioeconomic inequalities may 

play a role in the observed higher frequency and severity of 

stroke in minorities. Hypercholesterolemia, diabetes and 

28 

hypertension (HTN) are modifiable cardiovascular risk factors 

with higher prevalence in minorities. 

Design: We evaluated patients with acute ischemic 

stroke/TIA presenting to Boston Medical Center from 1/ 

2009-12/2010. Patients were stratified by race (Whites, 

Blacks, Other) and insurance status (Medicaid/no insurance, 

Medicare, Private). Cardiovascular risk factor control 

(HTN, hyperlipidemia, diabetes) was evaluated on 

admission. 

Results: Among 833 patients (mean age 61yrs, 50.4% 

women), 34.8% were white, 45.5% black and 19.7% other 

races; 50.4% were insured and 49.6% uninsured. Compared 

to white, black and other races were more likely to be uninsured 

(p


arterial and venous thrombosis. The more common, and 

dangerous form of HIT, HIT II, is caused by prothrombotic 

anti-heparin/platelet factor PF4 complex antibodies, which 

activate platelets, induce their aggregation, and trigger coagulation 

(1). The consequences, especially if not diagnosed 

early, can be devastating, ranging from deep venous 

thromboses to myocardial infarctions and strokes. HIT is 

most commonly associated with the use of heparin after orthopedic 

procedures, as compared to cardiovascular surgeries 

and medical conditions requiring admission to an intensive 

care unit. HIT is much less frequently associated with the 

use of low molecular weight heparin, such as enoxaparin 

(3). Here, we present the case of a 64 year-old woman who 

developed both a lower extremity deep venous thrombosis 

and cerebral venous sinus thromboses from the administration 

of enoxaparin after an orthopedic surgery. Our patient 

is only the second reported case of HIT-induced CVST 

related to enoxaparin administration. Our poster will 

include brain imaging, graphs and tables regarding the clinical 

case. 

Study supported by: Residency Program 

S125. Renal Failure Increases Risk for Intracranial 

Hemorrhage Following Stroke 



Background: Anticoagulation is associated with increased 

risk of intracranial hemorrhage. Renal failure can lead to 

platelet dysfunction, which may increase the risk further. 

We followed patients admitted with acute ischemic stroke 

who had an indication for anticoagulation, to determine if 

renal failure was associated with increased risk for intracranial 

hemorrhage. 

Methods: 63 patients admitted with acute ischemic 

stroke and with an indication for anticoagulation were 

included. Age-adjusted logistic regression was used to evaluate 

the association between history of renal failure and glomerular 

filtration rate (GFR) (>60 (normal), 30–60 (mildly 

reduced),


rapid deterioration over hours to days with multi-territorial 

infarctions and global edema on imaging. All had angiographic 

findings of diffuse, severe, multifocal arterial narrowings. 

Aggressive treatment was attempted in most, 

including intravenous magnesium, corticosteroids, calcium 

channel blockers, balloon angioplasty, vasopressors, and osmotic 

agents. Despite aggressive measures, all patients suffered 

fulminant courses ending in death within 8–24 days 

after delivery. 

Conclusions: Postpartum vasoconstriction can be fatal 

with rapid progression of vasoconstriction, ischemia, and 

brain edema. Postpartum women with acute neurological 

symptoms deserve close monitoring with consideration of 

noninvasive cerebrovascular imaging. 

S129. Racial Disparities in Secondary Stroke Prevention 

Practices 

Pratik Bhattacharya, Seemant Chaturvedi, Flicia Mada, Leeza 

Salowich-Palm, Sabrina Hinton, Scott Millis, Sam Watson 

and Kumar Rajamani; Detroit, MI and Lansing, MI 

Racial differences in stroke risk, risk factor prevalence and 

outcomes are established. We explored racial differences in 

secondary stroke prevention practices among stroke patients. 

Methods: A retrospective review was conducted on ischemic 

stroke patients in 5 JC (Joint Commission) certified and 5 

noncertified hospitals. Secondary stroke prevention measures 

such as antiplatelet use, statin use, antihypertensives and 

smoking cessation steps were assessed. Results: 574 patients 

(430 white, 144 African Americans(AA); 47.9% evaluated at 

JC certified centers) were included. AA were younger (mean 

age 64.4 vs 72.5; p 100 with one improved 

and 3 with worse outcome. 

Conclusion: High ischemic volume have higher NIHSS 

and worse outcome. 

Study supported by: USF College of Medicine 

S131. Treatment Outside the NINDS Stroke Study 

Exclusion Criteria: A Case Report 

Nhu T. Bruce and Brett C. Meyer; San Diego, CA 

Introduction: The original NINDS stroke trial established 

IV rt-PA as effective and defined treatment parameters. 

Some exclusions were well founded on data, others were 

chosen via consensus opinion. Successful use of rt-PA outside 

of some criteria raises concern over the continued utility 

of some exclusions. We report an intriguing case where a 

patient with prolonged PTT was successfully treated with IV 

rt-PA. 

Methods: Case report from the University of California, 

San Diego 

Results: An 86 year old woman with no hematologic history 

presented with acute ischemic stroke within the 3 hour 

window. Given her lack of being on anticoagulants or having 

hepatic dysfunction, her elevated PTT was felt to be 

spurious, and she was treated with rt-PA despite not meeting 

this inclusion criteria. Patient had significant improvement 

and work up was positive for a diagnosis of anti-phospholipid 

antibody syndrome. 

Conclusions: rt-PA treatment is effective, though only a 

minority of patients is treated. Multiple cohort studies have 

shown that deviations from the original exclusions may not 

result in worse outcomes for specific exclusion criteria. 

Reevaluating the significance and limiting the exclusion criteria 

may help to increase thrombolytic treatments. 

Study supported by: NIH SPOTRIAS 

S132. Size Matters: Predictors of Intracranial 

Hemorrhage in Stroke Patients on Anticoagulation 



Background: Anticoagulation is associated with increased 

risk of intracranial hemorrhage. This may be higher in 

patients who have had a recent stroke. We followed patients 

with acute ischemic stroke who had an indication for anticoagulation, 

to determine what factors were associated with 

increased risk for hemorrhage. 

Methods: 63 patients admitted with acute ischemic 

stroke and with an indication for anticoagulation were 

included. We evaluated risk of hemorrhage associated with 

indicators of stroke severity; namely NIH Stroke Scale 

(NIHSS) score and infarct size (largest lesion and total 

lesion volume), using age-adjusted logistic regression. 

Results: Higher NIHSS scores predicted increased risk of 

hemorrhage. For every 3 point increase, risk for hemorrhage 

increased 51% (OR 1.51, 95% CI 1.01–2.25). Total volume 

of the infarct also approached statistical significance 

(OR 2.6 per larger quartile, 95% CI 0.86–7.56). 

Conclusions: In patients with acute ischemic stroke who 

also have an indication for anticoagulation, the severity of 



their stroke, indicated by their NIHSS score and volume of 

infarct, is a strong predictor of their risk for hemorrhage. 

Study supported by: The first author, EB Marsh, is supported 

in part by a NINDS R25 Research Grant- R25 

NS065729. 

EB Marsh is currently a resident, partially supported by 

funding from the R25 Research Grant. 

S133. A Rare Presentation of Anterior and Posterior 

Spinal Arteries Ischemia during Dilaysis 

Noor Yono, Adrian Marchidann and Rabih Kashouty; 

Manhasset, NY and New York, NY 

Spinal cord infarction is considered to be a rare event and 

usually presents with severe neurological deficits. Multiple 

etiologies have been described; however, this is the first case 

report of spinal cord infarction in the territory of the anterior 

and posterior spinal arteries secondary to hypotension 

during dialysis. We report a case of a 60 year-old man with 

past medical history of severe atherosclerosis, coronary artery 

disease and acute kidney failure, who presented with lower 

extremity paraplegia during hemodialysis secondary to hypotension. 

MRI of the spine demonstrated spinal cord infarction 

starting at the level of T8 and below. Clinical features 

suggested that the lesion was an ischemic infarct. In addition 

to paraplegia, he demonstrated absence of sensation to 

dull touch, pin prick, temperature, proprioception and 

vibration. Hypotension during dialysis could carry significant 

risk of spinal cord infarction. This unusual infarction 

demonstrates the importance of blood pressure control in 

susceptible patients with severe atherosclerotic disease. Physicians 

should be aware of such complications. 

S134. Neurosarcoidosis: A Case Presentation 

Amtul Farheen, Nancy Gadallah, Rony Dekermenjian, 

Michael Rosenberg and Sushanth Bhat; Edison, NJ 

27 y/o man with no significant medical history woke up 

with weakness and parasthesias of his right side. He smoked 

1ppd for more than 10yrs. Neurological examination was 

significant for decreased power on right side. Right upper 

extremity revealed power of 2/5 deltoid, 3/5 bicep/tricep/ 

brachio, 1/5 intrinsic hand muscles, Right lower extremity 

revealed: 4/5 iliopsoas, hams, quads 0/5 dorsi and plantar 

flexion 0/5. Sensation was mildly decreased on right side. 

Reflexes were asymmetric and increased with upgoing toe 

on the right. MRI brain showed small left medullary hyperintensity. 

Csf had opening Pressure of 23, RBC 222, WBC 

7, Prot 57.8, Glucose 65. CSF was negative for gram stain, 

protein electrophoresis, oligoclonal bands, and infectious 

work up. Csf ACE 0.1. Serum ACE was 109. CT chest 

showed mediastinal and bilateral hilar lymphadenopathy. 

Mediastinal lymph node biopsy revealed noncaseating granulomas 

diagnostic of neurosarcoidosis. The patient was 

started on iv steroids and was discharged on oral prednisone. 

He improved clinically and was later almost back to 

his baseline. 

Although sarcoidosis is rarely confined to the nervous system, 

its neurological features frequently occur early in the 

course of the disease leading to diagnostic confusion. 

S135. Stroke Outcomes Based on MERCI/PENUMBRA 

Intervention and Hemodynamics 

Jay-Ming Wang**, Zahid I. Choudary, Shazia Z. Choudhary, 

Michael Sloan, Harry R. Van Loveren, Karen R. Wilson and 

David A. Decker; Tampa, FL 

Hypothesis: What are the effects of MERCI/PENUMBRA 

and blood pressure on stroke outcome? 

31 

Background: Not all guidelines for stroke are well supported 

by evidence. We seek to retrospectively study the 

effects of MERCI/PENUMBRA stroke interventions and 

hemodynamics to see ischemic stroke outcome. 

Methods: A retrospective chart review of 24 patients with 

hospitalization with ischemic stroke with age range 18 to 

90, mean age 70.6, 13 males, and 11 females. The blood 

pressure was correlated with ischemic stroke outcome. The 

patients undergoing MERCI/PENUMBRA intervention 

were analyzed. 

Results: We noted a significant positive correlation 

between higher blood pressures and worse outcomes in 

patients with large volume lesions. 

In the 8 patients with MERCI/PENUMBRA intervention, 

we had 3 with worse outcomes and 5 with improvement 

at discharge. Of the 3 with worse outcomes, 2 had 

large lesions and 1 had a small lesion. The 5 improved outcomes 

had 3 small lesions and 2 medium lesions. Three of 

the five had received IV tPA prior to interventional therapy. 

Two did not. 

Conclusion: MERCI/PENUMBRA intervention has an 

overall positive outcome on patients suffering from ischemic 

stroke. 

Higher blood pressures predict a worse outcome in 

patients with stroke lesions greater than 100 ml. 

Study supported by: USF College of Medicine 

S136. Migraine-Related ICH – A Case Study and Review 

Shivani Ghoshal**, Sankalp Gokhale and Louis Caplan; 

Boston, MA 

54 year-old right-handed male, with a 25-year history of 

frequent migraine headache, presented with an episode of 

severe migraine headache associated with visual changes; 

imaging revealed new left frontal intracranial hemorrhage. 

Since then, the patient had two different types of paroxysmal 

attacks. One consisted of prolonged severe headaches 

with multisystem sensory deficits, most consistent with migraine 

with aura. The second consisted of brief visual and 

auditory perseveration, likely representing focal seizures near 

the region of ICH. Excluding the initial presentation, the 

subsequent episodes left no discernible damage on clinical 

exam or imaging. Migraine is a form of vascular headache, 

postulated to cause direct vasoconstriction of cerebral vessels. 

This vasospasm may, in some cases, lead to reperfusion 

edema, with intracerebral bleeding to follow. Though migraine-related 

ischemia has been well-documented, there 

have been few noted cases of migraine-related ICH and 

none to date in male patients; the association of migraine 

and ICH is likely under-recognized and hence underreported. 

Given the dangers for cerebral hemorrhage, future 

migraine treatment may benefit from weighing the risk of 

vasoactive agents. We take this opportunity to review the 

current literature to provide guidance for improvement in 

care. 

S137. Stroke as a Complication of Tuberculous 

Meningitis 

Amtul Farheen, Sumaiya Salim, Zoha Fasih, Rony 

Dekermenjian and Sushanth Bhat; Edison, NJ 

A 28 y/o Hispanic man who moved to US from Guatemala 

four years before presentation, had no significant past medical 

history presented with 2 days of progressive confusion 

and headache. He reported weight loss, severe cough for few 

days with low grade temperatures and night sweats. He 

smoked occasionally. On examination he was lethargic and 

cachectic, neck stiffness noted, enlarged lymph nodes of the 



neck. He was tachycardic and had diminished breath sounds 

with bilateral crackles. Neurological examination revealed 

normal cranial nerve examination. He moved all extremities 

spontaneously. Initial CT head was normal. Lumbar puncture 

revealed WBC 53 (neutrophils predominantly), RBC 7, 

Protein 170, Glucose 7. Sputum was positive for AFB. HIV 

testing was negative. He was diagnosed with disseminated 

tuberculosis with tuberculous meningitis and started on 

antituberculous medications. On the third day of admission 

he had decreased movement of the left side of his body and 

a repeat CT of his head showed a new infarct in the right 

internal capsule. 

Vasculitis of the vessels of circle of Willis which may 

present as stroke is one of the most dreaded complications 

of Tuberculous meningitis. 

S138. Patterns and Mechanisms of Head-Shaking 

Nystagmus in Anterior Inferior Cerebellar Artery 

Infarction 

Young Eun Huh and Ji Soo Kim; Seongnam-si, Gyeonggi-do, 

Korea 

Infarctions involving the anterior inferior cerebellar artery 

(AICA) territory give rise to unique clinical features of combined 

peripheral and central vestibulopathies. Evaluation of 

head-shaking nystagmus (HSN) may provide information 

on central and peripheral vestibular dysfunction. We analyzed 

patterns of spontaneous nystagmus (SN) and HSN in 

18 patients with acute infarction involving the AICA territory 

which was confirmed on MRI. Patients also underwent 

head impulse and bithermal caloric tests, and pure tone 

audiogram. Twelve patients (12/18, 67%) showed unilateral 

caloric paresis (n ¼ 11) or abnormal head impulse test (n 

¼ 9) on the side of the infarction and most of them (10/ 

12, 83%) also had acute hearing loss. Fifteen patients 

(83%) exhibited HSN, and the horizontal HSN was usually 

contralesional (10/14, 71%). However, 9 patients also 

showed patterns of central HSN which included perverted 

HSN (n ¼ 7), ipsilesional HSN (n ¼ 4), and HSN in the 

opposite direction of SN (n ¼ 4). Especially, perverted 

HSN was induced in half of the 6 patients without audiovestibular 

loss. In AICA infarction, HSN was common with 

both peripheral and central patterns. Careful evaluation of 

HSN may provide clues for AICA infarction in patients 

with acute unilateral audiovestibular loss. 

Study supported by: This study was supported by grant 

of the Korea Health 21 R&D Project, Ministry of Health 

& Welfare, Republic of Korea (A080750) 

S139. Co-Complaints Influence Odds of Stroke 

Diagnosis in ED Dizziness 

Ali S. Saber Tehrani, Yu-Hsiang Hsieh, Jonathan A. Edlow, 

Carlos A. Camargo and David E. Newman-Toker; Baltimore, 

MD and Boston, MA 

Background: Assess impact of co-complaints on stroke 

diagnosis in dizziness presentations to US emergency departments 

(EDs). 

Methods: Design—Cross-sectional study of ED visits 

from the National Hospital Ambulatory Medical Care Survey. 

Setting—Weighted sample of US ED visits (1993– 

2005). Participants— 16 years old; reason-for-visit code 

vertigo/dizziness (1225.0). Measures—co-complaints, ICD-9 

diagnoses. Co-complaints grouped as otologic (e.g., tinnitus); 

neurologic (e.g., weakness); medical (e.g., chest pain); or other 

(including multiple co-complaint groups). Diagnoses 

grouped using HCUP categories. 

32 

Results:7,925 dizzy patient complaints (3% of visits, 

weighted 28.3 million) sampled over 13 years. Dizziness 

was ‘‘isolated’’ (none, nausea/vomiting, or otologic co-complaints) 

in 21%; others with dizziness had only neurologic 

(10%), only medical (15%), or other (54%) co-complaints. 

Cerebrovascular causes were diagnosed in 4.1% (isolated), 

10% (neurologic), 0.9% (medical), 3.8% (other), 1.0% 

(non-dizzy controls). Neurologic co-symptoms increased 

odds (2.57, 95%CI 1.85 to 3.58) and medical co-symptoms 

decreased odds (0.21, 95%CI 0.10–0.41) of a cerebrovascular 

diagnosis relative to isolated dizziness. 

Conclusions: ED physicians use associated symptoms to 

diagnose dizzy patients. While this reflects an appropriate 

assessment of disease probability, it could increase the risk 

of stroke misdiagnosis in isolated dizziness or dizziness with 

medical symptoms. 

Study supported by: This study was supported principally 

by the National Institutes of Health, National Center for 

Research Resources (NCRR) grant K23 RR 17324-01. 

S140. Eclamptic Versus Non-Eclamptic PRES (Posterior 

Reversible Encephalopathy Syndrome): Comparison of 

Clinical Features and Response to Treatment 

Pavan Bhargava, Fazeel Siddiqui, Vivek Patel, Rodger J. Elble 

and Srikanth Vallurupalli; Springfield, IL 

Objective: To examine the hypothesis that eclamptic PRES 

responds more rapidly to treatment than non-eclamptic 

PRES. 

Background: PRES is characterized by reversible vasogenic 

edema, predominantly in the posterior circulation distribution, 

and can occur in various clinical settings such as 

eclampsia and malignant hypertension. It is unknown 

whether clinical, imaging and EEG features differ in 

eclamptic versus non-eclamptic PRES. 

Design/Methods: Retrospective data including clinical, 

EEG and MRI characteristics was collected on patients diagnosed 

with PRES between January 2003 and June 2010. 

Appropriate statistical methods were employed. 

Results: 45 episodes of PRES occurred in 43 

patients(69% women) with a mean age of 44.5 years(range 

9–82). The nine patients with eclampsia had significantly 

lower age(25 6 5 yrs vs. 50 6 20 yrs, p ¼ 0.002), shorter 

duration of stay following diagnosis(3.1 vs. 7.6 days, p ¼ 

0.002), less incidence of renal impairment(0% vs. 39%, p 

¼ 0.04) and lower likelihood of abnormal EEG finding(33% 

vs. 85%, p ¼ 0.07) compared to non-eclamptic 

PRES. The etiologies of non-eclamptic PRES were hypertension(86%), 

sepsis(17%), transplant(11%) and connective 

tissue disorder(17%). 

Conclusions: Eclamptic PRES appears to respond more 

rapidly with treatment of the underlying condition than 

non-eclamptic PRES, which has a more varied etiology. 

Movement Disorders 

S201. Allele Specific RNAi Against Triplet Repeat 

Disease-Causing Alleles in Huntington Disease 

Hirokazu Furuya, Masaki Takahashi, Shoko Watanabe, Miho 

Murata, Ichiro Kanazawa, Keiji Wada and Hirohiko Hohjoh; 

Omuta, Fukuoka, Japan and Kodaira, Tokyo, Japan 

Suppression of disease-causing alleles is a potential approach 

to treatment of intractable diseases caused by dominate-negative 

alleles, such as triplet repeat diseases. RNAi may be an 

applicable tool in medical treatment; however, the identification 

of nucleotide variations and the design of siRNAs 



conferring disease allele-specific RNAi are quite difficult. 

Here we report an innovative procedure that facilitated allele-specific 

silencing of disease-causing alleles in Huntington 

disease (HD), a triplet repeat disease. We developed a 

pull-down method to rapidly identify coding SNP (cSNP) 

haplotypes of triple repeat, disease-causing alleles, and demonstrated 

disease allele-specific RNAi that targeted cSNP 

sites in mutant Huntingtin (HTT) alleles, each of which 

possessed a different cSNP haplotype. We examined 5 

patients with HD, found heterozygous cSNP sites in two 

patients, and successfully suppressed the disease-causing alleles 

in lymphoblastoid cells derived from the patients by 

means of RNAi. Therefore, the methods allow for diseasecausing 

allele specific silencing targeting disease-linked 

cSNP haplotypes of triplet repeat diseases. 

Study supported by: This work was supported by research 

grants from the Ministry of Health, Labour and Welfare of 

Japan 

S202. Antigen-Sensitized Dendritic Cell Vaccine Against 

Human a-Synuclein: A Potential Cell-Based Therapy 

Against Parkinson’s Disease 

Chuanhai Cao, Xiaoyang Lin, Wang Li, Cai Jianfeng, Li 

Kunyu, Song Shijie and Juan Sanchez-Ramos; Tampa 

The goal of this study was to develop a cell-based vaccine 

directed against human a-synuclein (hAS). 

Human recombinant a-synuclein (hrAS) was expressed in 

BL21 cells and AS peptides were synthesized based on antigen 

analysis. Mouse bone marrow-derived dendritic cells 

(DCs) were generated ex vivo, sensitized with hrAS or AS 

mixed peptides and then delivered i.v. to Tg hAS mice. 

Mice vaccinated with DCs sensitized with a peptide mixture 

(DC-ASpep) exhibited detectable anti-AS antibody earlier 

than mice vaccinated with DC-sensitized with human 

recombinant protein (DC-hrAS). With repeated treatments, 

antibody levels in the DC-hrAS was higher than in the DC- 

ASpep group. AS levels were significantly decreased at both 

3 rd and 5 th treatment compared to the control group. In behavioral 

tests, treatment groups performed better on the 

rotometer than control mice; the DC-ASpep group exhibited 

the best performance. Among all cytokines measured, 

only GM-CSF and IL1a were significantly changed after 

treatment, indicating that this treatment method has no 

potential to induce inflammation. 

Conclusions: AS-sensitized dendritic cell vaccination is 

effective, specific, long-lasting and unlike antigen-based vaccines, 

does not elicit non-specific activation of the immune 

system. 

Study supported by: Parkinson Study Group and Helen 

Ellis Endowed Chair Fund 

S203. Depressive Symptoms and Neurodegeneration in 

the Locus Coeruleus: The Honolulu-Asia Aging Study 

Ross Webster, Robert D. Abbott, Helen Petrovitch, Kamal H. 

Masaki, Caroline M. Tanner, Jane H. Uyehara-Lock and 

Lon R. White; Honolulu, HI; Hiroshima, Japan and 

Sunnyvale, CA 

Depression commonly occurs in Parkinson’s disease (PD), 

often pre-dating diagnosis. Lewy pathology in the locus 

coeruleus (LC) occurs early in PD and LC neurodegeneration 

occurs in depressed PD patients. Whether depressive 

symptoms (DS) are associated with LC neurodegeneration 

prior to onset of classic PD motor features remains unclear. 

Our objective is to examine the association of DS and LC 

neurodegeneration in decedents from the longitudinal Honolulu-Asia 

Aging Study. DS were assessed using the 11-item 

33 

modified Center for Epidemiologic Studies Depression Scale 

at examinations from 1991–1993 in 403 autopsied participants. 

A score >8 was defined as significant DS. LC neurodegeneration 

was based on semi-quantitative assessments by 

a neuropathologist shielded from clinical information. After 

age-adjustment, significant neurodegeneration was observed 

in 32.9% of 55 decedents with DS versus 17.8% in 348 

without DS (p ¼ 0.010). DS continued to be associated 

with LC neuron loss after removing 27 PD or dementia 

with Lewy bodies cases (p ¼ 0.044), and after removing 89 

cases with any Lewy pathology (p ¼ 0.010). Findings suggest 

that DS correlate with LC involvement in PD prior to 

development of classic motor symptoms as well as in some 

individuals without Lewy pathology. 

Study supported by: NIA, NIH; NINDS, NIH; Department 

of Defense; Office of Research and Developement, 

Veterans Affairs 

S204. MRI in HDLS Shows a Unique Mechanism of 

Neuroaxonal Degeneration 

C. Sundal*, C. Wider, J.A. Van Gerpen, J. Lash, J.Y. 

Garbern, K.J. Schweitzer, J. Aasly, B. Goodman, B.K. 

Woodruff, C.W. Christine, M. Baker, J.E. Parisi, S. Roeber, S. 

DeArmond, R. Rademakers, D.W. Dickson, D.F. Broderick, O. 

Andersen and Z.K. Wszolek; Gothenburg, Sweden; Jacksonville, 

FL; Rochester, NY; Trondheim, Norway; Scottsdale, AZ; San 

Francisco, CA; Rochester, MN and Munchen, Germany 

Background: Hereditary diffuse leukoencephalopathy with 

spheroids (HDLS) is an autosomal dominant neurodegenerative 

disorder with symptomatic disease onset usually in 

adult age and with relatively rapid course leading to death 

in less than a decade. It is clinically characterized by a constellation 

of symptoms including personality changes, cognitive 

dysfunction and motor impairments, often leading to a 

clinical misdiagnosis. Neuropathology is characterized by 

axonal spheroids. The disease-causing gene is unknown. 

Methods: Over the last 7 years, we have collected HDLS 

families both retrospectively and prospectively. We performed 

head magnetic resonance imaging (MRI) studies 

with only neuropathological proven HDLS cases/kindreds. 

Results: From all 13 families available to us, 14 cases 

were studied. 4 had repeated examinations. MRI demonstrated 

unique distribution of changes in the periventricular, 

deep and subcortical white matter of frontal/parietal lobes 

and in corpus callosum. 

Conclusions: MRI is helpful to substantiate the diagnosis 

of HDLS. It will be an important biomarker for HDLS 

progression after the gene is identified. It will also be very 

useful to follow the patients undergoing future experimental 

treatments if such are developed. 

Study supported by: C.Sundal supported by research 

grants through Gothenburg University, Sweden 

S205. Appropriate Outcome Measures for Cognitive 

Trials in Huntington’s Disease 

Jody Corey-Bloom, Jody Goldstein, Shea Gluhm, Charles Van 

Liew, Stephanie Lessig, Jagan Pillai and Steven D. Edland; La 

Jolla, CA 

Suitable measures are needed for clinical trials of therapeutic 

agents for cognition in Huntington’s disease (HD). The 

Montreal Cognitive Assessment (MoCA), Mini-Mental State 

Examination (MMSE), and Mattis Dementia Rating Scale 

(DRS) are commonly used cognitive instruments; however, 

little is known about longitudinal change on these measures 

in HD. We used mixed effects models to analyze MoCA, 

MMSE, and DRS scores obtained during 2.5–5 years of 



follow-up of up to 153 subjects with HD. Annual rates of 

decline on the MoCA and MMSE were 1.0 and 0.69 

points/year, respectively, over 2.5 years of follow-up. Rate of 

decline on the DRS was 3.04 points/year in the first 5 years 

of observation. For cognitive trials in HD, sample size 

requirements (90% power, critical alpha level 0.05) for a 3year 

trial to detect 50% reduction in mean rate of decline 

using the MoCA, MMSE, or DRS would be 106, 173, and 

165 subjects/ treatment arm, respectively. We conclude that, 

although all three measures decline significantly over time in 

an unselected HD population, the MoCA may be a more 

efficient instrument for assessing effects of a therapeutic 

agent for cognition in HD. 

Study supported by: Huntington’s Disease Society of 

America and Shiley-Marcos Alzheimer Disease Research 

Center NIH P50 AG005131. 

S206. Distinctive Neurocognitive Profiles Associated 

with Right and Left Motor Symptom Onset in 

Parkinson’s Disease 

Paul J. Eslinger, Daymond Wagner, Suman Sen, Mechelle M. 

Lewis, Guangwei Du, Michele L. Shaffer and Xuemei Huang; 

Hershey, PA 

Parkinson’s disease (PD) is commonly diagnosed in patients 

presenting with asymmetric motor impairments. However, 

the literature remains mixed with regard to the significance 

and prognostic value of identifying first-onset motor symptoms 

particularly for cognitive functioning and progression. 

We investigated the hypothesis that asymmetric motor 

impairment and attendant dopamine loss are associated with 

distinctive right- and left-onset profiles in 36 PD subjects 

(21 with right-sided,15 with left-sided motor symptom 

onset) and 44 matched healthy controls recruited for longitudinal 

cognitive and anatomical studies. Although samples 

did not differ on screening cognitive measures (Mini-Mental 

Status Examination, Montreal Cognitive Assessment), contrasting 

profiles of neurocognitive impairments were 

detected in PD. Specifically, left-sided motor symptoms 

were associated with prominent deficits in visuospatial learning 

and memory, visuospatial speed/accuracy in mirror tracing, 

and design fluency. In contrast, right-sided motor 

symptoms were associated with decline in cognitive flexibility 

and heightened symptoms of depression. Results support 

the hypothesis that asymmetric motor symptoms in PD are 

associated with distinctive patterns of early cognitive change 

that may require different management approaches. Analyses 

are underway to examine the relationship between such cognitive 

profiles and cortical changes in PD. 

Study supported by: National Institutes of Health 

S207. The Clinical and Pathological Features of Familial 

Parkinsonism with EIF4G1Gene Mutation 

Shinsuke Fujioka, Owen A. Ross, Rosa Rademakers, Matthew 

J. Farrer, Dennis W. Dickson and Zbigniew K. Wszolek; 

Jacksonville, FL and Vancouver, BC, Canada 

Recently, we have performed genome-wide linkage analysis 

of several families with autosomal-dominant late-onset parkinsonism 

and identified mutations in a novel susceptibility 

gene for Parkinson’s disease, eukaryotic translation initiation 

factor 4-gamma 1 isoform (EIF4G1) on chromosome 3q26- 

27. The missense mutations, EIF4G1 c.2057G>T 

(p.G686C) and c.3589C>T (p.R1197W) were identified 

together in two siblings of US 331-95 family. We reviewed 

available medical records and brain autopsy reports for both 

siblings. Mean age at symptomatic onset was 79 years and 

mean disease duration was 5.5 years. Both siblings presented 

34 

with hallucinations or depression followed by memory 

impairment. The younger brother developed levodopa responsive 

asymmetrical parkinsonism characterized by resting 

tremor, rigidity, and bradykinesia during the course of his 

illness. The autopsy showed transitional (limbic) type of 

Lewy body disease (LBD) in the brother and diffuse LBD 

in the older sister, with mild to moderate Alzheimer’s disease 

pathology (Braak stage II-IV) in both. We will present 

our clinicopathologic review with future genealogical and 

clinical research planned on this family. 

Study supported by: ZKW is partially supported by the 

NIH/NINDS 1RC2NS070276, NS057567, P50NS072187, 

Mayo Clinic Florida (MCF) Research Committee CR programs 

(MCF #90052018 and MCF #90052030), and gift 

from Carl Edw and Bolch, Jr., and Susan Boss Bolch (MCF 

#90052031/PAU #90052). FS is partially supported by 


(MDF #90052018). 

S208. Alcohol Consumption and Risk of Parkinson 

Disease 

Rui Liu, Yikyung Park, Xuemei Huang, Xuguang Guo, Albert 

Hollenbeck, Aaron Blair and Honglei Chen; Research Triangle 

Park, NC; Rockville, MD; Hershey, PA and Washington, DC 

Objective: To prospectively examine the association between 

overall and individual types of alcohol consumption and 

risk of Parkinson disease (PD). 

Methods: Participants comprised 1,086 PD cases and 

299,684 individuals without PD from the NIH-AARP Diet 

and Health Study. Alcohol consumption was assessed in 

1995–1996 and PD was diagnosed in 2000–2006. 

Results: Total alcohol consumption was not associated 

with PD. However, moderate beer consumption was associated 

with lower risk of PD. After controlling for potential 

confounders and other types of alcohol consumption, the 

multivariate odds ratio (OR) for beer drinkers was 0.81 

(95% confidence interval [CI] 0.70–0.94) for


kindred in 1984. An additional 11 kindreds and 8 sporadic 

cases have been reported over the years. Inheritance is autosomal 

dominant. HDLS presents with psychiatric, cognitive 

and motor symptoms. A definite diagnosis is made pathologically 

by demonstrating axonal spheroids. The causative 

gene is unknown. 

Method: Brains and brain biopsy specimens were collected 

through world wide collaborations and only those 

cases/families demonstrated pathological features of HDLS 

were included in our study. 

Results: 20 affected individuals were identified in 13 kindreds. 

During the course of the illness all patients developed 

parkinsonian signs along with other classic symptoms of 

HDLS. There was no response to levodopa. The mean age 

of onset was 45 years (range, 16–68), and mean disease duration 

was 4 years (range, 1–11). 

Discussion and Conclusions: HLDS is an under-diagnosed 

neurodegenerative condition. Cases are misdiagnosed 

as AD, PD, CBD, MS, FTD, FTDP, and others. Finding 

the gene for this condition will be of paramount importance 

in understanding the neurodegeneration associated with 

white matter abnormalities. 

Study supported by: C.Sundal supported by research 

grants through Gothenburg University, Sweden 

S210. Co-Existing HTT and ATXN8OS Repeat 

Expansions and a ‘Face of the Giant Panda’ Sign on 

MRI in a Patient with a Complex Movement Disorder 

Shin C. Beh, Cherian A. Karunapuzha and Shilpa Chitnis; 

Dallas, TX 

We present a 66 year old woman with a complex movement 

disorder syndrome. The disease began in her mid-fifties with 

fidgetiness. She later developed progressive gait ataxia and 

chorea. Unintended weight loss, mild dysphagia, depression 

and mild memory impairment were also present. Examination 

revealed square-wave jerks, grimacing, motor impersistence, 

generalized choreiform movements, hyperreflexia and 

an ataxic, choreic gait. Her mother had a similar movement 

disorder. In addition to cortical atrophy, T2-weighted brain 

MRI demonstrated mesencephalic white matter hyperintensity 

with areas of hypointensity in the red nuclei, substantia 

nigra and superior colliculi taking on the appearance of the 

‘‘face of the giant panda’’ sign. Genetic testing revealed coexisting 

huntingtin and ATXN8OS triplet repeat expansions. 

We discuss phenotype-genotype correlation and the unusually 

variable phenotypes of spinocerebellar ataxia 8. 

S211. Clinical Differences among PD-MCI Subtypes 

Jennifer G. Goldman, Glenn T. Stebbins, Bryan Bernard and 

Christopher G. Goetz; Chicago, IL 

Objective: To examine PD-MCI subtypes. 

Background: Mild cognitive impairment in PD (PD- 

MCI) may represent a pre-dementia state. MCI subtypes distinguish 

between amnestic (aMCI) and nonamnestic (naMCI) 

phenotypes and single (SD) or multiple-domain (MD) 

impairment. Whether PD-MCI subtypes differ in characteristics, 

progression or neuropathology remains unknown. 

Methods: 96 PD-MCI patients (not demented but with zscore 

of < 1.5 on at least 1 of 5 cognitive domains) were classified 

as: aMCI-SD, naMCI-SD, aMCI-MD, and naMCI-MD. 

Results: Group demographics included: age 71.8 (9.2), 

education 14.5 (2.9), PD duration 6.9 (5.2) years; MMSE 

26.9 (2.1); UPDRS motor score 31.6 (11.4). In our sample, 

25% had aMCI-SD, 44% naMCI-SD, 21% aMCI-MD, 

and 10% naMCI-MD. Subtypes differed significantly 

regarding age, PD onset age, MMSE, and H&Y stage. Post- 

35 

hoc analyses revealed older PD onset age in aMCI-SD, 

worse MMSE scores in naMCI-MD, and worse H&Y stage 

in naMCI-MD. Mean UPDRS motor scores were higher in 

both MD subtypes. Axial function differed among groups 

with worse scores in naMCI-MD. 

Conclusion: Nonamnestic and single domain impairment 

predominated. Multiple-domain PD-MCI had worse motor 

function. Increased burden of cognitive dysfunction (multiple-domain), 

rather than deficit type (amnestic/nonamnestic), 

may be associated with greater motor impairment and 

dementia risk. 

Study supported by: NIH K23060949, Parkinson’s 

Disease Foundation 

S212. Loss of Cortical Gray Matter in Parkinson’s 

Disease 

Suman Sen, Paul J. Eslinger, Daymond Wagner, Guangwei 

Du, Mechelle M. Lewis, Michel L. Shaffer and Xuemei 

Huang; Hershey, PA 

In addition to classic motor dysfunction, Parkinson’s disease 

(PD) patients exhibit cognitive deficits that are related to 

cortical network dysfunction. To investigate PD changes in 

cortical gray matter, high-resolution T1-weighted magnetic 

resonance images were acquired from 40 right-handed PD 

subjects and compared to 40 age-, gender-, education-, and 

handedness-matched healthy controls. Screening cognitive 

measures of Mini-Mental Status Examination (p ¼ 0.32) and 

Montreal Cognitive Assessment (p ¼ 0.81) were also comparable 

between groups. Imaging data were analyzed using 

voxel-based morphometry (VBM). Permutation-based, nonparametric 

testing (5000 permutations) was applied within 

the framework of a general linear model, adjusting for age 

and gender within groups. Group differences were considered 

significant at p


Methods: Analysis is based on 199 PSP cases and 129 

age-gender and geographically matched controls. Trained 

interviewers conducted standardized telephone surveys to 

collect information including demographics, residence history, 

lifetime occupational history and home pesticide use. 

An industrial hygienist and toxicologist blinded to whether 

data were from cases or controls assessed self-reported exposure 

to chemicals and determined if the subjects had direct, 

indirect or no exposure based on occupational histories. 

Odds ratios (OR) and 95% confidence intervals (CI) were 

estimated using univariable and multivariable logistic regression 

adjusting for significant covariates. 

Results: PSP cases were significantly more likely to report 

exposure to occupational pesticides (OR:2.22; CI:1.15-4.37, 

p ¼ 0.015) and organic solvents (OR:1.57; CI:1.05-2.34, p 

¼ 0.026) than controls. Using assigned exposures, cases 

were significantly more likely to have direct exposure to any 

chemical (OR:1.99; CI:1.26-3.16, p ¼ 0.003), and pesticides 

(OR:2.14; CI:1.06-4.61, p ¼ 0.034). 

Conclusions: This is the first epidemiological study to 

find a significant association between occupational chemical 

exposures and PSP. Future studies should determine which 

chemicals are associated with the development of this primary 

tauopathy and whether genetic and environmental risk 

factors interact. 

Study supported by: National Institutes of Aging, 

National Insitutes of Health 

S214. Psychiatric Co-Morbidities and Mortality among 

Hospitalized Parkinson Disease Patients 

Nicte Mejia and Zeina Chemali; Boston, MA 

Affective and anxiety disorders are determinants of poor 

quality of life and survival for Parkinson disease (PD) 

patients. Their impact on in-hospital PD mortality has not 

been examined. 

The study evaluates the effect of depression and anxiety 

on in-hospital PD mortality. 

PD patients with depression or anxiety would have higher 

in-hospital mortality compared to PD patients with no psychiatric 

co-morbidities. Alcohol or drug use would further 

increase mortality. We sampled 1998–2007 HCUP-NIS for 

PD, depression, anxiety, alcohol and drug use were identified 

through ICD-9 codes. All statistical models were 

adjusted for age, gender, and race/ethnicity. 

3,013,346 collected PD discharges-age 77.9 (SD 9.0 

years), 52.8% male, 84% white. Depression identified in 

10.6%; anxiety in 4.7%, an underestimate to population 

predicted prevalence for those disorders in same settings. 

Statistically significant lower mortality was found for PD 

patients with known depression (OR ¼ 0.64; p< 0.001) or 

anxiety (OR ¼ 0.50; p< 0.001). Substance and alcohol use 

were assessed as modifiers. 

Depression and anxiety are under-diagnosed in hospitalized 

PD patients. Contrarily when recognized and appropriately 

treated, depression and anxiety are associated with 

lower in-hospital PD mortality. Alcohol and illicit substances 

impact negatively on PD outcome. 

Study supported by: The authors have no disclosure. Dr. 

Nicte Mejia was awarded 2010 AAN Foundation Clinical 

Research Training Fellowships 

S215. Comparison of Subthalamic (STN) and Pallidal 

(GPi) Deep Brain Stimulation (DBS) on Gait and 

Balance in Patients with Parkinson’s Disease (PD) 

Jyhgong Gabriel Hou, Minn Thant, Aliya Sarwar, Linda 

Fincher, Monthaporn S. Bryant, Farrah Atassi and Eugene C. 

Lai; Houston, TX and Galveston, TX 

36 

Objective: To study if STN-DBS or GPi-DBS has different 

effects on gait and balance in individuals with PD. 

Background: Both STN- and GPi-DBS improve motor 

functions in PD. However, their differential benefits on gait 

and balance are unclear. Comparison of their effects may 

help clinical decisions in choosing the target. 

Method: Composite scores of 5-UPDRS items (arising, posture, 

gait, postural instability, bradykinesia) and 3-item standwalk-sit 

test of the STN-DBS (N ¼ 25) and GPi-DBS (N ¼ 

23) groups were compared. Variables during ‘‘off-on’’ (medication-off, 

stimulation-on), ‘‘off-off’’ and ‘‘on-on’’ were measured. 

Result: During ‘‘off-on’’, the sum of 5-item UPDRS 

improved by 1.96(p


S217. First Neurological Examination Can Predict 

Course of Parkinson’s Disease (PD) 

Ali H. Rajput, Michele L. Rajput and Alex H. Rajput; 

Saskatoon, SK, Canada 

Our previous publications concluded that, when the entire 

clinical course is considered, the tremor dominant (TD), 

mixed (MX), and akinetic/rigid (AR) subtypes of PD have 

different pathology and different outcomes. There are no biological 

markers or early clinical manifestations to distinguish 

between those phenotypes. 

All patients were followed at Movement Disorders Clinic 

Saskatchewan and autopsied between 1972 and 2006. Those 

with Lewy body PD and no co-morbidity to modify the motor 

features were included. Excluded were cases that at baseline 

had 15 years or longer duration of PD motor symptoms. The 

patients were assessed in the state as they attended our clinic. 

We hypothesized that predominant tremor at baseline 

would evolve into lifelong TD, predominant akinesia/rigidity 

into AR and those with equal severity into MX subtypes. 

156 PD cases in the study, included 39 AR, 106 MX, and 

11 TD subtypes. Mean duration of symptoms at baseline was 

4.4 years. 133 (85%) of all – 90% of AR, 88% of MX, and 

55% of TD could be accurately identified at baseline. 

These observations are helpful for patient care, drug trials 

patient selection and for studies of pathophysiology of PD. 

Study supported by: Have received honorarium for lectures 

and meeting participation from Novartis. Have 

received travel support to meeting from Teva. Have been 

involved in Botulinum toxin study sponsored by Allergan. 

Research funding from Curling Classic, Parkinson Society 

Saskatchewan, and Greystone Golf Classic. 

S218. Difficulty with Balance Occurs Early in PD: It 

Isn’t Appreciated Because It’s Not Asked about Nor 

Tested For 

Abraham N. Lieberman, Samea Husain, Naomi Salins and 

Anthony Santiago; Phoenix, AZ 

Objective: Devise a question and tests to assess balance 

early, before patients fall. 

Background: Balance difficulty resulting in falls is a wellrecognized 

feature late in PD. Less well known is that balance 

difficulty occurs early in PD. 

Method: In addition to questions and testing of gait on 

the UPDRS we added a question about balance and 3 simple 

balance tests. We assessed balance and gait in 102 consecutive 

non-demented PD patients. The ANOVA analysis 

was used for continuous variables and a logistic regression 

analysis was used for categorical variables. 

Results: Duration of PD in patients who answered ‘‘yes’’ 

on our balance question was 7.11 (6 4.5) versus 3.95 ( 6 

1.79) yrs in patients who answered ‘‘no.’’ This was significant: 

p< 0.0007. It’s noteworthy that 45% of 62 patients 

with PD for 1- 5 yrs had balance difficulty separate from 

gait difficulty. There were statistically significant differences 

on the tests between patients who complained of balance 

difficulty and those who did not. 

Conclusions: the balance question and tests provide a 

simple way of assessing balance early in PD. 

Study supported by: Self supported 

S219. Poor Dementia Screening with Mattis Dementia 

Rating Scale Cutoffs in Highly Educated Parkinson’s 

Disease Patients 

Travis H. Turner and Vanessa Hinson; Charleston, SC 

Introduction: The Mattis Dementia Rating Scale (DRS) is 

widely used to assess cognition and screen for dementia in 

37 

Parkinson’s disease (PD). In 2008, Llebaria and colleagues 

reported excellent sensitivity and specificity detecting dementia 

in PD using a DRS total score cutoff of 123. However, 

this study used a sample with rather low educational 

achievement. 

Methods: Linear discriminant function analysis was used 

cross-validate the 123 cutoff score in a sample of PD 

patients with rather high educational. 

Results: From a sample of 38 PD patients, 12 were diagnosed 

with dementia following comprehensive neuropsychological 

evaluation. A total score cutoff of 123 misidentified 

9 patients with dementia (25% sensitivity, 100% specificity). 

Age and education corrected scaled scores did not 

improve overall classification. 

Discussion: Results suggest caution in applying DRS-2 

total score cutoffs in highly educated patients, and highlight 

the importance of comprehensive neuropsychological evaluations 

for clinical diagnosis of dementia in PD. 

S220. Impaired Social Problem-Solving in Huntington’s 

Disease 

Charles Van Liew, Jody Goldstein, Shea Gluhm, Jagan Pillai 

and Jody Corey-Bloom; San Diego, CA 

Huntington’s disease (HD) is a genetic neurodegenerative 

disorder characterized by motor, cognitive, and psychiatric 

dysfunction. In HD, the inability to solve problems successfully 

affects not only disease coping, but also interpersonal 

relationships, judgment, and independent living. In the current 

study, we examined social problem-solving (SPS) in 

well-characterized HD and at-risk (AR) subjects using the 

Social Problem-Solving Inventory-Revised:Long (SPSI- 

R:L)—a 52-item, well-validated, standardized measure of 

SPS. Items are aggregated under five subscales (Positive, 

Negative, and Rational Problem-Solving; Impulsivity/ Carelessness; 

and Avoidance). Thirty-five subjects, including 12 

HD (mean age ¼ 53, mean CAG ¼ 44) and 23 gene-positive 

AR (mean age ¼ 41, mean CAG ¼ 42), were compared 

with two-tailed t-tests on SPSI-R:L scores. HD subjects 

scored significantly worse on Total SPS (p


8/18 (44%) subjects developed ICBs. Subjects developing 

ICBs showed a significantly higher rate of progression on 

the Psychoticism (PSY) (p ¼ 0.004), Paranoid Ideation 

(PAR) (P ¼ 0.01), Global Severity Index (GSI)(p ¼ 0.03), 

and Positive Symptom Total (PST) (p ¼ 0.01) subscores. 

Subjects randomized to pramipexole had a significantly 

higher rate of progression on the PAR subscore (p ¼ 0.02). 

Careful monitoring of psychological symptoms after initiation 

of DA therapy may aid in identifying patients at risk 

for developing ICBs. Pramipexole may play a role in the 

manifestation of ICBs by augmenting certain psychological 

symptoms. Clinicians should consider medication effects 

when prescribing DA in patients with underlying or progressive 

psychological distress. 

S222. Balance Difficulty in PD Correlates with Step 

Length and Velocity 



Objective: To determine step length and velocity in PD 

patients. 

Background: PD patients often report balance difficulty 

without falls. 

Method: In 102 consecutive non-demented PD patients, 

we compared patients with reported imbalance without falls 

to patients without imbalance as regards age, PD duration, 

UPDRS/Pull-Test, BNI Balance Scale, Stride Length and 

Velocity. There were no confounding causes of balance or 

gait difficulty upon historical review or physical exam. 

Results: Patients with balance difficulty had a shorter 

stride: 1.72 (60.47) vs 2.16 (60. 27) feet/ step, p


controls. Lower MeDi adherence was associated with higher 

odds for PD after adjustment for all covariates (OR per 

MeDi point ¼ 0.894, 95%CI ¼ 0.800–0.998, p ¼ 0.045). 

Mean age-at-PD-onset was 61.6yrs. Lower MeDi score was 

associated with earlier age-at-PD-onset (b ¼ 1.275, p ¼ 

0.001). 

Conclusions: PD patients adhere less than controls to 

the MeDi. Dietary behavior may relate to PD risk. 

Study supported by: NIH (NS32527 and KL2 

RR024157) and the Parkinson’s Disease Foundation 

S226. Autonomic Dysfunction in Early Parkinson’s 

Disease 

Anna L. Molinari, Fenna T. Phibbs, Lily Wang, Yanna Song 

and P. David Charles; Nashville, TN 

Background: Orthostatic hypotension (OH) and suppressed 

heart rate (HR) response to standing are frequent symptoms 

of autonomic dysfunction (AD) in advanced Parkinson’s 

Disease (PD), but their incidence in early PD is unknown. 

Methods: 30 Hoehn & Yahr Stage II idiopathic PD subjects 

underwent a 7 day medication washout. Seated and 

standing blood pressure (BP) and HR measurements were 

recorded daily. Subjects were divided into 2 groups based 

on medication regimen: Group 1 levodopa only (n ¼ 9), 

Group 2 dopamine agonist monotherapy or in combination 

with levodopa (n ¼ 21). 

Results: HR response to standing improved in all 30 subjects 

during medication washout with an average increase of 

2.9 6 7.7 beats/minute (p ¼ 0.03). No subjects exhibited 

symptomatic OH. On average, systolic BP (SBP) decreased 

by 2.60 mmHg and diastolic BP (DBP) decreased by 3.13 

6 9.26 mmHg on Day 8, but these differences were not 

significant. Subjects in Group 1 experienced a smaller difference 

between seated and standing SBP and DBPs on Day 8, 

but these differences were also not significant. 

Conclusions: In early PD, the normal compensatory 

increase in HR upon standing is diminished while SBP and 

DBP are unaffected. HR response should be considered 

when evaluating AD in early PD. 

Study supported by: The clinical trial from which this case 

is reported is funded by Medtronic, Inc., by Vanderbilt CTSA 

grant 1 UL1 RR024975 from the NCRR-NIH, and by private 

donations. Medtronic representatives did not take part in 

data collection, management, analysis, or interpretation of the 

data or in preparation, review, or approval of the manuscript. 

Dr. Charles has received personal compensation and Vanderbilt 

University has received grants to support research 

from Medtronic in excess of $10,000. Dr. Charles has also 

received funding from Allergan for speaking and consulting 

services as well as research grants. 

S227. Balance Difficulty in PD Correlates with the BNI 

Balance Scale 



Objective: To compare PD patients with and without 

reported balance difficulty by several variables to assess the 

validity of the BNI Balance Scale. 

Background: Balance difficulty is a late complication of 

PD. We devised a questionnaire and tested 3 tasks to calculate 

the BNI Balance Scale. 

Method: In 102 consecutive non-demented PD patients, 

we compared patients with reported balance difficulty without 

falls to patients without imbalance as regards age, PD 

duration,UPDRS/Pull-Test, BNI Balance Scale (one foot 

stance, turning 360 degrees, timed-gait). There were no 

39 

confounding causes of balance or gait difficulty upon historical 

review or physical exam.Results: Patients with reported 

balance difficulty had a longer duration of PD: 7.2 (6 4.6) 

vs 4.4 (6 2.4) yrs, p < 0.008; a worse BNI Balance Score: 

7.9 (6 3.8) vs 2.8 (6 2.3), p 30% difference relative to 

placebo. The interim tolerability analysis was preplanned after 

50 subjects (50%) completed 12 week dose titration phase. 

Results: The 20 mg dose was declared intolerable based 

on the interim tolerability data reviewed by the Data Safety 

Monitoring Board in July 2010. Investigators remained 

blinded to the dose assignment. 

Conclusion: These results support previously reported 

data on the dose dependent tolerability of isradipine in PD. 

Final data analysis and dose choice for the pivotal study will 

be available Fall 2011. 

Study supported by: Michael J. Fox Foundation and 

Northwestern Memorial Foundation Dixon Priority 

Research Initiative Award 

S229. Nigella sativa Oil Controls Astrogliosis and 

Reduces Haloperidol-Induced Deficit in Rats 

Tafheem Malik**, Darakhshan Jabeen Haleem, Shema Husan, 

Shahid Pervez and Tasneem Fatima; Karachi, Sind, Pakistan 

and Karachi, Pakistan 

The neuropathological status of Haloperidol (HP) induced 

Extrapyramidal symptoms (EPS) remains unclear.Evidence 

suggested persistent neuronal alterations in the basal ganglia 

cause EPS. This study evaluate the possible protective effects 

of the Nigella sativa (NS) oil on HP induced neuronal alterations 

and motor symptoms. EPS was monitored in HP 

treated groups and with NS oil alone and with placebo.HP 

treated group displayed (p


astrogliosis with no indication of cell loss and 82% normal 

neuronal densities were observed. We conclude that NS may 

prevent HP induced neuronal degeneration.We believe that 

further preclinical research into the utility of NS may indicate 

its usefulness as a protective agent from irreversible EPS. 

Study supported by: The University of Karachi, Pakistan. 

S230. Cost-Benefit Assessment of Two Forms of 

Botulinum Toxin Type-A in Different Pathologies 

Humberto Juarez, Santamaria Salvador, Leticia Hernandez 

and Enrique Molina; Mexico City, Mexico, Mexico 

Botulinum Toxin Type-A has been successfully used since 

20 years in the treatment of different movement disorder 

pathologies.The comparison with Merz Pharma VR 

’s Botulinum 

Toxin Type A regarding its equivalency has not been 

trustfully demonstrated.The Social Security Hospitals have a 

high captive population for each disease group.In 2008, due 

to administrative restructure, a 150 kD assessment of Botulinum 

Toxin Type-A was initiated.To effectively compare the 

potency, efficacy and safety of the new biologic, the same 

dilution and application standards were maintained. Video 

and photography were taken for all patients with a standard 

procedure.From May 2008 to April 2009 the responses of 4 

types of 150 kD different batches of the toxin were employed.Comparatively, 

patients injected with onabotulinumtoxin 

A from May 2007 to April 2008 were selected.Our surveillance 

regarding potency, maximum time effect, and maximum 

duration was that in all parameters the Botulinum 

Toxin Type A at 150 kD was lower and oscillates at 6 weeks 

in average for all pathologies. 

S231. Weight and Height Distribution in Children with 

Tourette Syndrome 

Katie Kompoliti, Glenn T. Stebbins and Christopher G. Goetz; 

Chicago, IL 

Objective: To compare height and weight of untreated children 

with Tourette syndrome (TS) with age and gender 

matched controls. 

Background: TS children are reported to have lower 

height and weight, with normal body mass index (BMI), 

indicating a possible dopaminergic over-activity. 

Methods: Weight and height of consecutive TS patients 

under 20 years were recorded. Patients were considered 

untreated if they had never taken medications for tics or TS 

co-morbidities. Age and gender standardized weight z-score 

and BMI z-scores were compared between the TS group and 

the CDC normative data from 2000 using one-sample t-test. 

Results: A total of 195 patients (155 males, 40 females), 

mean 11.9years (SD 3.5), were included, with 95 never 

exposed to medications. Untreated patients had higher 

adjusted average weight compared to the CDC sample’s mean 

(p ¼ 0.0005), but no difference in height (p ¼ 0.08). Patients 

with medication exposure had a higher adjusted weight compared 

to those never treated but no difference in height. 

Conclusion: Un-medicated TS children are heavier than 

the general population, arguing against a generalized hyperdopaminergic 

state. Medication treatment increases weight 

further, alerting physicians to monitor weight when treating 

TS children. 

S232. Motor Deterioration after Medication Withdrawal 

in Early Parkinson’s Disease 

Chandler E. Gill**, Anna L. Molinari, Thomas L. Davis, 

Mark Bliton, Stuart G. Finder, Michael G. Tramontana, Peter 

E. Konrad and David Charles; Maywood, IL; Nashville, TN 

and Los Angeles, CA 

40 

Without a biomarker of Parkinson’s disease (PD) progression, 

we rely on clinical ratings to estimate disease progression. 

Medication washout may be useful in these ratings. 

We are conducting a prospective, blinded, pilot trial of deep 

brain stimulation (DBS) in early stage PD subjects aged 50 

to 75, Hoehn & Yahr II, without dyskinesias or motor fluctuations, 

and on PD medications < 4 years. Inpatient medication 

washout and motor rating is conducted at baseline 

and every six months for two years. On Day 1, subjects are 

rated ON medication and then medications are withheld 

and motor function is rated daily. Thirty subjects were 

randomized (15 MED; 15 DBSþMED). Participants are 27 

males and 3 females aged 60.3 6 6.6 years at study entry 

and taking medication an average of 2.1 6 1.5 years. 

UPDRS-III score at baseline Day 1 (ON medication) was 

14.9 6 7.9 which increased by 1.7 points per day to 27.0 

6 7.9 after one week withdrawal. Median ON Hoehn & 

Yahr Score was 2 (Range: 1-2) which remained at 2 after 

one week (Range: 2–2.5). Schwab & England ADL score 

also did not significantly change (Day 1: 92% 6 5%; Day 

8: 89% 6 4%). 

Study supported by: The clinical trial from which this 

case is presented is supported by Vanderbilt CTSA grant 1 

UL1 RR024975 from the National Center for Research 

Resources, National Institutes of Health, by a research 

grant from Medtronic, Inc., and by gifts from private 

donors. 

Vanderbilt University has received support from Allergan 

and Medtronic for research led by Drs. Charles and Medtronic 

for researcfh led by Dr. Konrad. Drs. Konrad and 

Davis have received personal compensation in the past from 

Medtronic for consulting fees or honoraria. Dr. Charles 

receives income from Allergan, Medtronic, and Pfizer for 

education and consulting services. 

S233. Gender Differences in the Interleukin-6 G-174C 

Polymorphism and the Risk of Parkinson’s Disease 

Marta San Luciano, Laurie J. Ozelius, Richard B.. Lipton, 

Deborah Raymond, Kaili M. Stanley, Susan B. Bressman and 

Rachel Saunders-Pullman; New York, NY and Bronx, NY 

Reports of associations between the -174G>C SNP in the 

promoter region of the interleukin-6 (IL6) gene and the 

1730G>A SNP in the estrogen receptor beta (ESR2) and 

PD are conflicting. We investigated the association of both 

SNPs in a sample of 121 unrelated Caucasian Parkinson’s 

disease (PD) cases and 329 control subjects. The G allele of 

the G-174C SNP was more common in the group of men 

with PD when compared to controls (p ¼ 0.022), but not 

among women with PD or in the overall group. In men, 

having the GG genotype increased the risk of PD by over 

two fold (OR ¼ 2.11, 95%CI: 1.14–3.89, p ¼ 0.017). 

Analysis restricted to young-onset PD cases showed no association. 

No differences in allele or genotype frequencies 

were observed between PD patients and controls for the 

ERBeta G-1730A SNP in the overall group or either gender 

separately, or between younger onset PD patients and controls. 

Our data supports a possible inflammatory role in PD 

for IL6, and concurs with a prior report linking the G allele 

with PD, although a gender effect was not found. These 

findings were not replicated in two other samples. The contradictory 

findings may be explained by ethnic differences 

in distribution of IL6 polymorphisms, the existence of other 

polymorphisms in tight linkage disequilibrium with the C/ 

G SNP differentially influencing IL6 expression, methodological 

differences and sample size considerations, and 

inability to capture the complex functional network of 



cytokines and chemokines from single gene association studies. 

Analysis of IL6 SNPs in larger and more diverse populations 

and sequencing of surrounding regions is recommended 

to further assess a role for this cytokine in PD. 

Study supported by: Dr. San Luciano is supported by an 

American Academy of Neurology Foundation Clinical 

Research Training Fellowship. Dr. Saunders-Pullman is supported 

by the Pfizer Society for Women’s Health Research 

Scholar Grant for Faculty Development in Women’s Health. 

This study was supported by Grant Number K23NS047256 

from the National Institute of Neurological Disorders and 

Stroke (RSP), the Michael J. Fox Foundation (RSP, SBB), 

the Thomas Hartman Foundation (RSP), and The Einstein 

Aging Study is funded by the National Institute on Aging 

(AG03949, Principal Investigator: R.B. Lipton). This publication 

was made possible by the CTSA Grant UL1 

RR025750 and KL2 RR025749 and TL1 RR025748 from 

the National Center for Research Resources (NCRR), a 

component of the National Institutes of Health (NIH), and 

NIH Roadmap for Medical Research. 

S234. Serum Cholesterol Is Linked with Nigrostriatal 

Iron Deposition in Parkinson’s Disease 

Guangwei Du, Mechelle M. Lewis, Michele L. Shaffer, 

Honglei Chen, Richard B. Mailman and Xuemei Huang; 

Hershey, PA and Research Triangle Park, NC 

Higher nigrostriatal iron content has been related to Parkinson’s 

disease (PD). Higher serum cholesterol levels also have 

been suggested to be associated with lower occurrence of PD. 

To understand the relationship between serum cholesterol and 

nigrostriatal iron content, 3T MRI (T1-, T2-weighted, T2*) 

were obtained from 40 PD and 29 matched Controls. Fasting 

serum lipid profiles were measured in all subjects. The mean 

R2* values of bilateral substantia nigra (SN), caudate, putamen, 

globus pallidus (GP), and red nucleus (RN) were calculated, 

and correlated with serum cholesterol levels after controlling 

for age, gender, and statin usage. In PD, higher serum 

cholesterol levels were associated with lower iron content in 

SN (R ¼ 0.337, and p ¼ 0.048), striatum (R ¼ 0.403, p 

¼ 0.017 for caudate; R ¼ 0.366, p ¼ 0.031 for putamen), 

and RN (R ¼ 0.436, p ¼ 0.009), but not GP (R ¼ 

0.250, p ¼ 0.147). In Controls, higher serum cholesterol 

levels were associated with lower iron content in striatum (R 

¼ 0.449, p ¼ 0.032 for caudate; R ¼ 0.451, p ¼ 0.027 

for putamen), but not associated with the SN (R ¼ 0.096, p 

¼ 0.654), RN (R ¼ 0.136, p ¼ 0.526), or GP (R ¼ 

0.191, p ¼ 0.371) iron levels. Thus, higher serum cholesterol 

concentrations are associated with lower iron content in 

nigrostriatal structures, warranting further studies. 

Study supported by: NS060722, and the HMC GCRC 

(NIH M01RR10732) and GCRC Construction Grant 

(C06RR016499) 

S235. Balance Difficulty Differs from Gait Difficulty in 

PD 



Objective: To compare reported balance difficulty from gait 

difficulty in PD. 

Background: Patients with PD often report imbalance 

without gait challenges. 

Method: In 102 consecutive non- demented PD patients, 

we compared 13 patients with reported imbalance without 

gait impairment with 22 patients with isolated gait difficulty 

as regards age, PD duration, UPDRS/Pull-Test, BNI Bal- 

41 

ance Scale. There were no confounding causes of balance or 

gait difficulty upon historical review or physical exam. 

Results: Patients with reported imbalance without gait 

challenges demonstrated postural instability to Pull-Test: odds 

ratio 6.00 (95% CL 1.331, 27.047), and one foot stance 

instability: odds ratio 5.440 (95% CL 1.217, 24, 321) compared 

with patients reporting isolated gait difficulty. 

Conclusions: Most patients with PD report a balance 

difficulty with a co-morbid gait challenge. We believe there 

is a subset of patients with subjectively perceived and objectively 

measurable impaired balance without noticeable gait 

challenges that may reflect a distinct circuitry aberration 

impacting balance separate from locomotion. 

S236. Adult Onset Dopamine Responsive Dystonia 

(DRD): Is There a New Gene? 

Hossein Ansari, Ludwig Gutmann and Laurie Gutmann; 

Morgantown, WV 

Background: DRD is a type of inherited dystonia typically 

presenting within the first decade of life. Various genes have 

been identified for this condition. 

Late-onset DRD is very rare and its gene has yet to be 

identified. 

Case Report: 29 y/o female with severe entire-body and 

facial dystonic movements. This was associated with occasional 

myoclonus. Mental status significantly decreased over 

the course of the patient’s nine-week hospitalization.Trials 

with different agents (including dopamine depletors) failed. 

Upon administration of Levopoda, however, the patient 

dramatically improved. After 2 doses, her mental status 

completely recovered and abnormal movements significantly 

decreased. With continued medication, patient normalized. 

Both the patient’s twin and one older sister have a history 

of similar movements. 

Conclusions: The rarity of late-onset DRD contributes 

to its misdiagnosis. Therefore, adult-onset DRD is probably 

an under-diagnosed condition with no known gene. We 

speculate this patient’s sisters also suffer from DRD. 

This family could be a prime case for genetic study used 

to identify the potential gene/mutation causing adult DRD. 

They are set to participate in a study by the NIH 

Undiagnosed Diseases Program. 

S237. Dystonia Induced Mechanical Stress as a Cause of 

DBS Extension Fracture and Expulsion 

Massimo Mondani, Elisa Petacchi, Roberto Eleopra, Silvia 

Molteni, Sabrina Gualdi, Miran Skrap and Andrea 

Martinuzzi; Udine, UD, Italy; Conegliano, TV, Italy and 

Sesto San Giovanni, MI, Italy 

The mechanical failure of the device, especially the most 

exposed and longest part connecting the electrode to the 

impulse generator (IPG) is a possible adverse eventa in DBS. 

We describe a 12 year old child in whom the successful 

treatment with GPi DBS for secondary generalized dystonia 

repeatedly failed for malfunction of the IPG-electrode connection 

including one internal fracture diagnosed by electrophysiology, 

and two aseptic scar breakage leading to expulsion 

of a 5 cm long stretch of the left extension and 

removal of the entire left implant. 

The study of the pattern of child’s movements and of the 

extension position along the left mastoid and neck revealed 

a region of mechanical stress on the left extension which 

was eluded by the right one thanks to its obliquity in 

respect to the axis of the prevalent neck movements. 

When implanting dystonia patients with DBS, especially 

when they are children and present neck dyskinesias, one 



should avoid positioning the IPG extension parallel to the 

major axis of the prevalent movement. A slight obliquity of 

the extension may suffice in preventing failures leading to 

suspension of an otherwise efficacious treatment. 

Sleep Disorders and Circadian Rhythm 

S301. Endogenous GABAA Receptor Enhancement 

Modulates Vigilance in the Primary Hypersomnias 

David B. Rye, Kathy Parker, Lynn Marie Trotti, Paul S. 

Garcia, James C. Ritchie, Michael J. Owens, Leslie Morrow, 

Donald L. Bliwise and Andrew Jenkins; Atlanta, GA; 

Rochester, NY and Chapel Hill, NC 

Primary hypersomnias lack an etiology or rational treatments. 

We investigated biological and neurobehavioral markers in 31 

such cases. Plasma and CSF assays ruled out sedative-hypnotic 

use, neurosteroid or gamma-aminobutryic acid (GABA) 

excess, pathological amino acid profiles, and hypocretin-1 deficiency. 

We tested for GABA-ergic activity employing ligand 

binding assays and electrophysiological recordings of cells 

expressing recombinant GABAA receptors. CSF from 31 

hypersomnolent and 16 control patients all enhanced a1b2c2s 

GABAA receptors. The magnitude of enhancement in hypersomnolent 

subjects exceeded that of controls (89% 6 46.5 vs 

41.4% 6 5.7, t ¼ 5.39, P < 0.001). Pharmacological profiling 

suggested that enhancement was due to an allosteric modulator 

unique to affected versus control CSF. Enhancement 

did not require a functional BZD binding site, was selective 

for a 2 > a1 receptors and negligible at a 4receptors,and 

was reversible with benzodiazepine antagonist flumazenil. Flumazenil 

normalized vigilance in seven index cases. Inhibitory 

GABAA receptor enhancement occurs by way of a positive allosteric 

modulator unique to patients with a primary hypersomnia. 

This altered biology is antagonized in vitro and in 

vivo by flumazenil and identifies a novel pathophysiology to 

primary hypersomnolence. 

Study supported by: Woodruff Health Sciences Center 

Fund and USPHS grants NS055015 and NS055015-03S1 

(D.B.R), NS-050595 (D.L.B), and GM073959 (A.J). 

S302. Frequency of Parsaomnias in Patients with 

Non-Epileptic Seizures 

Mitchell G. Miglis, Michael Boffa, Sujata Thawani, Alcibiades 

Rodriguez and Anuradha Singh; New York, NY 

Objective: To assess frequency of parasomnias in patients 

with Psychogenic Non-Epileptic Seizures (PNES) 

Background: PNES have been associated with a history 

of psychosocial stresssors, an association that has also been 

suggested for some parasomnias. There is little data on frequency 

of parasomnias in patients with PNES. 

Design/Methods: We selected a cohort of patients (n ¼ 

9) with vEEG-confirmed PNES from our Epilepsy Unit 

and administered sleep questionnaires (Epworth, Munich 

Parasomnia Scale) on follow-up visits and phone interviews. 

An age-matched group of patients with vEEG-confirmed 

epilepsy (n ¼ 9) were interviewed for comparison. Participants 

were scored on responses relating to twenty-one parasomnias. 

Responses of PNES and epilepsy patient were 

compared (Chi square analysis, SPSS v16). 

Results: PNES patients reported a higher frequency of 

NREM parasomnias when compared to epilepsy patients, 

notably hypnic jerks (77.8% vs. 11.1%, p ¼ 0.004), rhythmic 

foot movements (55.6% vs. 0%, p ¼ 0.023), exploding 

head syndrome (44.4% vs. 0% p ¼ 0.023), and bruxism 

(66.7% vs. 0%, p ¼ 0.003). 

42 

Conclusions: Patients with PNES in our study population 

reported a higher frequency of NREM parasomnias 

compared to epilepsy patients, and a much higher frequency 

compared to general prevalence estimates. Parasomnias 

should always be considered in the differential of paroxysmal 

nocturnal events in these patients. 

Study supported by: Self Supported 

S303. Endothelial Function in Patients with Obstructive 

Sleep Apnea 

Kanika Bagai, James Muldowney, Yanna Song, Lily Wang, 

Douglas E. Vaughan and Beth A. Malow; Nashville, TN and 

Chicago, IL 

Background: Obstructive sleep apnea (OSA) is implicated in the 

pathogenesis of stroke, although mechanisms need clarification. 

Methods: Severity of OSA [apnea-hypopnea index (AHI) 

of 5 or greater] was defined by overnight polysomnography. 

Endothelial function testing was done using flow mediated 

dilatation and computerized arterial pulse waveform analysis 

in 17 patients with OSA and 17 normal controls. 

Results: The mean AHI (6 standard deviation) in the 

OSA group was 18.68(25.48) and in the non-OSA group was 

0.84 (0.80). Using a linear regression model, with AHI as the 

main effect, and age as covariates, there was a trend towards 

greater mean velocity change with higher AHIs (p ¼ 0.06). 

Conclusions: The severity of OSA selectively affects the 

percent velocity change after reactive hyperemia as measured 

by flow mediated dilation. Consistent with prior studies with 

altered endothelial function in OSA patients, our results show 

a trend towards significant difference in the markers of endothelial 

function as measured by brachial artery flow- mediated 

dilatation testing. One reason for the less dramatic difference 

noted in our study may be because we included patients with 

mild OSA, as compared with other studies which included 

patients with moderate to severe OSA. 

Study supported by: Supported in part by Vanderbilt 

CTSA grant 1 UL1 RR024975 from NCRR/NIH. 

Education 

S401. The Effectiveness of Education Intervention on 

Health Knowledge among Neurological Patients 

Mercedes Jacobson and Polina Pomerants; Philadelphia, PA 

Objective: To assess the impact of education intervention 

on patient’s health literacy in outpatient neurology 

setting. 

Background: Inadequate health knowledge is a strong indicator 

for higher risk of non-compliance, poor self-management, 

and negative health outcomes. 

Methods: A single-blind randomized study was conducted. 

Designed using Ask Me 3 principles, a questionnaire was 

administered to study group before and a week after onsite 

education intervention and to demographically matched control 

group a week after intervention. Performance was scored 

on consistency between subjects’ responses and congruence 

with information from subjects’ medical charts. 

Results: Control and study group population (N ¼ 118) 

consisted of unemployed (84%) middle-aged adults (44.2 

þ/ 12.4) who are legally disabled (66%) and lack postsecondary 

education (82%). Study subjects retained more 

health information (0.19þ/ 0.08, p


Conclusions: Statistically significant improvement in 

health knowledge resulted from education intervention using 

Ask Me 3 tool. Patient education with attention to health 

literacy should be employed in neurology teaching clinics to 

improve patients’ adherence to treatment and comprehension 

of health information. 

S402. Teleneurology in Leading U.S. Medical 

Institutions 

Benjamin P. George**, Nicholas J. Scoglio, Jason I. Reminick, 

Kevin M. Biglan and E. Ray Dorsey; Rochester, NY and 

Baltimore, MD 

Objective: Telemedicine is used for the care of neurological 

conditions yet little is known about the details of telemedicine 

use in neurology departments. The study purpose is to 

determine current teleneurology opinions and practices 

among neurologists at leading U.S. medical institutions. 

Methods: A survey on teleneurology was performed 

among neurologists at the top 50 hospitals in neurology 

based on U.S. News and World Report. The survey was 

sent to department chairs, faculty involved in teleneurology, 

or department administrators. Respondents indicated level 

of telemedicine use by department, current applications, and 

opinions of telemedicine. 

Results: The initial response rate was 32% (16 institutions, 

18 responses). 50% of respondents were neurology 

faculty, the remaining were department chairs (33%) and 

department administrators (17%). 69% of institutions provide 

telemedicine; of those, all use telemedicine for stroke, 

18% for movement disorders, and 9% for neuroimmunology. 

45% of institutions using telemedicine services initiated 

services within the last year, and 60% of institutions not 

providing telemedicine have plans to within a year. Additionally, 

61% of respondents find telemedicine to be equal 

to the in-person care model. 

Conclusions: There is increasing use and acceptance of 

teleneurology by neurologists at leading U.S. medical 

institutions. 

S403. Standardized Sign-Out Improves Communication 

Skills 

Brian D. Moseley, Jonathan H. Smith, Gloria E. Diaz- 

Medina, Mateo Paz Soldan, Meredith Wicklund, Radhika 

Dhamija, Haatem Reda, Michael F. Presti and Jeffrey W. 

Britton; Rochester, MN 

Objective: Unstructured handoffs are vulnerable to communication 

failures. We implemented standardized sign-out on 

our inpatient Neurology services and assessed its effect on 

communication skills. 

Methods: Residents spent the first half of their rotations 

utilizing unstructured sign-out. They switched to a structured 

sign-out system (using the Situation-Background- 

Assessment-Recommendation format) during the second 

half. We analyzed survey responses before and after implementation 

to evaluate for an effect. 

Results: We documented improvements in many variables, 

including being told: pertinent past medical history 

(14/33, 42% versus 14/20, 70%, p ¼ 0.088); pending laboratory 

studies/tests (26/33, 79% versus 19/20, 95%, p ¼ 

0.234); recommendations for how to handle nursing/pharmacy 

calls (12/33, 36% versus 12/20, 60%, p ¼ 0.154); 

and up-to-date code status (14/33, 42% versus 14/20, 70%, 

p ¼ 0.088). Residents utilizing structured sign-out were significantly 

more likely to share test results with patients/family 

prior to shift changes (22/33, 67% versus 18/20, 90%, p 

¼ 0.037) and update our electronic service list (13/33, 39% 

43 

versus 15/20, 75%, p ¼ 0.045). The percentage who felt all 

important data were being transmitted increased from 49% 

(16/33) to 80% (16/20, p ¼ 0.041). Overall satisfaction 

(scale 1–10) increased from 6.2þ/ 1.6 to 7.4þ/ 1.3 (p ¼ 

0.002). 

Interpretation: Structured sign-out results in improved 

communication skills, and may improve patient safety and 

quality of care. 

S404. Improved Scores on the AAN Resident Inservice 

Training Examination (RITE) after Lecture Curriculum 

Intervention 

L. John Greenfield, Vicki Ramsey-Williams, Theresa Marshall 

and Boyd Koffmann; Toledo, OH and Little Rock, AR 

Objective: Determine whether curriculum intervention has 

a positive impact on resident education as measured by 

RITE scores. 

Background: Performance on the RITE predicted scores 

on the ABPN Part I board examination, and has been used to 

assess readiness to take ABPN Part I (Goodman et al. 2002). 

Methods: Retrospective analysis of RITE and USMLE 

scores for 3 years preceding and following curriculum intervention, 

which consisted of realigning the didactic syllabus 

with the subject areas of the ABPN (Neurology) exam, and 

pre-and post-lecture quizzes. 

Results: All neurology residents at the Medical College of 

Ohio/University of Toledo between 2003 and 2010 were 

included. The mean percent of correct answers on the RITE 

significantly improved from prior to 2007 (55.561.4, n ¼ 

30) to 2007 and after (61.361.6, n ¼ 27, p


S406. Adapting a Teaching Hospital Inpatient Neurology 

Service to New Duty Hour Requirements: The 

Washington University Adult Neurology Experience 

Robert Bucelli and Barbara J. Snider; Saint Louis, MO 

New duty hour regulations for ACGME accredited residency 

training programs will go into effect July 1, 2011. 

The complexity of neurological disorders makes balancing 

duty hours with educational and patient care goals particularly 

challenging for inpatient neurology services at teaching 

hospitals. The Washington University/Barnes Jewish Hospital 

(WU/BJH) Adult Neurology residency convened a workgroup 

of residents and faculty to create a rotation system 

designed to ease compliance with the proposed duty hour 

rules. This system was instituted July 1, 2010. We used a 

combination of local and national surveys of residents, 

patient satisfaction scores, and hospital quality data to assess 

whether this change altered duty hour compliance, resident 

satisfaction, patient satisfaction or patient outcomes. Our 

experience demonstrates the inherent difficulties in monitoring 

the effects of duty-hour driven changes in resident 

schedules and suggests that these changes may have unexpected 

negative effects on other aspects of residency education 

and patient care. Future adjustments will require resident 

feedback and patient care metrics to best balance 

resident education/quality of life and patient care responsibilities 

while maintaining compliance with program 

requirements. 

S407. Translational Research in Neuro-AIDS and Mental 

Health 

Amanda Brown, Valerie Wojna, Bruce Shiramizu, Avindra 

Nath and Justin C. McArthur; Baltimore, MD; San Juan, PR 

and Honolulu, HI 

HIV infection of the CNS frequently results in neuropsychiatric 

complications and is a major cause of disability. Despite 

effective antiretroviral treatment, nearly 80% of 

patients have asymptomatic cognitive impairment. HIV disproportionately 

affects racial/ethnic minorities and several 

barriers must be overcome for a significant impact on this 

illness to be realized. A better understanding of how social, 

cultural and genetics impact the pathophysiology of disease 

and the development of new therapies is needed. Another 

barrier is the dearth of scientists from racial/ethnic minority 

groups in the field of Neuro-AIDS research. The R25 program 

was designed to address these barriers by 1) supporting 

the education and training of minority graduate students, 

fellows and junior faculty and individuals who are 

not members of minority groups but are engaged in Neuro- 

AIDS disparity related research through a web-based didactic 

course; 2) to promote innovative Neuro-AIDS research, 

through pilot grants and 3) to provide long-term mentoring 

relationships to further career development. Since program 

inception 12 university partnerships have formed, 127 trainees 

have completed the course, 8 trainees were research 

scholars and/or received pilot grants, 11 independent grants 

were obtained by trainees, and more than 50 manuscripts 

have been published. 

Study supported by: 5R25MH080661, The National 

Institutes of Mental Health 

S408. Relationship between Medical Student Feedback 

and Grading 

and James M. Stankiewicz; Boston, MA 

It is common for students to be asked for feedback in order 

to foster clerkship improvement. It is less clear whether 

medical student feedback is ultimately related to perform- 

44 

ance on the clerkship and grade. Students were surveyed via 

zoomerang after completing their clerkship. Grades had not 

been assigned before evaluation, though students had 

received feedback from attendings and the clerkship director 

about floor performance. Attitudes towards night call, specific 

floor attendings, lecturers, and a clinical examination 

were numerically and qualitatively assessed. Histograms will 

be presented. Spearman rank correlations will be performed 

to relate student feedback ratings with their floor performance 

scores, clinical examination score, shelf performance, 

and overall grade. A Wilcoxon analysis will compare students 

assigning either extreme high/low feedback ratings and 

grades, shelf score, and clinical examination performance. 

Forty-six evaluations have to date been collected. Collection 

will continue until shortly before the meeting at which 

point the data will be analyzed. 









winner. 


M601. Behaviorally-Driven Anatomical Mapping of 

Hemispatial Neglect 

Alex R. Carter, Mark P. Mcavoy, Serguei V. Astafiev, Jennifer 

Rengachary, Daniel L.W. Pope, Abraham Z. Snyder, Kristi 

Zinn, Nick Metcalf, Gordon L. Shulman and Maurizio 

Corbetta; Saint Louis, MO 

Objective: A wide range of lesions have been associated 

with hemispatial neglect. Some heterogeneity may be attributable 

to the use of varying tests. We used a single computerized 

test with high sensitivity for deficits in both topdown 

control of attention and reorientation to salient stimuli 

to predict which brain regions are more likely damaged 

in subjects with those deficits after right hemispheric stroke. 

Methods: Reaction times and accuracy were measured in 

61 right hemisphere stroke patients using a computerized 

Posner reaction time task. Stroke lesions were quantified by 

MRI. Behavioral scores and lesion data were entered into a 

novel voxel-wise logistic regression analysis. 

Results: Two distinct patterns were observed. Impaired 

target detection in the contralesional visual field predicted 

damage to the subcortical paraventricular white matter 

underlying the middle frontal and inferior frontal gyri. 

However, impaired shifting of attention predicted damage 

to more cortical regions between the inferior parietal lobule 

and the temporo-parietal junction complex. 

Interpretation: Lesions of long-range fronto-parietal 

white matter tracts may be associated with deficits in the 

maintenance of spatial representations; in contrast ventral 

parietal lesions may be associated with deficits in phasic 

reorientation of attention. 

Study supported by: This study was supported by the 

National Institute of Mental Health [R01 HD061117-05A2 

to M.C.; 1K08NS064365-01A1 to A.R.C.], the Robert 



Wood Johnson Foundation Amos Medical Faculty Development 

Program [65592 to A.R.C.]. 

M602. Markers of Celiac Disease and Gluten Sensitivity 

in Patients with Cerebellar Ataxia 

Caroline Tan, Peter H. Green, Khalaf O. Bushara, Donald D. 

Kasarda, Ejaz Shamim, Norman Latov, Mark Hallett and 

Armin Alaedini; New York, NY; Minneapolis, MN; Albany, 

CA and Bethesda, MD 

Celiac disease is an autoimmune enteropathy resulting from 

sensitivity to wheat gliadin and related cereal proteins. A 

link between celiac disease and cerebellar ataxia has been 

postulated, based primarily on reports of increased anti-gliadin 

antibodies among patients with ataxia. Serum specimens 

from 21 patients with cerebellar ataxia and elevated antigliadin 

antibody titer, 20 celiac disease patients, and 20 

healthy subjects were assessed for antibodies to deamidated 

gliadin peptides and transglutaminase 2 (TG2) by ELISA. 

The anti-gluten antibody response was further characterized 

through examination of reactivity towards chromatographically 

separated gliadin and glutenin protein fractions by immunoblotting. 

In contrast to the celiac disease group, there 

was not a significant association between the anti-gliadin 

immune response and anti-TG2 or anti-deamidated gliadin 

antibodies in the ataxia group. Characterization of antibody 

specificity revealed differential reactivity towards specific 

gluten protein fractions between ataxia and celiac disease 

patients. The findings indicate that the mechanism and profile 

of the elevated antibody response to gluten in patients 

with cerebellar ataxia is significantly different from those in 

celiac disease patients. 

M603. Early Signs of Cognitive Impairment among 

Multiple Sclerosis Patients with Clinically Isolated 

Syndrome 

Theodora Panou, Vasileios Mastorodemos, Efrosyni Papadaki, 

Panagiotis G. Simos and Andreas Plaitakis; Heraklion, Greece 

and Rethymnon, Greece 

The study investigates primary and secondary verbal memory 

and motor/executive functions (response inhibition and 

strategy shifting ability) in multiple sclerosis (MS) patients 

with clinically isolated syndrome (CIS). We studied 44 CIS 

patients and compared them to 49 patients with relapsing 

remitting MS (RR-MS) displaying mild disability and to a 

large cohort of age- and education level-matched healthy 

volunteers. Results showed that both CIS and RR-MS 

patients evidenced a disproportionate impairment in the immediate 

and delayed recall of the second (as compared to 

the first) of two short narratives of the Logical Memory 

WMS-III subtest, and reduced performance on the Memory 

for Digits-Forward. Performance of either group on the executive 

tasks was not impaired, showing evidence of a 

reversed speed-accuracy trade-off. Illness duration emerged 

as a significant predictor of memory and executive task performance. 

Clinical, psychoemotional, and brain imaging 

findings were also examined as potential correlates of cognitive 

deficits and disease progression among CIS patients. 

These findings may signify early-onset decline of specific 

cognitive functions in CIS, which merits regular follow-up 

assessments and monitoring of psychoemotional adaptation 

and everyday functioning. 

Study supported by: This Research Project was partially 

supported by the Association for Research and Treatment of 

Neurologic Disorders of Crete (‘‘EY ZHN’’), which is philanthropic 

(non-profit) organization that supports the clini- 

45 

cal and research activities of the Neurology Department of 

the University Hospital of Crete. 

M604. Motor Chunking Is Correlated with 

Sensorimotor Cortex and Striatum Activation 

Nicholas Wymbs and Scott T. Grafton; Santa Barbara, CA 

Motor chunking facilitates the production of everyday 

movements by combining discrete motor elements into 

well-integrated units of behavior. We hypothesized that sensorimotor 

cortex and corresponding basal ganglia projection 

areas are essential for chunking. Healthy participants learned 

a motor sequencing task during fMRI scanning. Instructed 

to respond as quickly as possible, participants translated 

sequences from a pseudo-musical tablature displaying a series 

of twelve ‘notes’ on a four-lined staff to one of four 

response keys. Using multislice graph analysis, an unbiased 

estimator identified chunks on a trial-by-trial basis for a set 

of three extensively trained sequences (189 trials/sequence) 

performed over the course of three scanning sessions. A 

‘chunking score’ based on the element-to-element variance 

within each chunk was used to determine the strength of 

overall chunking for each trial and then entered as a covariate 

in the estimation of BOLD. Consistent with our predictions, 

we found activity in contralateral sensorimotor cortex 

and posterior putamen correlated with chunking strength, 

independent of movement speed or reaction time. Disruption 

of this circuit may result in deficits of concatenating 

complex serial actions into contiguous behavior and explain 

some symptoms of subcortical apraxia or Parkinson disease. 

Study supported by: Supported by PHS grant NS44393 

and contract no. W911NF-09-D-0001 from the U. S. 

Army Research Office. 

M605. Areas of Ischemia Associated with ‘‘Frontal Lobe’’ 

Task Failure 

Yessenia Gomez and Argye E. Hillis; Baltimore, MD 

Hypothesis: Measures of ‘‘frontal lobe function’’ evaluate 

broad functional networks including left frontal, temporal, 

and parietal cortex. 

Methods: We identified acute areas of ischemia associated 

with impaired performance on Trail Making, Phonemic 

Word Fluency (word generation in 1 minute), and word 

span, in 95 patients with acute left hemisphere ischemic 

stroke within 48 hours of onset. DWI and PWI were evaluated 

for infarct or hypoperfusion in 14 Brodmann areas 

(4,6,10,11,44, 45,21,22,38,37,39,40,18,19). ANOVA was 

used to identify areas where ischemia was associated with 

mean decrement in performance. 

Results: Patients with ischemia (DWI or PWI abnormality) 

in BA 6, 44, 45 (posterior frontal), 21, 22, 37 (temporal), 

39 or 40 (inferior parietal) had significantly impaired 

performance on word span (p ¼ .03-.001). Patients with ischemia 

in BA 4, 6, 21, 22, 37 and 39 had impaired performance 

on word fluency. Only patients with ischemia in 

BA 37 had impaired Trail Making, but many could not complete 

the task. Lowest word span scores were associated with 

ischemia in BA 45 (mean 1.0 6 0); lowest word fluency 

were associated with ischemia in BA 21 (mean 4.7 6 3.9). 

Conclusion: Word fluency and span tasks are sensitive 

but not specific for frontal lesions. 

Study supported by: NIH R01 DC05375 

M606. Thalamic Atrophy in Gastric Bypass Patients 

with Cognitive Complaints 

Jonathan Graff-Radford, Jennifer Whitwell, Max R. Trenerry, 

J.E. Ahlskog, Michael D. Jensen, Clifford R. Jack, Jr. and 

Keith A. Josephs; Rochester, MN 



Background: Gastric bypass patients are at risk for developing 

neurological complications, often from nutritional deficiencies. 

We have noticed a series of patients presenting for 

cognitive complaints that developed after gastric bypass, 

without any identifiable etiology. We set out to determine 

whether such patients have any focal brain atrophy that 

could account for the complaints. 

Methods: Retrospective case series to identify patients (n 

¼ 10) with cognitive complaints following gastric bypass 

that had a volumetric MRI. Voxel-based morphometry and 

atlas-based parcellation were used to assess patterns of grey 

matter volume loss in all patients compared to a group of 

10 age and gender-matched controls, and a group of 10 

controls matched by body mass index before surgery. 

Results: Patients underwent gastric bypass at a median 

age of 54. Cognitive complaints began at a median age of 

57. All were taking multi-vitamins with nine receiving vitamin 

B12. Formal neuropsychometric testing revealed only 

minor impairments. No nutritional abnormalities were identified. 

Focal thalamic atrophy was identified in the gastric 

bypass patients when compared to controls. 

Conclusions: Patients with cognitive complaints after 

gastric bypass surgery have focal thalamic brain atrophy that 

could account for the cognitive impairment. 

Study supported by: KAJ is funded by the NIH grant 

R01 DC010367 (PI), the Dana Foundation (PI) and the 

Morris K. Udall PD Research Center of Excellence NIH/ 

NINDS P50 NS40256 (Co-investigator). 

M607. Parietal Lobe Lesions Affect the Generation of 

Antisaccades 

James A. Sharpe, Ping Cheng and Moshe Eizenamn; Toronto, 

ON, Canada 

Antisaccades are directed away from visual targets, requiring 

suppression of reflexive saccades toward a target. Impaired 

antisaccade generation has been attributed to frontal lobe 

damage. The role of the parietal lobe is not established. We 

studied antisaccade generation by magnetic search coil technique 

in 13 patients (age 45 6 7 years) with unilateral focal 

parietal lobe lesions (9 tumors; 5 vascular lesions), and in 

10 age-matched controls. 

Patients were instructed to make horizontal antisaccades 

away from a 100ms target flashed randomly 10 degrees to 

the right or left of center. The task was to look 10 opposite 

to the target flash. 

The patient group made antisaccades in only 49.7 6 

32.7% of contraversive trials (visual target flash ipsilateral to 

lesions) and 49.6 6 38.4% of ipsiversive trials. In the other 

trials they made reflexive saccade errors toward the target 

flash. The control group made antisaccades in 82.8 6 

13.7% of trials. Eight patients showed subnormal antisaccade 

generation. Their imaged lesions overlapped in parietal 

lobe white matter.Generation of voluntary saccades is 

impaired by parietal lobe lesions. Antisaccades provide a 

means of measuring voluntary saccade function of the parietal 

lobes independent of visual guidance. 

Study supported by: Canadian Institutes of Health 

Research (CIHR) 

M608. Cognitive Ability Correlates of Psychiatric and 

Social Behaviors in Williams Syndrome 

Rowena Ng, Anna Järvinen-Pasley and Ursula Bellugi; San 

Diego, CA 

Williams syndrome (WS) is a genetic disorder characterized 

with cognitive impairment, a gregarious personality and significant 

anxiety. Research on WS indicates that individuals 

46 

with the disorder present a variety of psychiatric symptoms 

including obsessive compulsion, general anxiety, specific 

phobia, and depression. However, it is unknown the extent 

to which these behavioral problems are related to cognitive 

ability or hypersocial behaviors in WS. Thus, this study 

aimed to examine the relationships among cognitive ability 

(Verbal, Performance, Full IQ), psychiatric symptoms, and 

social behaviors (social-emotionality, social approach) in WS 

versus typical development (TD). A cognitive battery 

(WISC, WASI, WAIS), Brief Symptom Inventory and Salk 

Institute Sociability Questionnaire were administered to 41 

WS and 31 TD participants. Results suggest that verbal 

intelligence is predictive of greater psychiatric symptoms 

(e.g., somatization, interpersonal sensitivity, paranoid ideation, 

psychoticism) in WS but not in TD. Interestingly, 

greater reported psychiatric symptoms in WS were associated 

with poorer socio-emotionality (i.e., emotion recognition, 

desire to please) but not with approach behavior, while 

no significant correlations between social and psychiatric 

measures were observed in TD. Possible role of cognition in 

psychiatric ailments and social outcomes of WS will be 

discussed. 

Study supported by: Grant P01-HD033113-13 

M609. Human Brain Mapping at the Single Cellular 

Level: Neuronal and Area Specific Differences in Health 

and Diseases 

Ryan P. Moore, Irisa Mahaparn, Eliezer Masliah and Pavel V. 

Belichenko; La Jolla, CA 

The Human Brain Mapping Project is an ongoing effort to 

characterize neural circuitry at the level of single cells (Belichenko 

and Dahlstrom, 1994). Lucifer yellow microinjection 

and high resolution confocal microscopy imaging were 

employed to reveal disparities in dendritic geometry of von 

Economo neurons (VENs) vs. pyramidal neurons in the cingular 

cortex complemented with bilateral analysis of speech 

areas to find morphological substrates for the speech area 

asymmetry. For the VENs, significant differences were 

revealed in spine density (p


(Inattentive Type) and 24% with ADHD plus additional 

disorder (i.e. sleep apnea; brain dysfunction excluded). Neuropsychological 

tests included Trail Making Tests (A and B) 

and SDMT-W. Self-report measures assessed hyperactivity 

and anxiety (PPCA, PHCA, PBC, PCC). 

Results: Hyperactivity (> 45%) and anxiety (> 50%) 

were frequent with no significant difference between 

ADHD and ADHD plus. We found a significant relationship 

between hyperactivity and anxiety. Individuals who 

reported yes to one or both anxiety items had higher hyperactivity 

scores than those who said no to both items. 

ADDþ performed worse on all neuropsychological tests (p 

< 0.001 for all comparisons). 

Conclusions: Findings indicate ADHD (Inattentive 

Type) and ADHDþ can be differentiated on cognitive 

measures, but not on self-reported symptoms of anxiety and 

hyperactivity as these co-morbid factors are highly prevalent 

in both populations. 

Study supported by: Personally funded and donated time 

M611. The Use of Quetiapine in Agitated Patients with 

Acquired Brain Injury: A Case Control Study 

Sara Piccoli, Gabriella Paparella, Alec Vestri and Andrea 

Martinuzzi; Pieve di Soligo, TV, Italy 

Background: Experimental studies on neurocognitive effects 

of quetiapine showed improvements of cognitive functions 

in psychiatric patients. Patients with Acquired Brain Injury 

(ABI) often present behavioral disturbances (BD) requiring 

drug treatment which might impair responsiveness and cognition. 

The use of quetiapine may provide in these patients 

adequate control of BD without cognitive side effects. 

Methods: 40 consecutive ABI patients (LCF 4–6) attending 

intensive neurorehabilitation were recruited, 20 of 

whom with BD scored by Aggressive Behaviour Scale (ABS) 

requiring medical treatment, and 20 with ABS score


and females did not differ in performance on SDMT and 

Trail Making Tests. 

Conclusions: Gender differences are present across lifespan 

on tasks of information processing (PASAT). Depression 

moderates performance on tasks assessing distractibility 

(Stroop) to a greater degree for females than males. Significant 

gender differences did not occur systematically on 

SDMT and Trail Making Tests, which involve speeded performance 

and whole brain functioning. 

Study supported by: Personally funded 

M615. Hazard Perception in Cognitively Impaired Older 

Drivers 

Nazan Aksan, Monica Lees, John D. Lee, Shaun Vecera and 

Matthew Rizzo; Mountain View, CA; Madison, WI and Iowa 

City, IA 

Aging and neurological impairment reduce situation awareness 

and increase injury risk as in falls and car crashes. Hazard 

perception ability (HPA) is important to driver safety 

and can be indexed by reaction time (RT) to traffic scenarios 

(Horswill & McKenna, 2004; Horswill et al., 2008). 

Declines in HPA correlate with reductions in Useful Field 

of View (UFOV), a measure of spatial area within which 

individuals can detect visual stimuli (Ball & Rebok, 1994). 

We examined whether vehicular warning systems can 

improve RT in HPA paradigms in elderly drivers with and 

without UFOV deficits. 27 of 51 elderly (67-87yrs without 

neurodegenerative disease, min MMSE ¼ 25) were randomly 

assigned to receive auditory/visual warnings in a simulator 

while watching video scenarios and 10 of those had 

deficits in UFOV. Warnings improved RTs slightly 

(300msec) and a large difference of 1sec in RTs were noted 

between those with and without UFOV deficits. Warnings 

did not differentially improve RTs of those with deficits. 

However, they improved sensitivity, d’, of elderly without 

deficits more than those with deficits. Findings imply warning 

systems do not provide uniform benefits to elderly 

drivers. 

Study supported by: NIH R01 HL091917 

M616. Differentiation of Alzheimer’s Disease and 

Depression with Standard Cognitive Measures 

Meredith C. Frederick**, Stefan Sillau, David B. Arciniegas, 

C. Alan Anderson, Katherine L. Howard and Christopher M. 

Filley; Aurora, CO and Denver, CO 

Objective: Alzheimer’s disease (AD) and depression can 

both present with cognitive impairment. To compare the 

neurobiology of these disorders, we assessed patients with 

AD and depression using the Mini-Mental State Examination 

(MMSE) and Frontal Assessment Battery (FAB). 

Methods: Medical records were reviewed of 475 consecutive 

patients seen at a behavioral neurology clinic over 34 

months. From this group, patients with AD (n ¼ 59) and 

depression (n ¼ 44) were selected after receiving a consensus 

diagnosis by three physicians certified in Behavioral 

Neurology & Neuropsychiatry (DBA, CAA, CMF). MMSE 

and FAB data were analyzed using Z-score transformation. 

Results: AD patients performed significantly worse than 

those with depression on both the MMSE (z-scores 3.93 

vs. 0.05, p < 0.0001) and FAB ( 3.1 vs. 1.0, p ¼ 

0.009). Patients with depression scored significantly worse 

on the FAB than the MMSE (p ¼ 0.0003). AD patients 

performed equally poorly on both tests. 

Conclusions: The MMSE and FAB may assist in the differentiation 

of AD and depression. Whereas both measures 

were abnormal in AD, depressed patients had poorer per- 

48 

formance on the FAB compared with the MMSE, consistent 

with frontal-subcortical dysfunction in depression. 

Study supported by: University of Colorado Denver 

School of Medicine. 

M617. Attention Deficit Hyperactivity Disorder in 

Depressed Adults 

Ildefonso Rodríguez Leyva, Rubén Haro SIlva and Ana A. 

Rentería Palomo; San Luis Potosi, San Luis Potosi, Mexico 

Objective: Attention deficit hyperactivity disorder (ADHD) 

is now recognized as a common disorder, as well as in 

children and adults. ADHD has an estimated prevalence of 

3–5% in adults. Evidence points to an increased rate of 

a co-morbidity in adult ADHD patients. The most common 

are disorders of the affective area, including depression. 

We investigated the comorbidity between these two 

conditions. 

Methods: Two questionnaires were applied, the first is 

the Hamilton Depression Scale D and the second is the 

Adult Self-Assessment Scale (EAVA), developed by the 

working group on adult ADHD, which includes the New 

York University Medical Center, Harvard Medical School 

and Massachusetts General Hospital. 

Results: 50 patients, 42 women and 8 men. 74% had 

some level of depression. 18% had criteria for ADHD 

(12% inattentive, 6% impulsive). By correlating the scale of 

Hamilton with the score of EAVA, results that in patients 

with ADHD and depression, impulsive symptom severity 

decreased in proportion to the degree of depression. 

Conclusions: In patients with ADHD and depression, 

the intensity of the symptoms of inattention and not those 

of impulsiveness, are related in proportion to the degree of 

comorbid depression measured by the Hamilton Scale. 

M618. Cognition and EEG Abnormalities in Non- 

Epileptic AD(H)D/LD Patients with and without Anti- 

Epileptic Drug/Stimulant Therapy 

Brittany M. DiVito, Heather A. Koch, Spencer A. Leblang, 

Erik C. Bakken and Drake D. Duane; Scottsdale, AZ and 

Tempe, AZ 

Background: Epileptiform EEG abnormalities of unknown 

but non-epileptic clinical significance are not rare in developmental 

disorders of attention and learning (ADD/LD). 

The fate of these EEG patterns, their correlation with behavioral 

manifestations and effects of AED therapy is 

unknown. 

Methods: Retrospective analysis of 56 non-epileptic 

ADD/LD with an initial Dysrhythmia Grade II or III EEG 

(Mayo Clinic classification) and subsequent EEG later for 

developmental dx, cognitive test profile, noting if stimulant, 

AED, both or none were employed. 

Results: Baseline Abnormal EEG-M38/F18, mean age 10 

5/12 years, Dx:AD(H)D-47, LD-40, both-32 

EEG and cognitive improvement in at least 1 of 5 cognitive 

tests: F/U EEG Nl-50%; F/U EEG Abn-52% 

In both, proof reading and verbal learning most apt to 

improve, rarely computerized attention 

Rx and cognitive improvement (cumulative score 5 

tests): No Rx-11, Stim-20, AED-12, Both-23 

Conclusions: In AD(H)D/LD with Abn EEG: 

-Normalization of EEG has no general correlation with 

improved cognition. When cognition is improved, it is most 

often in verbal learning/memory/proof reading not computerized 

attention tasks. 

-Irrespective of follow up EEG, cognition is most apt to 

improve when both an AED and stimulant are employed. 



M619. Evolution of EEG Abnormalities in Non-Epileptic 

AD(H)D/LD Patients with and without Anti-Epileptic 

Drug/Stimulant Therapy 

Heather A. Koch, Brittany M. DiVito, Spencer A. Leblang, 

Erik C Bakken and Drake D. Duane; Scottsdale, AZ and 

Tempe, AZ 

Background: Epileptiform EEG abnormalities of non-epileptic 

clinical significance are not rare in developmental disorders 

of attention and learning (ADD/LD). The fate of 

these EEG patterns and their evolution over time with or 

without AED therapy is unknown. 

Methods: Retrospective analysis of 56 non-epileptic 

ADD/LD with initial Dysrhythmia Grade II or III EEG 

(Mayo Clinic classification) and subsequent EEG later for 

developmental dx, noting if stimulant, AED, both or none 

were employed. Population was contrasted with 56 control 

ADD/LD with normal EEG. 

Results: Baseline Nl EEG- M38/F18, mean age 10 9/12 

years, Dx: AD(H)D-49, LD-50, Both-43 

Baseline Abn EEG- M38/F18, mean age 10 5/12 years, 

Dx: AD(H)D-47, LD-40, Both-32 

Follow Up EEG: interval mean 3 9/12 years (range 8/12 

to 15 years) 

Nl- 25(45%) No Rx-3, Stim-5, AED-8, Both-9 

Abn- 31(55%) No Rx-9, Stim-11, AED-4, Both-7 

Conclusions: In AD(H)D/LD with abnormal EEG: 

-Diagnosis distribution is similar to normal EEG. 

-Whether on or off AED Rx, EEG abnormalities often 

persist, but less often if AED in use. 

M620. Transection of CA3 Does Not Affect Memory 

in Rats 

Mohamad Z. Koubeissi, Saifur Rashid, Gemma Casadesus, 

Joseph LaManna, Kui Xu, Hans Luders and Dominique 

Durand; Cleveland, OH 

Objective: To investigate the effect of CA3 transections on 

memory in rats. 

Rationale: Longitudinal hippocampal pathways are 

needed for seizure synchronization, and their transection 

may abolish seizures. However, the effect of such transection 

on memory is unknown. 

Methods: Sprague-Dawley Rats (247–285g) were used. 

A stereotactic knife was implanted 4mm ventrally, 3.3mm 

posterior and 4.0mm lateral to bregma, targeting CA3. 

The knife was protruded from its sheath to transect CA3. 

Sham surgery (n ¼ 4), unilateral (n ¼ 5), and bilateral (n 

¼ 5) CA3 transections were made. Novel object recognition 

(NOR) and Morris water maze (MWM) tests were 

started 18 days later. Cut locations were confirmed by cresyl-violet 

staining. 

Results: For MWM, the ratio of the amount of time 

spent in the target quadrant to that spent in the other three 

quadrants showed no difference between groups. For NOR, 

discrimination scores were also not different between controls 

and transected animals. Histology confirmed the locations 

of transections in the CA3 region. 

Conclusion: Normal performance in NOR and MWM 

does not appear to require intact transmission throughout 

the whole length of CA3. 

Significance: Since CA3 transections do not interfere 

with memory function, they may be tried for treatment of 

temporal lobe epilepsy. 

Study supported by: Dr. Durand is supported by the 

National Institute of Health (5R01NS032845-13, 

5R01NS060757-03, and 5R01NS064157-02) and the 

COULTER Foundation 

49 

M621. Cruetzfeldt-Jakob Disease Presenting as a Rapidly 

Progressing Dementia with Non-Convulsive Status 

Epilepticus 

Natasha Tilluckdharry, Megan McGarry and Dipak P. 


Background Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative 

disease caused by the accumulation of misfolded 

prion proteins in the brain. Although rare, it is the 

most common prion body disease. Clinical manifestations 

include rapidly progressive dementia, behavioral changes, 

extrapyramidal signs, akinetic mutism and myoclonus. We 

report a unique case of CJD with nonconvulsive status 

epilepticus. 

Case Report: 73 year old woman was admitted with a 

rapidly progressive dementia of one year. The patient was 

noted to have intermittent jerky movements, decreased 

responsiveness and mutism. Physical examination revealed 

vegetative mental state, whole body stiffness, myoclonic 

jerks and right sided rigidity. Electroenchephalogram (EEG) 

demonstrated generalized periodic,rhythmic and lateralized 

complexes. Magnetic Resonance Imaging (MRI) of the brain 

revealed global cerebral atrophy. CSF evaluation showed 

presence of 14-3-3 protein and extremely high levels of Tau 

protein. The brain biopsy was refused by family. 

Conclusion The clinical triad of rapid progressive dementia, 

myoclonus, akinetic mutism in conjunction with EEG 

and CSF findings are usually conclusive for CJD. Brain biopsy 

is the diagnostic choice with histopathologic findings 

of spongiform changes, cortical neuronal loss and abnormal 

deposition of prion protein. Nonconvulsive status epilepticus 

is usually a rare finding with CJD. 

Epilepsy 

M701. Controlled Cortical Impact in Adult Rats and 

Posttraumatic Seizures and Epilepsy 

Kevin M. Kelly, Elena A. Kharlamov, Eric R. Miller, Bo Lu 

and Zakaria Mtchedlishvili; Pittsburgh, PA and Philadelphia, 

PA 

The CCI model of TBI has been used in mice and immature 

rats to model posttraumatic epilepsy. We used young 

adult rats and long-term video and video-EEG with cortical 

and hippocampal electrodes to investigate posttraumatic epileptogenesis 

and associated neuropathological changes. A 

total of 28,956 hours of monitoring was obtained from 128 

CCI-injured and 15 sham-operated animals. Class 3–5 provoked 

seizures occurred in 7/72 (9.7%) animals video-monitored 

for 1 week immediately after CCI. Epileptic seizures 

occurred in 26/118 (22%) animals monitored beyond 1 

week post-CCI. Class 3–5 seizures occurred in 19 animals; 

ictal discharges appeared generalized at onset or from the 

contralateral frontal cortex. Nonconvulsive seizures occurred 

in 7 animals characterized by motor arrest or no behavioral 

change associated with continuous 1-2 Hz high amplitude 

spikes or spike-waves averaging 26.2 6 2.8 seconds. No 

control animal had seizures. CCI resulted in severe cortical 

and subcortical injury and alterations in NeuN and GFAP 

staining. Timm staining showed mossy fiber sprouting in 

the inner molecular layer of the dentate gyrus of epileptic 

and nonepileptic animals. These results indicate that the 

CCI model can be used in adult animals to investigate 

mechanisms underlying posttraumatic epileptogenesis. 

Study supported by: Pennsylvania Department of Health 

Research Formula Fund RFA 01-07-26 and Epilepsy Foundation 

Research Grant (ZM) 



M702. Serotonin 1A Receptors and Memory in 

Temporal Lobe Epilepsy 

William H. Theodore, Edythe Wiggs, Ashley Martinez, Irene 

Dustin, Omar Khan, Shmuel Appel, Patricia Reeves-Tyer and 

Susumu Sato; Bethesda 

Memory deficits are common in patients with temporal lobe 

epilepsy (TLE). Previous PET studies have shown reduced 

mesial temporal 5HT1A receptor binding. We studied 40 

patients (24 male; mean age 34.5). Seizure diagnosis and 

focus localization were based on ictal Video-Electoencephalographic 

recording. Patients had Weschler Adult Intelligence 

Score III, Weschler Memory Score III, Beck Depression inventory, 

and interictal PET with [18F]FCWAY, a highly specific 

5HT1A ligand. MRI partial volume correction for hippocampal 

atrophy and tracer plasma free fraction (f1) 

measurement were used to obtain [18F]FCWAY volume of 

distribution (V/f1). V/f1 was significantly lower ipsilateral 

than contralateral to the epileptic focus (73.7 þ/ 27.3 versus 

95.4 þ/ 28. We found a significant relation between 

left hippocampal FCWAY V/f1 and delayed auditory memory 

score (r ¼ 0.41; p


increased LTD (long term depression) and cognitive deficits 

at maturity (PND 60þ). These changes occur in the absence 

of pronounced hippocampal injury. We speculate that 

increased LTD expression may be responsible for the 

observed learning abnormalities. LTD induction can be 

mediated by two distinct mechanisms, NMDA receptor 

(NR) dependent and metabotropic glutamate receptor 

(mGluR) dependent. The NR-mediated and mGluR-mediated 

forms are differentiated by the effectiveness of different 

chemical and electrical LTD inducing stimulation paradigms. 

Using these paradigms, we found that alterations in 

LTD following ELS are the result of changes exclusively in 

mGluR-mediated LTD. Furthermore, we found that ELS 

results in abnormal signaling consistent with that mediated 

by the Fragile X Mental Retardation Protein (FMRP) to 

result in increased mGluR-mediated LTD, with similarities 

to that found in genetic disruption of FMRP. Expression of 

proteins associated with mGluR-mediated LTD and FMRP 

signaling were also altered in a consistent fashion, suggesting 

a mechanism for the observed changes in LTD and possible 

targets for therapeutic intervention. 

Study supported by: NIH-NINDS, Epilepsy Foundation, 

The Childrens Hospital Research Institute 

M707. Heterozygous Loss of the Epilepsy-Associated 

GABAA Receptor a1 Subunit Causes Spontaneous EEG 

Spike Discharges in Two Mouse Strains 

Fazal M. Arain and Martin J. Gallagher; Nashville, TN 

Autosomal dominant juvenile myoclonic epilepsy and absence 

epilepsy are associated with missense (A322D) and 

frameshift (S326fs328X) mutations in the GABAA receptor 

a1 subunit. Although both mutations substantially reduce 

a1 subunit protein expression in vitro, previous studies 

failed to detect seizures in heterozygous a1 subunit knockout 

(GABRA1 KO) mice. Here, we performed video-EEG 

studies in wild type and heterozygous GABRA1 KO mice in 

the seizure-resistant C57BL/6 and susceptible DBA/2J 

strains. Consistent with previous results, both strains of wild 

type mice had a low incidence of spike discharges (SD), and 

this baseline incidence was greater in DBA/2J (7 6 4SD/ 

hr) than C57BL/6 (2 6 1 SD/hr) mice. Heterozygous 

GABRA1 KO caused an ethosuximide-sensitive increase in 

SD incidence to 14 6 3 SD/hr in DBA/2J mice and 12 6 

3 SD/hr in C57BL/6 mice. The typical SD duration was 

brief (1–3 seconds) and no definite associated behavior 

changes were observed. Therefore, heterozygous loss of a1 

subunit produces thalamocortical hypersynchrony in vivo 

and likely represents a principle mechanism by which the 

A322D and S326fs328X mutations cause seizures. 

Study supported by: National Institute Of Neurological 

Disorders And Stroke R01NS064286 

M708. Regional Network Disruption in Temporal Lobe 

Epilepsy 

Luigi Maccotta* and Edward Hogan; St. Louis, MO 

Medial temporal cortex forms a network of connections 

with both the contralateral medial temporal lobe and with 

extratemporal brain regions. It is unclear how temporal lobe 

epilepsy (TLE) affects these connections, and whether the 

effect is pathologic/dysfunctional or compensatory. We 

investigated the functional connections of the medial temporal 

region with contralateral temporal and extratemporal 

brain regions in TLE patients using resting state fMRI. 

Twenty-three TLE patients underwent resting-state BOLD 

fMRI. Seizure localization was based on video-EEG. 

Healthy controls served as comparison. Regions of interest 

51 

were defined anatomically a priori and functioned as seeds 

in a functional connectivity analysis. Results indicate that 

TLE exerts a complex effect on the functional connections 

of the medial temporal region. Intrahemispheric coupling 

between the hippocampal head and entorhinal cortex was 

surprisingly stronger in TLE patients (p


TPM exposure was equivalent between USL255 and 

TPM-IR; furthermore, USL255 displayed a lower C max and 

later T max than TPM-IR. While bioavailability of USL255 

was unaffected by food, T max was delayed by 4 hours with 

food as compared with the fasted condition.Single-dose 

USL255 demonstrated equivalent TPM exposure and a lack 

of food effect analogous to TPM-IR dosed twice-daily, suggesting 

that USL255 may provide a once-daily alternative to 

TPM-IR. 

Study supported by: Upsher-Smith Laboratories, Inc. 

All authors are employees of Upsher-Smith Laboratories, 

Inc. (study sponsor) 

M711. Cingulate Epilepsy, Reporting 3 Clinical and 

Electrophysiologic Subtypes with Surgical Outcomes 

Mhd Rafeed Alkawadri, Norman K. So, Paul C. Van Ness 

and Andreas V. Alexopoulos; Cleveland, OH and Dallas, TX 

Objective: To characterize epilepsy arising from the cingulate 

gyrus. 

Design/Methods: We describe 14 cases of lesional cingulate 

gyrus epilepsy treated surgically. The cases were identified 

from the Cleveland clinic and the UTSouthwestern epilepsy 

monitoring unit databases 1992–2009. 

Results: All 14 cases had cingulate epilepsy confirmed by 

MRI lesions and remarkable improvement after surgery. 

They were divided into 3 groups based on the anatomical 

location and the semiology. In the posterior cingulate group 

4 of 4 cases had clinical or electrophysiologic findings suggestive 

of temporal origin of the epilepsy. The anterior cingulate 

cases were divided into a ‘‘typical’’ (BANCAUD, 

1992) group: 6 cases with hypermotor seizures and infrequent 

generalization with the presence of fear, laughter, or 

interictal personality changes; and an ‘‘atypical’’ group: 4 

cases presenting with simple motor seizures and a tendency 

for more frequent generalization and less favorable surgical 

outcome. The pathology of the atypical cases was 

astrocytoma. 

Conclusions: Posterior cingulate gyrus epilepsy presents 

with electroclinical findings that are suggestive of temporal 

lobe epilepsy and can be considered an example of ‘‘pseudotemporal 

epilepsy’’. The electroclinical presentation and surgical 

outcome of lesional anterior cingulate epilepsy is possibly 

influenced by the underlying pathology. 

M712. GABAB Receptor Antagonists Reduce Pro- 

Epileptic Activity in Hippocampal Slices of Ts65Dn 

Mice, a Genetic Model of Down Syndrome 

Alexander M. Kleschevnikov, Brett Rasmuss, Pavel V. 

Belichenko and William Mobley; La Jolla, CA 

Epilepsy is a known co-morbidity of Down syndrome (DS). 

GABAB receptor antagonists have been proposed recently as 

medicines for improving memory in DS. We examined 

effects of GABAB antagonists on pro-epileptic properties in 

Ts65Dn mouse model of DS. In a high-potassium in vitro 

model of epilepsy, elevation of extracellular [Kþ] provoked 

seizures in the dentate gyrus in slices from both Ts65Dn 

and 2N (control) mice. The frequency of seizures was significantly 

greater in Ts65Dn (0.47 6 0.03 Hz vs. 0.33 6 

0.06 Hz, p ¼ 0.013), while the amplitude did not differ (p 

¼ 0.45). GABAB antagonist CGP52432 reduced the amplitude 

of seizures more considerably in Ts65Dn than in 2N 

slices (63.5 6 4.6% vs. 80.2 6 8.1%, p ¼ 0.033). The frequency 

of seizures was not affected (p > 0.4). GABAB 

antagonists reduced also ratio of amplitudes of population 

spikes (PSA2/PSA1) evoked by paired stimuli. This result 

suggests an increase in the feedback inhibitory efficiency fol- 

52 

lowing suppression of the GABAB receptors, an effect that 

may contribute to antiepileptic properties of the drug. 

Thus, GABAB receptor antagonists reduce pro-epileptic activity 

in DS model mice. 

Study supported by: Down syndrome research and treatment 

foundation (DSRTF); Larry L. Hillblom Foundation 

M713. Lacosamide: Long-Term Safety and Efficacy in 

Partial-Onset Seizures 

William Rosenfeld, Nathan B. Fountain, Gintaras Kaubrys, 

Elinor Ben-Menachem, Cindy McShea, Jouko Isojarvi and 

Pamela Doty; St. Louis; Charlottesville; Vilnius, Lithuania; 

Göteborg, Sweden and Raleigh 

Long-term ( 8 years exposure) safety and efficacy of the 

antiepileptic drug (AED) lacosamide were evaluated from a 

completed open-label extension (SP615; NCT00552305) in 

partial-onset seizures. 

Patients enrolled following double-blind or open-label 

lacosamide trials. Dosage adjustment of lacosamide (100- 

800mg/day) and/or concomitant AEDs occurred to optimize 

tolerability and seizure reduction. Treatment-emergent 

adverse events (TEAEs), vital signs, body weight, clinical 

laboratory data, electrocardiograms, and seizure frequency 

from subject diaries were evaluated. 

Of 370 enrolled patients, 77%, 51%, and 39% had >1, 

>3, or >5 years lacosamide exposure, respectively (median 

modal dose 400mg/day). Common TEAEs ( 15%) were 

dizziness, headache, nausea, diplopia, fatigue, upper respiratory 

tract infection, nasopharyngitis, contusion, and coordination 

abnormal. Discontinuations due to TEAEs were 

12.7%; only dizziness and convulsion led to discontinuation 

in 1% of patients. Median 28-day seizure frequency was 

12.0 at Baseline of previous trials; median percent reduction 

from Baseline was 50.8% across Treatment, and was 47.3%, 

56.8% and 65.2%, respectively, for 1-year, 3-year and 5year 

completers. The 50% responder rate was 51.2% 

across Treatment, and was 48.8%, 57.2% and 63.4% for 1year, 

3-year and 5-year completers, respectively. 

Long-term adjunctive lacosamide treatment was generally well 

tolerated, reduced seizure frequency and maintained efficacy. 

Study supported by: UCB Inc. 

Elinor Ben-Menachem, within the past year, has had contracted 

research, consulting, speaking, and teaching, relationship 

with UCB. Cindy McShea and Jouko Isojarvi are 

employed by UCB BioSciences Inc. Pamela Doty is 

employed by UCB BioSciences, Inc. as a Principal Clinical 

Program Director for Lacosamide Epilepsy. As Principal 

Clinical Program Director, she receives stock option grants. 

William Rosenfeld is a principal investigator and has 

received research support from UCB for participating in 

double-blind and open-label studies. William Rosenfeld is 

on the UCB speaker’s bureau. 

M714. Lacosamide: Long-Term Safety in Partial-Onset 

Seizures 

Robert F. LeRoy, Gregory Krauss, Nathan B.. Fountain, 

Deanne Dilley, O’Neill D’Cruz and Pamela Doty; Dallas, TX; 

Baltimore, MD; Charlottesville, VA and Raleigh, NC 

Long-term safety of the antiepileptic drug (AED) lacosamide 

was evaluated in an open-label extension trial SP926 

(NCT00655486). 

Eligible participants were enrolled from IV infusion trial 

SP925 (NCT00655551). Investigators could adjust lacosamide 

oral tablet (100–800mg/day) and/or concomitant AED 

dosage to optimize treatment. Safety assessments included 

adverse events (AEs), ECGs and clinical laboratory data. 


Of the 97 enrolled patients, 58.8% and 38.1% had 

>12- and >18-months of lacosamide exposure, respectively. 

The median modal lacosamide dose was 500mg/day. 

TEAEs (incidence 15%) included dizziness (44.3%), diplopia 

(17.5%), and vomiting (16.5%); most were mild/ 

moderate in intensity. Only one serious AEs (SAEs) 

occurred in >1 patient(convulsion,n¼ 2). One patient 

discontinued due to SAEs (arrhythmia supraventricular 

and atrial fibrillation) and continued commercial lacosamide 

after treatment of the SAEs. One TEAE led to discontinuation 

in >1 patient (dizziness, n ¼ 3). Median 

clinical laboratory values remained within normal range; 

changes from Baseline were not of clinical relevance. Small 

increases in mean PR interval and QRS duration were consistent 

with the known lacosamide safety profile and did 

not vary with lacosamide exposure. 

Safety evaluations indicate long-term lacosamide administration 

(100–800mg/day) is generally well tolerated as adjunctive 

treatment for patients with partial-onset seizures. 

Study supported by: UCB Inc. 

Dr. RF LeRoy has a significant financial relationship with 

UCB, the sponsor of the study which generated this 

abstract. He participated in funded research as an investigator 

in study SP926 and other UCB funded studies as well 

as a speaker for the Vimpat Speaker ProgramHe has participated 

in UCB sponsored advisory boards for Vimpat. Dr. G 

Krauss has received research support from UCB S.A., Johnson 

& Johnson Inc., SK-Biosciences Corporation, Eisai Inc., 

Sepracor Inc., and Icagen, Inc., and has served as a paid 

consultant for Eisai Inc. and UCB S.A. Deanne Dilley: Employee, 

UCB BioSciences, Inc. O’Neill D’Cruz: Employee, 

UCB BioSciences, Inc. Pamela Doty is employed by UCB 

BioSciences, Inc. as Principal Clinical Program Director for 

Lacosamide Epilepsy. As a Principal Clinical Program Director, 

she receives stock option grants 

M715. Withdrawn 


M716. Computational Models of Ligand-Gated Receptor 

Function Characterize Anticonvulsant Drug Actions at 

GABAergic and Glutamatergic Synapses 

and David E. Naylor; Torrance, CA 

Many neurological drugs act on inhibitory GABAergic or 

excitatory glutamatergic receptors. Little is known about the 

direct effects of endogenous ligand or drug exposure on 

receptors in situ, and even less about broader pharmacological 

effects on normal or pathological circuit activity. Here, 

computational models of receptors characterize drug actions 

on multiple scales from submillisecond transmitter release, 

diffusion and uptake at single synapses to near seconds of 

residual receptor desensitization after multisynaptic 

synchronized release. GABA-A and NMDA receptor-kinetic 

models at synaptic and extrasynaptic sites in dentate gyrus 

granule cells are optimized to fit inhibitory and excitatory 

postsynaptic currents (PSCs) as well as evoked paired-pulse 

and tonic current responses after ligand and drug exposure. 

Synaptic GABA-A receptors over-exposed to GABA paradoxically 

show rapid downregulation/amplitude reduction 

from receptor desensitization. Hi-frequency 200Hz pulsatile 

release of GABA for 400 ms (as with epileptic fast ripples) 

also degrades synaptic inhibition, and recovery from desensitization 

is prevented by 0.5 to 2 Hz interictal-like discharges. 

Subsequent model fits of IPSCs/EPSCs after exposure 

to anticonvulsant agents quantify and optimize the 

effects of single and combination agents on receptor 

properties. 

53 

M717. Status Epilepticus: An Independent Predictor of 

Poor Survival after Anoxic Brain Injury 

Jennie Luna, Nelly Awkar, Megan McGarry, Deepthi 

Karanam and Dipak P. Pandya; Paterson, NJ 

Introduction: Evaluation of prognosis in Anoxic brain 

injury is a very difficult task due to lack of clinical and biological 

markers. The delay in neurological recovery is a poor 

prognostic sign. Status epilepticus (SE) may present in up 

to one third of patients with cardiac arrest. We report the 

significance of SE and myoclonic status as an independent 

clinical marker in anoxic brain injury. 

Methods: After IRB approval, we studied adult patients 

with SE in anoxic brain injury. Status epilepticus was 

defined with clinical history and EEG findings. Patients 

without EEG findings were excluded. Total 48 patients were 

identified. 

Results: 20/48(41%) had SE. 16/20(80%) expired and 4/ 

20(20%) survived. A 15% of the patients with SE had 

anoxic myoclonus with 100% mortality. All SE patients 

were comatose. Patients with SE, 60% reported duration of 

CPR 10 minutes. The other 40% had unknown duration. 

All our study patients were resistant to antiepileptic 

medications. 

Conclusion: Status epilepticus was present in 41% of 

patients and anoxic myoclonic status remains a grave prognostic 

sign for any survival. Overall, comatose mental status 

with SE or anoxic myoclonus remains an independent clinical 

marker for poor outcome. 

M718. Periodic Lateralized Epileptiform Discharges 

(PLEDs)-Rhythmic Discharges (RDs) in Anoxic 

Encephalopathy 

Sushanth Bhat, Sombabu Maganti, Eli S. Neiman, Divya 

Gupta and Sudhansu Chokroverty; Edison, NJ 

Objective: To describe an unusual EEG pattern of stereotypical 

PLEDs coupled with RDs in anoxic encephalopathy. 

Case Discussion: Following cardiac arrest, a comatose 79 

year-old woman had left facial twitching, sluggish pupils, 

absent corneal’s/Doll’s eyes reflexes and no withdrawal to 

stimuli. Facial twitching subsided with intravenous levetiracetam. 

CT scan of the head showed cerebral atrophy. An 

EEG showed right anterior/midtemporal PLEDs with slowsharp 

morphology recurring at 0.3–0.5 Hz, coupled with a 

9 Hz RD from the same area every 24–29 seconds and lasting 

5.5–7 seconds, not evolving into electrographic seizures 

and unchanged in morphology or frequency. There was no 

clinical/EEG background change to noxious stimulation. 

Discussion: PLEDs may be PLEDS-proper or PLEDSplus 

(associated with RDs). Both may occur with acute cerebral 

lesions, with PLEDs-plus more likely to be associated 

with seizures. However, focal and chronic lesions more often 

produce PLEDs-proper. 

The presented case is unusual, with no evolution of the 

PLEDs- RDs complex into electrographic seizures; such a 

pattern has not been described in acute, diffuse cortical injury 

such as anoxic encephalopathy, where BiPLEDs would be 

expected. Our EEG findings are reminiscent of those in isolated 

cortex in an animal model (Kristiansen, 1949). 

M719. Periodic Lateralizing Epileptiform Discharges 

(PLEDs) Causing Persistent Magnetic Resonance 

Imaging (MRI) Changes in Ipsilateral Thalamus 

Umang Shah, Umer Akber and Chunyang Wang; Camden, NJ 

Objective: To recognize PLEDs as potential cause of persistent 

MRI changes in ipsilateral thalamus. 



Background: Transient diffusion weighted image (DWI) 

abnormalities in ipsilateral thalamus following status epilepticus 

are well-recognized, but persistent changes in fluidattenuated 

inversion recovery (FLAIR) and T2-weighted 

images (T2WI) are rare. 

Design/methods: We report a patient who had persistent 

ipsilateral signal change in thalamus after complex partial 

status epilepticus and PLEDs. 

Results: Case: A 56 year-old woman presented with 

recurrent partial seizures involving the right arm and change 

in mental status. Electroencephalography revealed PLEDs in 

the left temporal lobe causing partial status epilepticus. Immediate 

brain MRI was normal but repeat MRI ten days 

later showed a focus of increased signal on FLAIR and 

T2WI in the left posterolateral thalamus. No abnormal 

enhancement was seen. Follow-up MRI one-month later 

demonstrated a persistent area of signal abnormality in the 

left thalamus. 

Discussion: Thalamic role in ictal events is unclear, but 

afferent and efferent cortical-interconnections may explain 

thalamic involvement in epileptic activity, which is generally 

considered a cortical phenomenon. Although DWI thalamic 

abnormalities are reported after status epilepticus, persistent 

FLAIR and T2WI signal is rare. 

M720. Ammoniacal Encephalopathy Presenting as 

Complex Partial Seizure-Like Episodes: A Case Series 


Objective: To report a series of cases with hyperammonemia, 

presenting as seizure-like episodes. 

Background: Seizures are caused due to several causes.- 

While accumulation of ammonia is a common cause of 

encephalopathy, it is also known to cause a broad spectrum 

of neurological manifestations. Seizures have not been 

reported in association with chronic hyperammonemia. 

Methods: Case Series 

Case report: Index case: A 53-year old man with chronic 

hepatitis C presented with confusional episodes associated 

with stereo-typed movements. Physical examination revealed 

psychomotor slowing. Routine serological and CSF evaluations 

were normal, except elevated ammonia (177 mmol/l). 

A diagnosis of ammoniacal encephalopathy was made. The 

patient was started on lactulose therapy with improvement. 

Four other patients with similar history, presentation, and 

elevated ammonia levels were seen. Complete resolution of 

symptoms were observed with treatment of hyperammonemia 

with lactulose in these patients. 

Conclusion: Here we present a series of patients with 

episodes of various neurologic dysfunctions. The only metabolic 

abnormality that was identified was an elevated ammonia 

level. We believe that his seizure-like episodes are associated 

with hyperammonemia. To our knowledge, this is the 

first report that links complex partial seizure-like episodes to 

hyperammonemia. 

M721. Status Epilepticus as an Initial Manifestation of 

Sneddon’s Syndrome 

Anish Shah, Saurav Sen, Jennie Luna and Dipak P. Pandya; 

Paterson, NJ 

Background: Sneddon’s Syndrome is a rare progressive noninflammatory 

vasculopathic disease affecting small and medium 

size arteries in conjunction with ischemic cerebrovascular 

disease, possibly livedo reticularis in absence of well 

recognizable connective tissue disease stigmata. It may associate 

with hepatitis C, thrombocytopenia and positive antiphospholipid 

antibodies. Seizures are rare during the course 

54 

of the disease. Status epilepticus has not been reported as a 

clinical manifestation in Sneddon’s Syndrome. 

Results: A 68 year old man with history of hypertension 

admitted with confusion, decrease responsivessness and subtle 

left sided weakness. He had non-itchy skin rash and mild left 

sided paresis. His laboratory tests showed thrombocytopenia, 

Hepatitis C, positive antiphospholipid antibodies. MRI 

showed acute right middle cerebral artery distribution infarct. 

EEG showed Periodic Lateralized Epileptiform Discharges 

from right hemisphere with changing morphology and evolving 

in nature. Patient was treated with antiepileptic medications, 

intravenous immunoglobulins, antiplatelet agents, statin 

and ACE inhibitors. His acute condition was improved and 

he was discharged to rehabilitative facility. 

Conclusions: Most cases of Sneddon’s syndrome are sporadic. 

It is unclear whether the status epilepticus is due to 

acute brain injury, from antiphospholipid antibodies or 

from inflammatory mediators. 

M722. Status Epilepticus Secondary to Milk Alkali 

Syndrome Induced by Hypercalcemia (Oral Antacids) 

Rabih Kashouty, Noor Yono and Mershed Al Samara; 

Manhattan, NY; Manhasset, NY and Southfield, MI 

Milk-alkali syndrome is mainly caused by the ingestion of 

large amounts of calcium and absorbable alkali. This syndrome 

can lead to metastatic calcification, renal failure and 

metabolic alkalosis secondary to hypercalcemia. Hypercalcemia 

is rarely a cause of seizure activity. Very few case reports 

have been published linking seizure to hypercalcemia, but 

only one recent case report about mesial temporal sclerosis 

relates the seizure activity to Milk-alkali syndrome. This is 

the first report regarding seizures associated with excess calcium 

carbonate intake without evidence of mesial temporal 

sclerosis. The patient described in this article, suffered from 

status epilepticus most likely secondary to hypercalcemia. 

Evaluations for malignancy, thyroid, and parathyroid dysfunctions 

were non conclusive, therefore hypercalcemia in 

our patient was attributed to milk-alkali syndrome given the 

history of the prolonged calcium carbonate intake. 

M723. A Case of Magnesium-Responsive Paraneoplastic 

Non-Convulsive Status Epilepticus 

Robert K. Shin, Anna V. Rosenbaum and Nicholas Frost; 

Baltimore, MD 

Objective: We present a case of paraneoplastic non-convulsive 

status epilepticus resistant to multiple AEDs that 

resolved with magnesium. 

Background: Paraneoplastic syndromes affecting the 

nervous system have been described in association with 

numerous cancers, some presenting as limbic encephalites 

associated with seizures, including an encephalitis in young 

women with ovarian teratoma associated with anti-NMDA 

receptor antibodies. 

Results: A 57-year old female with clear cell ovarian cancer 

became unresponsive following a one week prodrome of 

increasing confusion and forgetfulness. EEG demonstrated 

non-convulsive status epilepticus. Imaging, serum and CSF 

studies were non-diagnostic. Over a course of two weeks, she 

did not respond to multiple anti-epileptic medications and 

continued to have up to 30 seizures daily. IV magnesium was 

administered, and the following morning she was alert, oriented 

and able to converse with her husband. 

Conclusions: While magnesium is routinely used to treat 

seizures in obstetrics, its use in neurology is uncommon. 

Given that it is a known NDMA inhibitor, it may represent 


a useful treatment option, particularly in cases of paraneoplastic 

seizures. 

Study supported by: Study is a case report. There are no 

sponsors or affilitations associated with it. No individual 

associated with this case report (or their relatives) has a relevant 

financial interest relating to the support of the abstract. 



M801. Nicotinamide Mononucleotide (NMN) Treatment 

of Diabetic Neuropathy 

Ankit Sura, Mitch Onken, Krish Chandrasekaran, Helen Chen 

and James W. Russell; Baltimore, MD 

Nicotinamide adenine dinucleotide (NAD) is a critical 

metabolite in energy metabolism and mitochondrial (Mt) 

electron transfer. Nicotinamide mononucleotide (NMN) is a 

direct substrate for generation of NAD. A decreased NAD 

level in diabetes may increase the severity of diabetic neuropathy. 

We determined if NMN prevented the development 

of diabetic neuropathy. Adult rats were made diabetic 

with streptozotocin. NMN was injected every second day. 

There were 4 treatment groups: (1) non-diabetic control 

with vehicle (2) diabetic (3) diabetic þ 50 mg/kg NMN (4) 

diabetic þ 100 mg/kg NMN. The Von Frey monofilament 

sensory withdrawal threshold was normalized in diabetic rats 

treated with 50 mg/kg or 100 mg/kg NMN for a period of 

2 months from the onset of diabetes (n ¼ 6 rats/group, 

P


which reproduces key aspects of human disease, including 

the presence of MN cytosolic aaggregates, and pronounced 

astrocytic as well as MN pathology. 

Study supported by: NIH grant NS36548 

M806. Reevaluating Disease Progression in 

Facioscapulohumeral Dystrophy 

Jeffrey M. Statland*, William B. Martens, Kathryn R. Wagner, 

John Kissel, Shree Pandya, Michael P. McDermott and Rabi 

Tawil; Rochester, NY; Baltimore, MD and Columbus, OH 

Background: Recent breakthroughs in the molecular pathophysiology 

of Facioscapulohumeral dystrophy (FSHD) have 

identified potential therapeutic targets. Consequently, an 

accurate understanding of disease progression in FSHD is 

crucial for the design of future clinical trials. 

Methods: Data from 164 subjects in 2 negative clinical 

trials and 1 natural history study were combined to examine 

disease progression in FSHD. All studies utilized the same 

techniques for manual muscle testing (MMT) and quantitative 

myometry (QMT). Both techniques yield a total 

strength score that can be followed over time as an indicator 

of disease progression. 

Results: Whereas, natural history data showed a statistically 

significant decrease in strength by both QMT and 

MMT at 1 year, combined data from all 3 studies demonstrated 

a reduced estimate of loss of strength for both techniques 

at 1 year, which was no longer significant for QMT. 

Comparing natural history to combined clinical trial data 

suggested this reduced estimate of disease progression was 

due to a placebo effect, most noticeable at 6 months. 

Conclusions: Contrary to estimates based only on natural 

history data, treatment durations of longer than 1 year will 

be required to demonstrate arrest of disease progression in 

future FSHD therapeutic trials. 

M807. Dysferlin (DYSF) Is Absent from the 

Muscle-Fiber Sarcolemma in Various Neuromuscular 

Diseases, and in Sporadic Inclusion-Body Myositis 

(s-IBM) It Forms Cytoplasmic Inclusions Colocalizing 

with Amyloid-b42 (Ab42) 

Mafalda Cacciottolo, Anna Nogalska, Carla D’Agostino, 

W King Engel and Valerie Askanas; Los Angeles, CA 

DYSF is a transmembrane protein participating in muscle 

plasmalemma repair. In normal human biopsies, DYSF is 

present at the muscle-fiber sarcolemma. In dysferlinopathies 

– autosomal-recessive muscle diseases caused by mutations 

in the DYSF gene – DYSF is immunohistochemically absent 

from the sarcolemma, and is absent or prominently 

decreased by immunoblots. Immunohistochemically-absent 

DYSF at the sarcolemma is sometimes considered ‘‘diagnostic’’ 

of dysferlinopathies. However, sarcolemmal absence of 

DYSF, and a cytoplasmic ‘‘neolocalization’’, was previously 

reported in some sarcoglyconopathies and DMD patients. 

We now report absence of DYSF at the sarcolemma of 

virtually all muscle fibers of 4 ALS, 4 peripheral neuropathy, 

5 s-IBM, and 1 polymyositis biopsies. In s-IBM, vacuolated, 

very abnormal muscle fibers often had DYSF-immunoreactive 

cytoplasmic inclusions that colocalized with Ab42. Four 

age-matched controls had normal sarcolemmal DYSF distribution. 

By immunoblots, DYSF levels were comparable in 

all biopsies. Thus: a) immunolocalization of DYSF cannot 

be a diagnostic criterion of dysferlinopathy; b) Ab42 might 

be a novel binding partner of DYSF, and in that complex 

DYSF might contribute to the s-IBM pathogenesis. 

Study supported by: MDA 

56 

M808. Novel Demonstration of Conformationally- 

Modified Tau in Sporadic Inclusion-Body Myositis (s- 

IBM) Muscle Fibers. Possible Importance to s-IBM 

Pathogenesis 

Anna Nogalska, Carla D’Agostino, King W. Engel and Valerie 

Askanas; Los Angeles, CA 

Molecular phenotype of s-IBM muscle fibers, the most 

common myopathy of older persons, has similarities to Alzheimer-disease 

(AD) brain, including intra-muscle-fiber 

accumulations of Ab42 and its oligomers, and large clusters 

of paired-helical filaments (PHFs) immunoreactive with various 

antibodies recognizing phosphorylated tau (p-tau). 

In AD brain, conformational changes of tau, including its 

modifications detectable with specific antibodies TG3, 

Alz50 and MC1, are early and important modifications 

leading to tau’s abnormal folding and assembly into PHFs. 

We have now identified conformationally modified tau (ctau) 

in 13 s-IBM muscle biopsies by a) light-and electronmicroscopic 

immunohistochemistry, b) immunoblots, and c) 

dot-immunoblots, using TG3, Alz50 and MC1 antibodies. 

Interestingly, in the very atrophic degenerating fibers, TG3 

co-localized with PHF-1 antibody recognizing p-tau, considered 

a later change in the PHFs formation; however, most 

of TG3-positive inclusions in non-atrophic fibers were 

PHF-1 immunonegative. None of the 12 disease- and normal-control 

muscle biopsies contained c-tau or PHF-1 immunoreactive 

tau. This first demonstration of c-tau in s- 

IBM suggests that, because of its abundance in non-atrophic 

muscle fibers, c-tau might play an early role in s-IBM PHF 

formation and thus be pathogenically important. 

Study supported by: MDA 

M809. Anti-Ganglioside Antibodies Mimic CNS 

Inhibitors of Axon Regeneration 

Kazim Sheikh and Gang Zhang; Houston, TX 

Anti-ganglioside antibodies (Abs) are strongly associated 

with axonal forms of Guillain Barré syndrome (GBS). Several 

studies indicate that GBS patients with anti-ganglioside 

Abs directed against GM1, GD1a, or ganglioside complexes 

have poor prognosis and/or incomplete recovery. We 

recently demonstrated that experimental monoclonal and 

GBS patient derived anti-ganglioside Abs can inhibit regeneration 

of injured axons in an animal model suggesting that 

Ab-mediated inhibition of nerve repair is one mechanism of 

delayed recovery. We have now established motor and sensory 

primary neuronal culture models to examine the cellular 

and molecular mechanisms of this Ab-mediated inhibition 

of axon regeneration. We found that GBS patient’s 

anti-ganglioside Abs can inhibit neurite outgrowth in primary 

neuronal cultures. This Ab-mediated inhibition of 

neurite outgrowth involves the activation of small GTPase 

RhoA and downstream effector ROCK pathway and this 

activation is through the engagement of specific cell surface 

gangliosides by Abs. In summary, these studies directly link 

patient autoantibodies to an intracellular inhibitory signaling 

pathway that is also central to inhibitory signaling induced 

by almost all CNS inhibitors of axon regeneration. Our 

results support the hypothesis that specific anti-ganglioside 

Abs mimic intracellular signaling induced CNS inhibitors of 

axon regeneration. 

Study supported by: NIH/NINDS 

M810. Evaluating Mechanoreceptors in Glabrous Skin in 

Diabetic Neuropathy 


Nashville, TN 



Meissner corpuscles (MCs) and their myelinated afferents 

are found only in glabrous skin. We hypothesized that alterations 

in MCs occur in diabetic neuropathy and correlate 

with other measures of axonal loss. Immunohistochemistry 

was performed on 2 and 3 mm skin punches from the index 

finger and distal leg, respectively. Four diabetic patients 

(ages 45–75), with neuropathy confirmed by exam and 

nerve conduction studies and four control patients (aged 

35–50), were studied. Average Meissner corpuscle density 

(MCD) in control patients was 15.3 6 7.1 MCs/ mm 2 

with significant reductions observed in two patients (3.3, 

6.2 MCs/ mm 2 ) with near normal or higher density in two 

other patients (11.1, 28.1 MCs/mm 2 ), suggestive of proliferation. 

MCs in diabetics often displayed abnormal morphology. 

Density of intrapapillary myelinated endings (IME) 

correlated with MCD in all patients (r ¼ 0.97). Diabetic 

patients’ intraepidermal nerve fiber density (IENFD) in the 

distal leg was 4.2 6 5.9 fibers/mm, compared to 13.7 6 

1.3 in controls. IME density did not correlate with IENFD, 

suggesting that these populations of fibers are independently 

affected by hyperglycemia. Glabrous skin biopsies afford 

evaluation of mechanoreceptors which are important in 

studying pathophysiology of diabetic neuropathy. 

Study supported by: Supported by NINDS K23 

NS056009 (A.C. P.), NINDS R01NS066927-01(J.L.), Vanderbilt 

CTSA grant 1 UL1 RR024975. 

M811. Amiodarone Associated Myopathy. A Report 

of 4 Cases 

Eoin P. Flanagan, Charles M. Harper, Erik K. St. Louis, 

Michael H. Silber, Ronald C. Petersen and Keith A. Josephs; 

Rochester, MN 

Myopathy is a known side effect of amiodarone. However, 

the characteristic features and outcome are not widely 

known. We identified 4 patients from 1996 – 2010 at our 

institution with electrophysiologically confirmed myopathy 

and a final diagnosis of presumed amiodarone induced myopathy. 

The median age was 71 years (range, 67–75) and all 

had an insidious onset. Initial presenting symptoms 

included: lower extremity weakness, 3; and swallowing difficulty, 

1. Amiodarone was begun a median time of 20.5 

months (range, 1–36) before symptom onset. Laboratory 

features included elevations in: creatine kinase, 2; myoglobin, 

2; and aldolase, 1. EMG demonstrated short duration 

motor unit potentials in proximal muscles in all four 

patients and features of myonecrosis were seen in two. Muscle 

biopsies performed in two patients demonstrated severe 

necrotic myopathy with tubular aggregates in one and 

vacuolated atrophic fibers in the other. All 3 patients with 

follow up available improved after discontinuation of amiodarone, 

but in two the recovery was prolonged (>6 months) 

and incomplete. Amiodarone induced myopathy appears to 

affect mostly proximal muscles with variable electrophysiological 

and pathological findings. Discontinuation of amiodarone 

is associated with improvement, but recovery may be 

prolonged and incomplete. 

M812. Cervical Cord 1 H-Magnetic Resonance 

Spectroscopy ( 1 H-MRS) in Amyotrophic Lateral Sclerosis 

(ALS): Relationship to Clinoco- Electrophysiological 

Dysfunction 

Ken Ikeda, Yasuhiro Yoshii, Kiyoko Murata, Riya Nagata, 

Kiyokazu Kawabe, Osamu Kano and Yasuo Iwasaki; Tokyo, 

Japan 

Background: To examine whether clinico-electrophysiological 

aspects are related to spinal cord dysfunction on 1 H-MRS in 

57 

ALS patients. Methods: Fifteen patients with definite or 

probable ALS fulfilled the El Escorial revised criteria, and 15 

age- and sex-matched controls underwent cervical cord 1 H- 

MRS. A volume of interest with dimensions of approximately 

6.0 8.0 40.0 mm (19.2 mL) was located along the main 

axis of C1-C3 cord on T2-weighted images. N-acetyl-aspartate 

(NAA), choline-containing compounds (Cho), creatine 

plus phosphocreatine (Cr) and myo-Inositol (m-Ins) were 

determined. ALS functional rating scale (FRS) and forced 

vital capacity (FVC) were assessed monthly. Electromyography 

was performed. Results: NAA/Cr was decreased significantly 

(p


parameters. Mean infection rate 6 standard deviation(SD)before 

and after HFCWO was compared with paired, unpaired 

two-tailed t-tests and Wilcoxan rank tests. 

Results: ALS patients[36]patients pre-HFCWO had 0.07 

6 0.13(SD)infections/month which reduced to 0.02 6 

0.06 infections/[t test p ¼ 0.0638; Wilcoxon ranked test p 

¼ 0.0415]. Compliance in each patient compared well with 

the mean national compliance assessed by Hill-Rom in neuromuscular 

disease. 

Conclusion: HFCWO significantly reduces the perpatient 

infection rate in ALS patients. Observational studies 

employing pre-post-intervention design can provide important 

clinically meaningful information supporting certain respiratory 

interventions in ALS patients. 

Study supported by: Carolinas ALS Research Fund of the 

Carolinas Healthcare Foundation and Hill-Rom 

M815. Dominant Cardiomyopathy and Very Distal 

Myopathy with Rod, Myofibrillar and AVSF 

Myopathology 

Stanley J. Iyadurai, Chris Weihl, Bob Baloh and Alan 

Pestronk; St. Louis, MO 

We describe a multigenerational family with a distal myopathy 

with unusual features. The syndrome segregated by history 

as a dominantly-inherited disorder with affected family 

members of both sexes. Four affected patients without cardiac 

pacing died before age 40. Two affected patients with 

cardiac pacing were examined in their fifth decade. Symmetric 

weakness was most severe in intrinsic muscles of the 

hands and feet, averaging less than 10% of normal. Intrinsic 

muscles of the hands and feet were severely atrophic. Proximal 

muscle strength was normal. Tendon reflexes and sensation 

were normal. Serum CK was less than 250. EMG 

revealed a distal-predominant, irritable myopathy. Nerve 

conduction studies were normal. Muscle ultrasound showed 

a distal myopathy involving the hands and legs. Muscle biopsy 

showed varied fiber size, splitting, rods, desmin aggregates, 

and vacuoles containing granular debris with rims 

staining for sarcolemmal proteins, including dystrophin, sarcoglycans 

and caveolin. Distinctive features include very distal 

weakness and wasting, early sudden death, and myopathology 

with rods, myofibrillar changes in addition to 

AVSFs. Genotyping will be required to further define the 

identity of this distal myopathy syndrome. 

Study supported by: Department of Neurology and Psychiatry, 

Saint Louis University, St. Louis, MO. 

M816. Adult-Onset Rod Myopathy Syndrome 

(AORMS): Sustained Benefit from IVIG Plus Rituximab 

Shalini Mahajan, King W. Engel, Valerie Askanas, 

Indermohan Luthra and Varun Gupta; Los Angeles, CA and 

Rancho Mirage, CA 

AORMS (Engel WK,’66), is a rare, progressive, myopathy 

associated with intrafiber rods, often monoclonal gammopathy 

(MG), 6 dysschwannian neuropathy, and often fatal. 

Patients were unresponsive to anti-dysimmune therapies, 

including rituximab (Keller CE,’06). We report successful 

treatment of a 55yr old AORMS patient. Before treatment, 

he had for 1yr, sub-acute, progressively severe proximal limb 

and neck weakness, and dysphagia. Tendon reflexes were 

absent, vibratory and pinprick sensations decreased. Serum: 

IgG-kappa MG, normal CK. CSF: protein elevated, 59/45. 

NCVs: dyschwannian (demyelinating) neuropathy. EMG: 

fibrillations and brief small abundant motor-units. Muscle 

biopsy: groups of trichrome-red intrafiber rods, and a few 

small angular fibers. Rx: IVIG, 0.4 gm/kg infusions twice- 

58 

weekly, continuing for 6 yrs, arrested progression and sustained 

partially-improved strength, breathing and swallowing. 

In 3/10, added rituximab, 375mg/m2 q2wksx4 loading, 

plus q12-6wks maintenance, produced further increased 

strength, regained ability to walk independently 150ft, climb 

stairs, turn in bed, shower standing, and drive a car. This 

benefit from the continuing IVIG-plus-rituximab therapy 

emphasizes treatability of the AORMS and suggests a dysimmune 

pathogenesis. His concurrent CIDP-like aspect 

may be relevant to his beneficial response. (Interestingly, 

experimentally, tenotomy can produce rods (Engel et al, 

‘66).) 

M817. Geographic Trends of ALS in Minnesota 

Eric J. Sorenson and Lisa Kronk; Rochester, MN and 

Minneapolis, MN 

Background: This study examined geographic trends of 

ALS across Minnesota and assessed the completeness of ALS 

case ascertainment within the ALS association’s database 

(Minnesota/North Dakota chapter). 

Methods: The ALS association’s database was queried for 

deaths during 2009 in Minnesota with the county of residence 

recorded for each. 2009 census data was recorded for 

each Minnesota county with an incidence rate calculated for 

each county and for Minnesota overall. A chi-squared analysis 

compared incidence rates among the 88 counties. 

Results: 97 deaths occurred from a population of 

5,266,203, giving an incident rate of 1.8 cases per 100,000 

person-years (95% CI from 1.5 to 2.2). This agreed with 

incidence rates from Olmsted County of 1.7 per 100,000 

person-years suggesting that the ALS Association’s database 

has near complete case ascertainment throughout Minnesota. 

County incidence rates varied from 0 to 12.7 per 

100,000 person-years. However, comparison across counties 

failed to demonstrate any significant differences (p ¼ 0.42). 

Conclusions: No significant geographic trends were identified 

among the counties in Minnesota suggesting the clustering 

of cases in some counties is likely due to random case 

aggregation. The ALS association’s database proved effective 

at near complete case ascertainment in the state of 

Minnesota. 

Study supported by: ALS Association 

M818. Clinical Features Associated with Fine Specificity 

of IgG Anti-GQ1b Antibodies 

Susumu Kusunoki, Seiko Suzuki, Masami Ueda and Motoi 

Kuwahara; Osaka-Sayama, Osaka, Japan 

Anti-GQ1b IgG antibodies are present in most cases of 

Fisher syndrome (FS), Bickerstaff brainstem encephalitis 

(BBE), and Guillain-Barré syndrome (GBS) with ophthalmoplegia. 

Anti-GQ1b antibodies frequently bind to GT1a, 

but the relative binding activities with the GQ1b and GT1a 

antigens vary. Some anti-GQ1b antibodies have higher activity 

against a mixture of GQ1b and phosphatidic acid 

(GQ1b/PA). We investigated anti-GQ1b IgG-positive cases 

including FS (n ¼ 197), BBE (n ¼ 20), GBS (n ¼ 78) and 

atypical FS (n ¼ 52) to see whether diversity in the fine 

specificity of IgG anti-GQ1b antibodies is related with variations 

in clinical features. Higher antibody activity with 

GQ1b compared to GT1a (GQ1b>GT1a) was significantly 

more frequent in BBE and FS than in GBS (pGQ1b/PA) was significantly more frequent in BBE 

than in FS (pGQ1b were found in patients 

with bulbar palsy (p


titer was significantly higher in patients with ophthalmoplegia 

(pbulbar >depression 

>pseudobulbar affect. 

Study supported by: Carolinas ALS Research Fund of the 

Carolinas Healthcare Foundation 

M820. A New Phenotype in Neurodegeneration: 

Trigeminal Sensory Deficits Preceding Rostro-Caudal 

Progressive Motor and Sensory Neuronopathy, Chorea 

and Dementia 

Camilo Toro, Justin Y. Kwan, Tanya J. Lehky, Bryan J. 

Traynor, Catherine A. Groden, Rena A. Godfrey, Michele E. 

Nehrebecky, Wiggs A. Edythe and Gahl William; Bethesda, 

MD 

We describe five adults with a slowly progressive bulbaronset 

ALS like phenotype but preceded by trigeminal sensory 

symptoms, with or without late development of chorea 

and dementia. Age of onset was 53–69 years; disease duration 

was 5–12 years. 

Three women and two men experienced facial/oral sensory 

disturbance involving the unilateral infra-orbital area 

(3), tongue, or lips 6–18 months before bulbar motor deficits. 

At evaluation, all had impaired cornea reflexes, facial/ 

oral hypoesthesia or anesthesia extending to posterior scalp 

plus truncal and shoulder weakness, while lower limb 

strength was relatively preserved. Three patients had frontotemporal 

dysfunction and two had chorea. Neuroimaging, 

CSF examinations, metabolic, toxic and immunological 

59 

screening (including Sjogren’s and anti-ganglioside antibodies) 

was uninformative. Electrophysiology revealed motor 

neuronopathy involving 3–4 body segments and sensory 

neuropathy. Blink reflexes in four and autonomic testing in 

two were abnormal. In 3 patients, nerve biopsies showed 

non-specific axonal degeneration/regeneration. Four patients 

failed immunotherapy. One autopsy examination revealed 

loss of spinal motor neurons. 

This disorder, distinct from sporadic bulbar-onset ALS 

resembles FOSMN, but with dementia and/or chorea. We 

are applying new genomics tools to study this unique 

patient group. 

Study supported by: National Institues of Health (NIH)- 

Intramural Program 

M821. Update on a Phase 1 Study of ISIS 333611 in 

Familial ALS Due to SOD1 Mutations 

Timothy M. Miller, Richard Smith, Swati Aggarwal, Alan 

Pestronk, William David, Jeffrey Rothstein, Ericka Simpson, 

Benjamin Brooks, Isaac Bakst, Patricia Andres, Peggy Allred, 

Katie Alexander, Kathie Bishop, C.F. Bennett and Merit 

Cudkowicz; St. Louis, MO; La Jolla, CA; Boston, MA; 

Baltimore, MD; Houston, TX; Charlotte, NC and 

Carlsbad, CA 

Objective: To evaluate the safety, tolerability, and pharmacokinetics 

of intrathecal infusion of ISIS 333611, an antisense 

oligonucleotide inhibitor of SOD1 mRNA. 

Background/Methods: Mutations in SOD1 cause 20% 

of familial ALS. In animal models, ISIS 333611 distributed 

widely in the brain and spinal cord, decreased 

SOD1 mRNA, and prolonged survival. A randomized, 

placebo controlled Phase 1 safety trial of ISIS 333611 is 

underway. In Cohort 1, ISIS 333611 (0.15 mg) was 

infused intrathecally over 12 hours in 6 SOD1-positive 

ALS patients (2 patients received vehicle control). Safety 

measures and neurological exams were assessed during the 

infusion and after 1, 8 and 29 days. CSF and plasma 

drug levels were measured. This completes 1 of 4 

cohorts, each with increasing dose and similar active:placebo 

ratio. 

Results: No serious adverse events occurred and common 

adverse events are consistent with procedure-related findings. 

Changes in neurological exams are consistent with ALS. 

CSF and plasma drug levels are consistent with levels predicted 

from preclinical studies. An update on the SOD1 

antisense program will be provided. 

Conclusion: In Cohort 1, ISIS 333611 was well tolerated 

and CSF drug levels are as predicted. 

Study supported by: ALS Association, Muscular Dystrophy 

Association, Isis Pharmaceuticals 

M822. Retrospective Analysis of a Cohort of Non 

Systemic Vasculitic Neuropathy 

Raghav Govindarajan, Jagadish B. Agadi, Anita Mahadevan 

and S.K. Shankar; Weston, FL and Bangalore, India 

Background: Nonsystemic vasculitic neuropathy (NSVN) is 

an infrequent neuropathy without systemic manifestations. 

Only few studies have reported the clinicopathologic spectrum. 

Fewer have looked into the treatment aspects. 

Methods: Nerve biopsies done over 10 years were 

reviewed. Patients who met criteria for NSVN at time of diagnosis 

and in whom 6 month follow-up was available were 

included. They were treated with steroid therapy (ST) or 

with steroids and azathioprine-combination therapy (CT). 

Independent T-tests were performed for age, duration of 

symptoms prior to therapy and improvement in Prineas 



disability score (PDS) between groups. Differences between 

pre and post-therapy PDS within groups were analyzed by 

paired T-tests and Chi-Square. 

Results: 21 cases were in the series with 12 males and 9 

females.Sensori-motor asymmetric axonopathy was seen in 

71%. Biopsy showed microvasculitis in 52%. 12 were 

treated with ST, 7 with CT,2 opted out. CT was superior in 

improving disability (1.57 vs. 1.08 p < 0.05). CT was 14 

times more likely to improve PDS by more >2 units (p ¼ 

0.01). The percentage of improvement did not significantly 

differ between them (paired T-test 50% vs.42% p ¼ 0.37). 

Conclusion: NSVN is an asymmetric sensory predominant 

axonopathy. Combination therapy may be superior to 

steroids alone in improving disability. 

M823. Optimizing a Hospital Discharge Database for 

Passive Surveillance of Guillain-Barre Syndrome (GBS) 

Christopher D. Lee and Timothy F. Jones; Nashville, TN 

The 2009–2010 H1N1 pandemic influenza immunization 

program prompted a nationwide active surveillance effort to 

determine the risk of GBS following vaccination. Active surveillance 

is unsustainable in the long-term. Therefore, we 

sought to optimize a statewide hospital discharge database 

to evaluate its utility for GBS surveillance, compared to 

active surveillance data. 

We examined data from all patients discharged from Tennessee 

hospitals with an ICD-9 code for GBS, from 2000– 

2010. A total of 2659 cases with Tennessee residency were 

identified. Of these, 1098 (41%) were excluded due to a 

prior hospitalization with a GBS diagnosis in the database. 

Annual incident cases ranged from 143 to 161 (mean 

adjusted incidence calculated as 1.41 per 100,000 individuals). 

Variation in annual case tallies was significantly narrowed 

with this method, compared to aggregate ICD-9 

totals. We calculate capability of detecting an increase in 

GBS cases with an effect size of 1.20. Sensitivity compared 

to active surveillance was 0.81. Positive predictive value was 

0.45. 

Although incidence calculations are overestimated, an 

optimized hospital discharge database shows low variation 

and is capable of detecting small increases in GBS cases. 

Such a database can be useful for long-term surveillance. 

M824. Gene Expression Analysis in Patients with 

Amyotrophic Lateral Sclerosis and Multifocal Motor 

Neuropathy 

Alexander Shtilbans, Soon Gang Choi, Mary E. Fowkes, Greg 

Khitrov, Mona Shahbazi, Jess Ting, Stuart C. Sealfon and 

Dale J. Lange; New York, NY 

Objectives: To confirm the previously identified gene 

expression pattern in muscles from patients with Amyotrophic 

lateral sclerosis (ALS) and multifocal motor neuropathy 

(MMN) compared to controls. 

Methods: RNA extracted from skeletal muscles of 3-ALS, 

3-MMN and 3-control patients were subjected to RT-PCR 

confirmation analysis based on the previously published genome-wide 

microarray gene expression data (Shtilbans et al, 

Ann Neurol.,2009). Four additional ALS patients and four 

new controls were also used. 

Results: Validation analysis of the most significant expression 

pattern differences confirmed our previous microarray 

data for leucine-rich repeat kinase-2 (LRRK-2), follistatin, 

collagen type XIX alpha-1, ceramide kinase-like and sestrin- 

3 which were overexpressed only in the ALS group. 

CXorf64 was increased in ALS and decreased in MMN 

compared to controls. Western blot analysis of LRRK-2 

60 

gene protein level from ALS muscles showed no obvious 

difference compared to controls. 

Conclusions: The up-regulation of the above genes in 

the muscles of ALS patients relative to MMN and controls 

discovered previously by our microarray analysis is reproducible 

and statistically significant. Further studies are necessary 

to evaluate the identified genes in larger patient 

groups and different tissues. 

Study supported by: This work was supported by grants 

from the Muscular Dystrophy Association and the Horace 

Havemeyer Neuromuscular Research Fund. 

M825. Small-Fiber Polyneuropathy (SFPN) Can Cause 

Chronic Pain and Somatic Complaints in Youth 

and Anne Louise Oaklander; Boston, MA 

Patients with unexplained diffuse pain and multisomatic 

complaints are enigmatic and hard to treat, with pediatric 

cases especially concerning. An index youth with chronic 

pain from steroid-responsive SFPN prompted record analysis 

from 41 consecutive young patients with widespread 

pain and/or multisomatic complaints; age-matched volunteers 

provided controls. Age of onset averaged 13y; 73% 

were female. Most were seriously ill; 71% were hospitalized 

and 1 died. Half reported preceding injuries or infections. 

29% had other autoimmune illnesses. 98% had chronic 

pain and 98% had somatic symptoms, often cardiovascular 

and/or gastrointestinal. Abnormal fatigue, headaches, and 

sweating each affected more than half. Sensory abnormalities 

were present in 71% and erythromelalgia in 28%. 

94% of 33 autonomic-function tests were abnormal, 6% 

borderline, and most skin (25) and 2/2 nerve biopsies 

implicated SFPN. Etiologic testing spotlighted only 

immune markers (in 86%). Many symptoms became manageable 

once explained as neuropathic. Corticosteroids or 

IVIG helped 70% of 14 treated. This characterizes a disabling 

illness, juvenile-onset SFPN, sometimes caused by 

organ-specific autoimmunity. This verifiable and treatable 

diagnosis merits consideration in youngsters with syndromes 

of widespread pain and multisystem complaints, eg 

fibromyalgia, chronic fatigue, POTS, and seronegative 

Lyme. 

Study supported by: NINDS, Dept. of Defense, Bradley 

family foundation, Curvey family foundation 

M826. Median Nerve Ultrasound in Diabetic PN with 

and without Carpal Tunnel Syndrome 

Anhar Hassan, Andrea Leep Hunderford, James Watson, 

Andrea Boon and Eric Sorenson; Rochester, MN 

Background: Gold standard diagnosis for carpal tunnel syndrome 

(CTS) is nerve conduction study (NCS). However 

sensitivity decreases in peripheral neuropathy (PN). Ultrasound 

detects median nerve enlargement but its utility in 

coexistent PN is unknown. 

Methods: Case-control study enrolling diabetic PN 

patients (NCS-proven) referred to our EMG lab, with or 

without clinical symptoms of CTS. Demographics, CTS 

symptom score, median and ulnar NCS are recorded and 

correlated with blinded median and ulnar nerve ultrasound 

cross-sectional areas (CSA). 

Results: Full data to be presented. Six patients (100% 

male; 2 cases, 4 controls) are enrolled so far: age 50.5 years 

(range 35–68), with diabetes duration 106.5 months (10– 

300) and PN 13.5 months (0–93). Median nerve CSA was 

significantly larger (p ¼ 0.03) in CTS cases, and median 

antidromic sensory distal latency was significantly prolonged 

(p ¼ 0.04). There was no significant difference in diabetes 



(p ¼ 0.13) or PN duration (p ¼ 0.16); neurophysiologic 

PN severity (p ¼ 0.18); median motor (p ¼ 0.14) or sensory 

palmar NCS (p ¼ 0.05). 

Conclusions: In diabetic patients with peripheral neuropathy, 

median nerve CSA appears significantly enlarged with 

coexistent clinical CTS. If findings are confirmed, median 

nerve ultrasound may be a useful addition to NCS. 

M827. Subacute Paraneoplastic Motor Neuronopathy 

with Ri Immunoeactivity and Breast Cancer: A 

Clinicopathologically Studied Patient 

and David S. Younger; New York, NY 

A 49 year old woman had fatigue, cramps, and leg twitching 

in February 2010, four months before noting a breast 

mass. Lumpectomy in June 2010 showed stage II invasive 

ductal breast cancer, and estrogen and progesterone receptor 

positivity. Examination showed leg weakness, atrophy, 

and widespread twitching. Initial EMG/NCS showed 

motor predominant polyneuropathy of the legs with 

demyelinating features and persistent fasciculation. MRI of 

the brain, spinal cord, and cerebrospinal fluid showed no 

evidence of metastases. Blood studies showed positive 

ANNA-2 (Ri) titer 1:7680 from an extensive panel of paraneoplatic 

autoantibodies. Intravenous immunoglobulin 

(IVIg) was followed by combination chemotherapy. Followup 

EMG/NCS showed persistent motor polyneuropathy 

and fascicuation with resolution of demyelinating features. 

Prophylactic radiotherapy was well tolerated. She 

continued to decline whereupon followup EMG/NCS in 

February 2011showed motor neuropathy with active leg fibrillation 

and fasciculation. Sural nerve and soleus muscle 

biopsy were consistent with subacute motor neuronopathy. 

CSF evidenced Ri immunoreactivity using purified human 

recombinant antigen and Western blot analysis. Whole 

body PET/CT showed no evidence of malignancy. This is 

the first report of subacute motor neuronopathy in association 

with the Ri (ANNA-2) antibody. 

M828. Magnetic Resonance Neurography Versus 

Electromyography for the Diagnosis of Radigulopathy 

Youssef A. Dakka, Andrew Biondo, John Corrigan, Shehanaz 

Ellik and Ximena Arcilla-Londono; Detroit, MI 

Introduction: Evaluation of spinal nerve disorders relies on 

an accurate history, examination, MRI, MR Neurography 

and EMG/NCS. The purpose of our study was (a) to determine 

if MRN could detect radiculopathies earlier than 

EMG. 

Methods: Inclusion criteria included adults with cervical 

radiculopathy, MRN and EMG studies. Exclusion criteria 

included peripheral neuropathy, muscle disease, diabetes or 

tumor. Of the 438 patients reviewed, 64 patients were 

included. Symptoms, time course, MRN/EMG findings and 

consistency of MRN/EMG results with clinical findings 

were obtained retrospectively. The patient population was 

subdivided into males/females and symptom duration < or 

> 2 months. McNemars test and Generalized Estimating 

Equation Model were used. Interaction was evaluated at the 

significance level of 0.10. 

Results: Differences in agreement between MRN and 

EMG were 23.5% for females and 6.7% for males. For 

those with 

2 months duration, it was 20.9%. However, because of the 

small sample size, the power to detect these differences was 

reduced and they were not statistically significant. 

61 

Conclusion: In our study there were no significant benefits 

to MRN over EMG in radiculopathy diagnosis. 

M829. Denervation Causes Lower Expression of Heat 

Shock Protein 27 in Regenerating Skeletal Muscle 

Takahiro Jimi, Yoshihiro Wakayama and Hajime Hara; 

Yokohama, Japan 

We have studied changes of gene expression in denervated 

skeletal muscles and found a remarkably increased expression 

of genes relating to quality control. Denervation results 

in the incomplete regeneration of skeletal muscles. So we 

examined the mRNA expression of heat shock protein 27 

(HSP 27) in regenerating rat muscles in innervated and denervated 

conditions. We made rat regenerating muscles by 

injection of bupivacaine in both extensor digitorum longus 

(EDL) muscles. At the same time of injection, right sciatic 

nerve was removed. At 1, 2, 4, and 6 weeks after surgery, 

both EDL muscles were excised from five rats. The concentrations 

of HSP 27 mRNA were estimated by real-time 

PCR analysis. The mRNA level in the innervated condition 

was rapidly increased (ten times) at 1 week after surgery, 

and gradually decreased to the original level, whereas the 

increase in the denervated condition was mild (four times) 

and delayed (at two weeks). Thus, we speculated that denervation 

causes some effects on the regeneration of skeletal 

muscles, and the lower expression of proteins relating to 

quality control may be involved in the incomplete regeneration 

under denervation of skeletal muscle. 

M830. Rapid Magnetic Stimulation Versus Conventional 

Physiotherapy in Bell’s Palsy 

Devathasan Gobinathan and Lea Dosado; Singapore, 

Singapore 

We compared the use of rapid magnetic stimulation (RMS) 

therapy in 23 subjects with 22 others who elected conventional 

electrical, muscle stimulation (CS) treatment and acupuncture. 

All patients had acyclovir for 7 days with varying 

dose of steroids. After one year the parameters assessed were 

the strength of frontalis, orbicularis oculis, zygomaticus, 

orbicularis oris and for autonomic dysfunction–score 2 

being normal, 1 partial recovery and 0 no recovery or distressing. 

Patients did their own scoring. 

RMS was administered in 1 or 2 sessions ten times or 

days during the acute phase at 15Hz, 1500 pulses, 5 second 

pause, 50 in each of 30 trains at strength of 30 to 35A/us. 

Stimulation sites were at the mastoid and more distally at 

the facial area over the affected muscles away from the eye 

globe. 

Significant difference was noted in favor of RMS ( p < 

0.004, t ¼ 7.1, df ¼ 21 mean score RMS ¼ 9.9 and CS ¼ 

6.0). 

High dose steroids may have introduced a bias element 

in favor of the RMS group as they received much less 

steroids. 

Study supported by: NIL 

M831. Establishing a Rare Disease Center in China: The 

Periodic Paralysis Program 

Qing Ke, Benyan Luo, Ming Ming, Michael Hanna, Jacob 

Levitt, Barbara Herr and Robert C. Griggs; Hang Zhou, Zhe 

Jiang, China; Rochester, NY; London, United Kingdom and 

New York, NY 

Objective: To establish a rare disease referral center in China. 

Methods: Analyze ways periodic paralysis (PP) patients 

reached Chinese centers and compare this process with US 

and UK approaches. 



Results: 116 patients were evaluated with PP in two center 

(Beijing and Hangzhou) from 2003–2009. Only 20% 

patients were local, the remaining patients traveled distances 

without referrals from other areas. The deciding factor in 

seeking care largely depended on patients’ choice. In contrast, 

at University of Rochester Medical Center, over 90% 

of patients are referred from physicians throughout the 

country by virtue of published information concerning physician 

expertise or on the basis of referrals from a patient 

advocacy group. In UK, a single center, supported by the 

National Health Service, provides assessment and genetic 

testing for all patients in the country. 

Conclusions: Currently, PP patients in China are practising 

self-referral to a larger center for medical care. Most of 

them are evaluated in brief outpatient visits. With the rapid 

development of computer access and expertise coupled with 

a website locating one or more centers, patients in the country 

will be able to identify specific specialty centers. 

Study supported by: NIH F05NS065682 

M832. Effect of Fatigue on Pulmonary Function Studies 

in ALS Patients 

Kathleen S. Alfuth, Ericka P. Simpson and Aparajitha K. 

Verma; Houston, TX 

Objective: To measure change in pulmonary function tests 

(PFTs) as relates to time of day and fatigue in ALS patients. 

Methods: Cross-sectional cohort study evaluating % 

change in PFTs from early to late-day and correlation with 

fatigue severity scale (FSS), disability (ALSFRS-r), bulbar 

compromise (ALSFRS-r bulbar subscale). PFT measures 

include forced vital capacity (FVC), maximal inspiratory 

pressure (MIP), maximal expiratory pressure (MEP) at times 

T1 and T2 (5–9hrs after T1) in sitting and supine positions. 

Results: Eight patients were recruited [age 56.9 6 7.9 

yrs, 75% female, 25% bulbar onset, FSS 42.3 6 14.7, 

ALSFRS 35.5 6 8.1, ALSFRS-r bulbar 12.8 6 1.7]. Highest 

of three recorded attempts were used to calculate average 

change between T1 and T2. FVCsitting -13% 6 27%; FVCsu pine -13% 6 28%; MIPsitting þ7% 6 14%; MIPsupine -9% 

6 20%; MEPsitting -3 6 17%; MEPsupine þ7% 6 29%. 

Correlation analysis showed a trend association with increasing 

fatigue (FSS) and decrease in MIPsupine (p ¼ 0.13). 

Conclusion: Preliminary data suggests PFTs in ALS may 

significantly change from early to late-day and this may correlate 

with fatigue. This study is ongoing to determine significance 

of the results and possible impact upon 

management. 

Study supported by: Methodist Neurological Institute 

Employment 

M833. Monomelic Amyotrophy – A Rare Case 

Presentation 

Amtul Farheen, Manpreet Multani, Aiesha Ahmed, Raji 

Grewal and Raji Grewal; Edison, NJ 

A 26 year-old right handed Indian man presented with 

weakness and atrophy of the left upper extremity over seven 

years. He denied pain, numbness, diplopia, dysphagia, ptosis, 

muscle cramps, fasciculations and neck pain. There was 

no history of febrile illness, poliomyelitis, and exposure to 

toxins or heavy metals. On examination there was atrophy 

of entire left upper extremity and weakness (power of 4/5) 

of left deltoid, biceps, triceps, and wrist flexion and extension, 

and hand muscles. Motor exam was normal in other 

extremities. Sensation and cranialnerve examination were 

normal. Serum electrolytes, thyroid panel, ESR, CPK, HIV 

were normal. MRI of C-spine showed vertically oriented 

62 

signal alteration in left mid anterior spinal cord from C 3- 

C7. Electrodiagnostic testing revealed normal nerve conduction 

studies. EMG showed positive sharp waves, fibrillation 

potentials and motor unit action potentials of increased amplitude 

and duration with reduced recruitment in almost all 

of the muscles in left upper extremity. 

Monomelic amyotrophy is a rare disorder presenting with 

atrophy and weakness restricted to one limb. The benign 

nature of Monomelic amyotrophy helps distinguish it from 

other lower motor neuron disorders like ALS. 

M834. Botulism: A Case Report 

Peter Struck, Amtul Farheen and Sushanth Bhat; New 

Brunswick and Edison, NJ 

A 3 month old boy presented with 15 days of constipation 

and 5 days of progressive generalized weakness. Parents 

noted weak cry, poor appetite, lethargy, and generalized 

muscle weakness. No respiratory or ocular symptoms were 

reported. No history of sick contacts. The baby was full 

term, born by c-section but otherwise healthy. On examination 

facial weakness, weak gag,head lag and diffuse hypotonia 

were noted. There were no focal motor deficits. Deep 

tendon reflexes were 1 in bilateral upper extremities and 2þ 

in lower extremities. Babinski was positive bilaterally. Blood 

tests including CBC, CMP, UA, Newborn Screen were normal. 

Motor nerve conduction studies revealed normal conduction 

velocity, slightly decreased amplitudes. RNS of right 

median nerve at 3 Hz showed 9 % decrement and at 20 Hz 

showed 30 % increment. EMG showed fibrillations at rest, 

short duration low amplitude MUAPs. Stool revealed botulinum 

toxin. Baby was diagnosed to have botulism and was 

treated with Baby Botulism Immunoglobulin. After treatment 

patient was noted to have a stronger cry, improved 

appetite and improved weakness. 

The diagnosis of botulism should be considered in infants 

and children with hypotonia. The detection of toxin in the 

stool and EMG are diagnostic. 

M835. A Novel Pentameric Thiophene Derivative (p- 

FTAA) Strongly Highlights Clusters of Paired Helical 

Filaments Containing Phosphorylated Tau in Sporadic 

Inclusion-Body Myositis (s-IBM) Muscle Fibers 

Therése Klingstedt, Anna Nogalska, Cristiane Blechschmidt, 

Stefan Prokop, Frank L. Heppner, King W. Engel, Valerie 

Askanas and Peter K.R. Nilsson; Linköping, Sweden; Los 

Angeles and Berlin, Germany 

p-FTAA is a fluorescent amyloid-specific ligand that can be 

used for the detection of protein aggregates in vivo as well 

as pre-fibrillar and fibrillar species in vitro. The probe spectrally 

discriminate amyloid-beta (Ab) plaques and phosphorylated 

tau (p-tau)-containing neurofibrillary tangles in Alzheimer’s 

disease (AD) brain. 

Muscle biopsies of s-IBM, the most common muscle disease 

of older persons, have several pathologic similarities 

with the AD brain, including intra-muscle fiber accumulations 

of Ab and p-tau-containing clusters of paired helical 

filaments (PHFs). We now report that in all 6 s-IBM muscle 

biopsies studied, fluorescence evaluation of p-FTAA 

staining, using excitation 450–490 nm and emission 520 

nm, revealed a strong and specific signal from inclusions 

positive with AT100 antibody recognizing p-tau and p62 

antibody indicating clusters of PHFs. All 12 normal and 

disease-control muscle biopsies were negative. 

Our study demonstrates a novel staining method for 

detection of p-tau inclusions in s-IBM muscle fibers. The 

quick staining protocol together with the high signal-to- 


noise ratio makes this probe an excellent tool for the diagnosis 

of s-IBM. 

Neurogenetics 



M1002. Clinico-Genetic Characterization of a Large 

Italian Cohort with Primary Spastic Paraplegia 

Andrea Martinuzzi, Mariateresa Bassi, Grazia D’Angelo, Sara 

Bonato, Gabriella Paparella, Olimpia Musumeci, 

Mariagiovanna Rossetto, Marianna Fantin, Francesca Peruch, 

Alessia Arnoldi, Claudia Crimella, Erika Tenderini, Paolo 

Bonanni, Vanessa Casanova, Giovanni Meola, Giacomo Comi, 

Antonio Toscano and Nereo Bresolin; Conegliano, TV, Italy; 

Bosisio Parini, LC, Italy; Messina, Italy and Milano, Italy 

Background: Diagnostic definition of hereditary spastic 

paraplegias (HSPs) is complicated by the wide genetic 

heterogeneity. 

Objectives: Establish in a large cohort of Italian HSP 

patients the relative frequency of the various forms, providing 

indications for an efficient diagnostic algorhythm. 

Methods: 478 index cases (72 familial, 98 pure, 380 

complicated) HSP were clinically and molecularly assessed. 

Results: 80 cases were molecularly defined. SPG4 was 

the most frequent form (55%), followed by SPG11 (16.6%) 

SPG7 (9%), SPG10 (8,8% ) and 5 (5.1%). SPG3a and 

SPG31 were rarer (2.5%). No mutations were identified in 

SPG6, 8, 13, 20, 21, 35, 48. There was wide inter and 

intrafamilial variation. Neurophysiology showed invariably 

increased central conduction time at lower limbs. Axonal 

polyneuropathy was detected in some SPG3a, 5, 10, 11, 17 

and SPG4 (15%). MRI showed abnormalities in SPG 5, 

10, 11 and 15. 

Conclusion: Frequency of SPG forms within this cohort 

of Italian HSPs confirms the prevalence of SPG4, reveals 

the recurrence of SPG11 and 7 and the low frequency of 

SPG3a and 31. Once SPG4 and SPG11 are excluded, family 

history, neurophysiology and neuroimaging may direct 

the choice of genetic testing. 

Study supported by: Italian Ministry of Health 

M1003. A Study of ACE and ADD1 Gene 

Polymorphism in Extra and Intracranial Atherosclerosis 

in Patients with Ischemic Stroke 

Jayantee Kalita, Usha K. Misra, Sunil Kumar, Bishwanath 

Kumar, Bindu I. Somarajan and Balraj Mittal; Lucknow, 

Uttar Pradesh, India 

Objective: To evaluate the hypertensive gene (ACE and 

aADDUCIN) and MR angiography (MRA) abnormality in 

patients with ischemic stroke (IS). 

Method: 151 patients with MRI proven IS were subjected 

to clinical and biochemical stroke risk factors evaluation. 

Both intra(IC) and extracranial(EC) MRA were done 

and more than 50% stenosis was considered significant. 

ACE and aADDUCIN gene polymorphism was done in 

patients and 188 controls. 

Results: The patients’ median was 60years and 26.5% 

were females. MRA was abnormal in 77.5%; ECMRA in 

58.3% and ICMRA in 66.7%. The conventional risk factors 

were not different between the patients with and without 

MRA abnormalities. Presence of DD genotype 

(OR3.86,95%CI0.78-2.28,P ¼ 0.0001) and aADDUCIN 

GW genotype (OR2.05, 95%CI1.28-3.27,P ¼ 0.003) 

increased the risk of IS significantly compared to controls. 

63 

Both genotype and allele frequency of ACE and 

aADDUCIN however were not different between patients 

with and without ECMRA or ICMRA abnormality after 

adjustment of putative stroke risk factors. 

Conclusions: ACE DD genotype and D allele and 

aADDUCIN GW genotype and W allele were associated 

with IS compared to controls but not related to IC or 

ECMRA abnormalities. 

Study supported by: Study financially supported by Indian 

council of medical research, Governmernt of India 

M1004. Generation and Characterization of MeCP2_270 

Mutant Mice 

Chiranjeevi Bodda, Karolina Can, Liakath Ali Kifayathulla, 

Hope Yao Agbemenyah and Ashraf U. Mannan; Goettingen, 

Germany 

MeCP2 is a transcription factor that binds to methylated 

target DNA sequence. Loss-of-function mutations in 

MECP2 gene is the main cause for Rett syndrome (RTT). 

The R270X is one of the most frequent recurrent MECP2 

mutations among RTT cohorts. The R270X mutation 

resides within the TRD-NLS region of MeCP2 and causes a 

severe clinical phenotype with increased mortality as compared 

to other mutations. To evaluate the functional role of 

R270X mutation, we first generated a transgenic mouse 

model expressing MeCP2_270 (human mutation equivalent) 

by BAC recombineering. The generated transgenic mice 

were crossed with Mecp2 knockout mice to produce 

Mecp2_R270X knockin mice. The expression pattern of 

MeCP2_270 was similar to that of endogenous MeCP2. 

Strikingly, MeCP2_270 localizes in the nucleus, contrary to 

the conjecture that the R270X could cause disruption of the 

NLS. Quantitative expression analysis of MeCP2 target 

genes revealed a similar trend in gene regulation pattern as 

observed in knock-out mice, however the level of differential 

regulation was variable. At the present, we are performing 

behavioral analysis to characterize the phenotype of the 

mice. Also, we are evaluating neuronal parameters to determine 

impairment in neuron development/differentiation 

and synaptic network dysfunction. 

Study supported by: DFG-Research Center Molecular 

Physiology of the Brain. Germany 

M1005. Identification of Epigenomic Modifications as 

Biomarkers for Amyotrophic Lateral Sclerosis 

Claudia Figueroa-Romero, Junguk Hur, Yu Hong, John S. 

Lunn, Crystal Pacut, Colin E. Delaney, Raymond Yung, Brian 

Callaghan and Eva L. Feldman; Ann Arbor, MI 

ALS is a progressive and terminal neurodegenerative disease 

leading to irreversible motor neuron degeneration. The high 

incidence of sporadic ALS (sALS) suggests that long-term 

influences of environmental factors on the genome may 

have a major impact in disease development. We hypothesize 

that epigenetic modifications play a key role in the 

pathogenesis of sALS. DNA from postmortem spinal cord 

(SC) tissue from sALS patients/controls was subjected to an 

epigene discovery phase. We identified 4,080 autosomal 

CpG sites with a significantly different methylation status in 

sALS compared to control (False Discovery Rate < 5%). 

The biological functions of ‘extracellular region’, ‘defense 

response’ and ‘immune response’ were highly associated 

with these differentially methylated sites. Furthermore, differential 

expression of six genes involved in human cytokine 

activity was observed in ALS/control human lymphocytes 

and/or SC. We have identified methylation profiles in diseased 

tissue, which might parallel those in blood as 



epigenetic biomarkers of sALS. This could enable early-stage 

diagnosis and therapeutic interventions to increase survival 

outcomes in sALS patients. 

Study supported by: The A. Alfred Taubman Medical 

Research Institute. 

M1006. A Drosophila Model of Williams Syndrome 

Ralph J. Greenspan and Jenee Wagner; La Jolla, CA 

Social cognition is a major frontier in neuroscience, and 

Williams Syndrome (WS) is a condition affecting social cognition 

associated with hemizygous deletions of 28 genes 

on human chromosome 7. The genetics of WS is apparently 

simple, consisting of a deletion of a defined chromosome 

segment, but complex because the deletions remove a block 

of genes, thus obscuring the contributions of interactions 

among subsets of the genes. These interactions are directly 

addressable in Drosophila, where ten of the genes in WS 

deletions have bona fide homologs in Drosophila, and one 

more has a more distant homolog, and all have extant mutant 

alleles. 

We are mapping the gene network interactions affected 

by different combinations of WS homologs in the fruit fly, 

capitalizing on the extensive conservation with humans of 

their genes and gene networks. Results from these experiments 

indicate that fly homologs of LIMK1 and CLIP190, 

among others, have substantial effects on behavior individually, 

but their contributions are not additive in combination 

with other WS genes. This non-linearity is reflected in the 

effects of these manipulations on gene network activities. 

Supported by NSF IOS-0840717 and NIH 

1R21EY020629-01 grants to R.J.G. 

Study supported by: NIH, NSF 

M1007. Clinical and Pathological Features of Progressive 

Supranuclear Palsy with Family History 

Shinsuke Fujioka, Avi Algom, Audrey Strongosky, Dennis W. 

Dickson and Zbigniew K. Wszolek; Jacksonville, FL 

Objective: To compare the clinical and pathological characteristics 

of progressive supranuclear palsy (PSP) patients 

with and without a family history of parkinsonism or PSP. 

Methods: We reviewed autopsy case records from Mayo 

Clinic Florida Brain Bank to retrieve PSP cases with positive 

family history of neurodegenerative disease, defined as PSP 

or parkinsonism, as well as PSP cases without a family history 

of neurologic disease. 

Results: This study was based on 375 pathologically confirmed 

PSP cases, of which 56 had a family history of PSP 

or parkinsonism. Among these, 19 patients had multiple 

affected family members with PSP, parkinsonism, or other 

neurodegenerative conditions, such as dementia or tremor. 

There was a trend (p ¼ 0.128) for PSP with a positive family 

history to have more atypical clinical features and diagnoses 

other than PSP, such as corticobasal syndrome or 

frontotemporal dementia. They also tended (p ¼ 0.131) to 

have more frequent atypical PSP pathology. 

Conclusion: PSP patients with a positive family history 

share many clinical and pathologic features with PSP 

patients without family history, but in general they tend to 

be atypical in clinical presentation and in final pathology. 

Study supported by: ZKW is partially supported by the 

NIH/NINDS 1RC2NS070276, NS057567, P50NS072187, 


(MCF #90052018 and MCF #90052030), and gift 

from Carl Edw and Bolch, Jr., and Susan Boss Bolch (MCF 

#90052031/PAU #90052). FS is partially supported by 

64 


(MDF #90052018). 

M1008. Aberrant Methylation by Mutations of DNA 

Methyltransferase 1 Cause Peripheral and Central 

Axonal Degeneration 

Christopher J. Klein, Maria V. Botuyan, Yanhong Wu, 

Christopher J. Ward, Garth A. Nicholson, Simon Hammans, 

Hiromitch Yamanishi, Adam R. Karpf, Douglas C. Wallace, 

Mariella Simon, Cecilie Lander, Julie M. Cunningham, Glenn 

E. Smith, William J. Litchy, Benjamin Boes, Elizabeth J. 

Atkinson, Sumit Middha, P. James Dyck, Joseph E. Parisi, 

Georges Mer, David I. Smith and Peter J. Dyck; Rochester, 

MN; Sydney, Australia; Southampton, United Kingdom; 

Hyogo, Japan; Buffalo, NY; Irvine, CA; Herston, Australia 

and Indianapolis, IN 

DNA methyltransferase 1 (DNMT1) is an essential component 

of genomic methylation. Neural gene expression, DNA 

mismatch repair and cell cycle regulation are all influenced 

by DNA methylation. Here we show mutations in DNMT1 

cause both central and peripheral axonal degeneration in 

one form of hereditary sensory and autonomic neuropathy 

with dementia and hearing loss. Two American, one European 

and one Japanese kindreds were studied. Next generation 

sequencing was utilized to identify two DNMT1 mutations, 

c.A1484G(p.Y495C) in American and Japanese 

kindreds and c.1470TCC-1472ATA(p.D490E-P491Y) in a 

European kindred. All mutations are within the DNA targeting 

sequence domain of DNMT1. Functional analysis 

shows premature degradation and reduced methyltransferase 

activity. Mutant proteins lost heterochromatin binding ability 

during the G2 cell cycle, leading to global hypomethylation 

measured by mass spectrometry analysis. Methylation 

of satellite 2 repetitive elements is preferentially decreased. 

Global hypomethylation and regional hypermethylation is 

shown in affected persons, a pattern commonly seen in 

unregulated cancer cells, suggesting post mitotic neural cells 

may undergo axonal degeneration by loss of cell cycle arrest. 

The discovered mutations in DNMT1 provide a new framework 

for the study of neurodegenerative diseases. 

Study supported by: NINDS K08 award 

M1009. Common and Distinct Associations of HLA- 

DRB1 and -DPB1 Alleles with Neuromyelitis Optica and 

Multiple Sclerosis in Japanese 

Satoshi Yoshimura, Noriko Isobe, Takuya Matsushita, Tomomi 

Yonekawa, Katsuhisa Masaki and Jun-ichi Kira; Fukuoka, 

Japan 

Background: A distinctive association between human leukocyte 

antigen (HLA) gene alleles and each clinical subtype, 

opticospinal multiple sclerosis (OSMS) and conventional 

MS (CMS), has been reported in Asians. However, patients’ 

sample sizes were relatively small in the previous studies. 

OSMS is now suggested to be the same as a relapsing form 

of neuromyelitis optica (NMO) in Westerners. 

Objective: To clarify the association of HLA-DRB1 and - 

DPB1 alleles with NMO and non-NMO MS phenotypes in 

a large series of Japanese patients. 

Methods: Genotyping of HLA-DRB1 and -DPB1 alleles 

was performed in 288 MS patients including 83 who fulfilled 

the NMO criteria, and 367 healthy controls (HCs). 

Results: DRB1*0901 frequency was significantly lower in 

both NMO (p corr ¼ 0.0003, OR:0.133, 95%CI:0.048– 

0.374) and non-NMO MS patients (p corr

OR:0.301, 95%CI:0.181–0.499) as compared with HCs. 

Susceptibility alleles for non-NMO MS are DRB1*0405 

(p corr ¼ 0.0189, OR:1.867, 95%CI:1.300–2.683) and 

DPB1*0301 (p corr ¼ 0.0022, OR:3.328, 95%CI:1.747– 

6.337) while those for NMO are DRB1*1602 (p corr ¼ 

0.0028, OR:12.942, 95%CI:3.355–49.923) and 

DPB1*0501 (p corr ¼ 0.0265, OR:2.608, 95%CI:1.412– 

4.816). 

Conclusion: There is a common resistant DRB1 allele 

between NMO and non-NMO MS while susceptibility alleles 

are distinct between the two conditions in Japanese. 

Study supported by: This work was supported in part by 

the Health and Labour Sciences Research Grant on Intractable 

Diseases (H20-Nanchi-Ippan-016) from the Ministry of 

Health, Labour and Welfare, Japan, and the grant-in-aid (B; 

no. 22390178) from the Ministry of Education, Culture, 

Sports, Science and Technology, Japan. 

M1010. Computer Simulations of Striatal Atrophy and 

Age at Onset of Huntington’s Disease 

Steven D. Edland and Jagan Pillai; La Jolla, CA 

Beyond polyglutamine repeat length on the huntingtin 

gene, little is known about predictors of age at onset of first 

symptoms of Huntington’s disease (HD). Cajevec et al. 

(Neurogenetics, 2006) proposed that variability in age at 

onset given repeat length could be explained by the random 

nature of striatal cell loss under the ‘‘one-hit’’ model of HD 

neurodegeneration proposed by Clarke et al. (Nature, 

2000). Alternatively, the rate of neuronal loss in HD could 

vary person to person due to environmental and background 

genetic influences affecting rate of neuronal loss or affecting 

susceptibility to the extent of cell loss. To investigate this, 

we simulated striatal neuron loss in HD as proposed by 

Cajevec et al. and under these alternative models. We found 

that stochastic cell death is unlikely to explain an appreciable 

fraction of variability in age at onset, while models 

with random rates of neuronal loss and susceptibility recapitulated 

empirical HD survival distributions perfectly. We 

conclude, contrary to Cajevec et al., that age at onset is a 

valuable phenotypic endpoint for investigations of environmental 

and background genetic factors affecting the expression 

of HD. 

Study supported by: NIH/NIA AG00513 and 

AG034439. 

Trauma/Injury 


M1101. A Human Natural IgM Drives Axon Outgrowth 

Xiaohua Xu, Arthur Warrington, Brent Wright, Allan Bieber, 

Virginia Van Keulen, Larry Pease and Moses Rodriguez; 

Rochester, MN 

We demonstrate that a human natural IgM, HIgM12 promotes 

axonal outgrowth by mechanisms coupling lipid raft 

to microtubule dynamics. Serum-derived human IgMs have 

been shown to support neurite extension of primary cerebellar 

granule neurons. In the current study with primary hippocampal 

neurons, we showed that a recombinant form of 

one of these IgMs, rHIgM12 regulated kinetics of membrane 

lipids. rHIgM12 bound to neuron surfaces and 

induced clustering of cholesterol and ganglioside, GM1. 

Following membrane association, rHIgM12 segregated into 

two pools, one associated with lipid raft fractions, and the 

other with detergent insoluble pellet composed of cytoskeletons. 

Moreover, rHIgM12 affected microtubule dynamics. 

65 

rHIgM12 co-localized with microtubules and was co-immunoprecipitated 

with b3-tubulin. rHIgM12 promoted tyrosination 

of a-tubulin. Finally, rHIgM12 enhanced polarized 

axon outgrowth when presented as substrate. Our results 

indicate that there exists a repertoire of natural IgMs with 

the potential to regulate membrane lipid and microtubule 

dynamics required for axonal outgrowth. These findings 

also identify a bioactive molecule that may be utilized to 

treat neurologic diseases. 

Study supported by: This work was supported by grants 

from the NIH (R01 GM092993, R01 NS024180, R01 

NS03219, R01 NS048357) and the National Multiple Sclerosis 

Society (CA 1011-A8). We also acknowledge with 

thanks support from the Applebaum and Hilton Foundations 

and the Minnesota Partnership Award for Biotechnology 

and Medical Genomics. 

M1102. Computed Tomography Characteristics of 

Pediatric Traumatic Brain Injury 

Korak Sarkar and Kia Shahlaie; Sacramento 

Little is known about the differences in CT findings 

between adult and pediatric TBI populations. A retrospective 

analysis of 1206 consecutive non-penetrating TBI 

patients treated at a Level 1 trauma center from August 

2008 to January 2011 was performed. Admission CT scan 

were evaluated for skull fractures, ICH, midline shift, and 

basal cistern compression. Of 1206 patients in our registry 

349(28.5%) were pediatric. Demographics and injury severity 

distribution were similar between the two populations 

(p> 0.05). The distribution of CT findings, however, was 

significantly different. Pediatric TBI patients were more 

likely to be associated with skull fractures (57.0% vs 23.4%) 

and EDH (17.2% vs 9.7%), and less likely to be associated 

with contusion, SDH, SAH, or basal cisterns compression 

(p


change in GOS or GOS-E between 3 and 6 months. The 

1- and 3-month NOS-TBI correlated with the 3- and 6month 

GOS, GOS-E and DRS (all p


Induction of anesthetic coma was necessary in 49/53 SE 

incidents. Patients required anesthetic agents for 9.6 (4, 0– 

60) days. Eighteen died, withdrawal of support preceded 

death in 13. Compared to a pre-morbid modified Rankin 

scale (MRS) of (2.09 6 1.6) 27 patients alive at 3–6 

months following hospital discharge had a MRS of (3.81 6 

1.52) (p


Imaging Procedures: Whole brain DTT was performed 

using streamtube tracing and culling techniques. DTT of a 

normal subject was obtained as a control. 

Results: Conventional brain MRI displayed complete absence 

of the corpus callosum in both cases. Whole brain 

DTT showed no corpus callosum fibers crossing the midline 

in either ACC case. Instead, robust fiber bundles passing 

through middle cerebellum peduncles via transverse pontine 

fibers were seen. 

Conclusion: DTT was useful in visualizing expected and 

unexpected alterations of white matter fiber connections in ACC. 

Study supported by: National Science Foundation 

(1017921,1018769, 1016623) 

M1302. Neurofibromatosis 1 (NF1) Vasculopathy in 

Children – An Emerging Entity 

Partha S. Ghosh, A.D. Rothner and Manikum Moodley; 

Cleveland, OH 

Objective: Vasculopathy in children with NF1. 

Methods: Retrospective review of children with NF1 

from 2000- 2010 at Cleveland Clinic. NF1 associated vasculopathy 

divided into: (1) Cerebrovascular, (2) Cardiovascular 

(3) Other. 

Results: Of 398 patients, 26 (6.5%) had vasculopathy. 

Group1: Ten (2.5%) had cerebrovascular abnormalities 

(mean age 12.8 years; 5 males). Presentation- headache 

(4), seizures (1), asymptomatic (5): all had normal neurological 

examination. MRA brain: Moyamoya disease-4, stenosis/occlusion 

of Internal carotid artery-3, Middle cerebral 

artery stenosis-2, Posterior cerebral artery stenosis-1. Ischemic 

changes noted in 1. On follow-up (mean 5.2 years); 

1 died of brain tumor, others did not develop stroke. One 

had encephaloduromyosynangiosis for moyamoya. 

Group2: Fifteen (3.7%) had cardiovascular abnormalities 

(mean age 6.6 years; 8 males). Presentation: hypertension (2), 

shortness of breath (2), chest pain (1), syncope (1), murmur 

(6). Cardiac lesions: Pulmonary stenosis-5, Coarctation-4, mitral-valve 

prolapse-1, supra-valvular aortic stenosis-1, anomalous 

right coronary artery-1, pulmonary artery hypoplasia-1. 

On follow-up (mean 4.6 years), 3 had surgical procedures. 

Group3: Two girls had renal artery stenosis (RAS); 1 with 

coarctation. Both were hypertensive: 1 underwent RAS repair. 

Conclusion: Children with NF1 should be carefully 

screened for vasculopathy. 

M1303. Macro CK-1 a Cause of Spuriously Elevated CK 

Associated with Leukoencephalopathy in an Infant 

and John B. Bodensteiner; Phoenix, AZ 

Macro CK-1 is a complex formed by the Creatine Kinase isoenzyme 

BB and monoclonal IgG. The complex is not formed 

by abnormal CK enzyme but by an autoimmune reaction 

and occurs in about 1% of patients studied. First identified 

as a cause of spurious elevation of the total CK in patients 

suspected of myocardial infarction in the 1970s the test has 

been largely replaced by the measurement of Troponin levels. 

We present an infant with delayed milestones and persistently 

elevated CK measurements ( 1000–4000 IU) normal EMG 

and brisk DTRs. Suspicion that this was not a myopathy 

prompted the measurement of CK isoenzymes and brain 

imaging which showed the presence of Macro CK-1 and 

extensive signal abnormality of the cerebral white matter. 

Macro CK-1 has been associated with cancer, infection, myositis 

and heart disease, not described in association with leukoencephalopathy 

or in infants. Macro CK-1 may be a cause 

of elevated total CK in patients without primary muscle dis- 

68 

ease. The significance of the Macro-CK in relation to the 

Leukoencephalopathy in this patient or the frequency of this 

association is completely unknown. 

Study supported by: Supported by The William Pilcher 

Endowment for Pediatric Neurology 

M1304. Pharmaceuticals in the Environment: A Focus 

on Neurological Medications 

and Ilene Ruhoy; Seattle, WA 

Pharmaceutical residues in the environment have been an 

issue of increasing interest amongst environmental scientists, 

toxicologists, and policy makers. Physicians have recently 

expressed concern regarding the potential ill effects on 

humans from chronic exposures to these active pharmaceutical 

residues (APIs). 

Research has demonstrated the potential for neurological 

effects from exposure to the multitude of pharmaceutical 

residues identified in our environment, specifically in our 

water systems. Studies in aquatic organisms have revealed 

altered brain size, poor neuronal growth, and neuropathy. In 

addition, many of the pharmaceuticals that have been identified 

are centrally acting. Carbamazepine is one drug of significant 

concern in that it is largely refractory to most treatment 

technologies and indeed it has been identified in all 

water sampling studies. Benzodiazepines are another common 

culprit that are found with increasing frequency and 

are ubiquitous in its prescription patterns. 

This presentation will discuss the known and unknown 

with regards to medications commonly prescribed in the 

neurological setting and environmental presence. It will correlate 

research on aquatic wildlife with the potential for 

human health effects from exposures to these medications 

and will recommend actions to minimize human exposures. 

Rehabilitation and Regeneration 

M1401. Human Induced Pluripotent Stem Cell-Derived 

Neural Progenitor Grafting into Rat Hippomcapus 

Alison L. Althaus, Yu Liu, Duriel Hardy and Jack M. Parent; 

Ann Arbor, MI 

Induced pluripotent stem cells (iPSCs) offer potential 

advantages as a neural progenitor cell (NPC) source for regenerative 

therapy, including readily available supply, vast 

differentiation potential and autologous grafts that obviate 

the need for immunosuppression. To begin developing 

iPSC-based transplantation therapy, we injected human 

iPSC-derived NPCs into neonatal rat hippocampus to determine 

their differentiation potential. We also examined how 

the degree of in vitro differentiation influenced graft behavior. 

GFP- or mCherry-labeled human iPSCs were neurally 

differentiated and transplanted (10^5 cells) into postnatal 

day 4 rat hippocampus either at the primitive neural rosette 

stage or after 3-day neurosphere differentiation. Grafts were 

examined after 2, 4 or 8 weeks by immunostaining for 

human nuclear antigen or GFP/mCherry and neuronal or 

glial markers. Grafts generated GFAPþ glia and TuJ1þ 

neurons at all time points. Neural rosette grafts tended to 

form large neural tube-like structures that deformed host tissue. 

Neurosphere grafts dispersed more widely and gave rise 

to mature-appearing neurons, some of which expressed a pyramidal 

cell marker (Tbr1). These findings suggest that 

iPSCs autografts may be useful for treating brain disorders. 

Further work is needed to determine optimal NPC differentiation 

states for grafting. 

Study supported by: NIH NS065450 



M1402. Local Molecular Manipulation and Peripheral 

Nerve Regeneration 

Douglas W. Zochodne, Kimberly J. Christie, 

Christine A. Webber, Chu Cheng and Jose A. 

Martinez; Calgary, AB, Canada and Edmonton, 

AB, Canada 

Regeneration of peripheral neurons following injury or 

neuropathy is more challenging than usually assumed. We 

describe new molecular players that may offer insights into 

early axon outgrowth, the initiating step in regeneration. 

After complete nerve transection, axon outgrowth requires 

an intimate molecular exchange with partnering Schwann 

cells (SCs). Using an accessible conduit to connect the 

stumps of transected peripheral nerves in rats, we show 

that several forms of intervention, including local siRNA 

knock down, can alter regenerative outcome: (i) CGRP, an 

axonally synthesized peptide, signals local SCs to proliferate 

in order to support axon growth: knockdown interrupts 

regeneration. (ii) PTEN, a tumour suppressor molecule, is 

a regenerative roadblock that attenuates growth factor support 

of regeneration: knockdown dramatically enhances 

axon outgrowth. (iii) DCC and Unc5H are netrin facilitatory 

and inhibitory developmental pathfinding receptors 

respectively that are re-expressed in regenerating adult SCs: 

DCC knockdown attenuates adult regeneration whereas 

Unc5H knockdown enhances it. Local molecular manipulation 

can have significant impacts on the critical initiating 

step of peripheral nerve regeneration by eliminating roadblocks 

or upregulating growth signals. The approach may 

offer new forms of intervention for irreversible peripheral 

nerve lesions. 


Research, Alberta Heritage Foundation for Medical 

Research 

M1403. Very Early Gait Training after Acute Stroke; a 

Dose-Escalation Study 

Randolph S. Marshall, Ying K. Cheung, Clare Bassile, Laura 

A. Evensen, Roujie Chen, Veronica Perez, Ronald M. Lazar 

and Bernadette Boden-Albala; New York 

Intensive rehabilitation within days of stroke may benefit 

from an altered inhibition/disinhibition environment which 

promotes neuroplasticity. Conversely, early rehab may be 

impeded by excitotoxicity, medical co-morbidities, logistics 

of diagnostic testing, and low motivation. 

We used a stepwise sequential probability ratio test 

(sSPRT) with 6 tiers to determine the maximal tolerated 

dose of standard gait training at 24–96 hours after ischemic 

or hemorrhagic stroke. A multidisciplinary acute stroke/ 

rehab team assessed cardiac and neurological safety outcomes. 

Inclusion criteria: NIHSS leg motor or ataxia 1 

and prestroke Rankin 3. 

After 28 patients the study met the sSPRT algorithm 

requirements of dose-escalation to the highest tier, with 5 of 

6 patients completing 60 min/day at 24–96 hours. Safety 

outcomes included 3 cardiac withdrawals (2 patients at tier 

3, 1 at tier 6), and 3 fatigue withdrawals (1 at tier 4, 1 at 

tier 5, 1 at tier 6). One patient failed to complete his 

assigned dose because of sedation for diagnostic testing. 

These preliminary data suggest that very early rehabilitation 

with gait training is safe and feasible beginning 

24–48 hours after stroke. Further study is required to establish 

efficacy of early rehabilitation intervention in 

acute stroke. 

Study supported by: This work was supported by a Pilot 

Grant Award through Columbia University’s CTSA. 

69 









winner. 


T1501. Amyloid-Beta Dynamics and Prevention Trials in 

Dominantly Inherited Alzheimer’s Disease 



Amyloid-beta is a key pathologic protein in Alzheimer’s disease(AD) 

and extensive research has started an era of clinical 

trials targeting amyloid-beta. Understanding the dynamics 

of amyloid-beta formation and clearance in the human 

CNS and the processes that lead to clinical disease are essential 

to design better clinical trials. Developing efforts to prevent 

AD in autosomal dominant mutation carriers may test 

the amyloid hypothesis, determine the timing of treatment, 

and lead the way to AD prevention. 

Amyloid-beta production and clearance rates were measured 

with stable isotope labeling kinetics. The Dominantly 

Inherited Alzheimer’s Network interim findings of clinical, 

cognitive, MRI, PET, CSF, and blood biomarkers were analyzed 

with respect to the expected age of onset. 

Amyloid-beta clearance rate is decreased by 30% in AD 

compared to controls. Changes in clinical, cognitive, MRI, 

PET, CSF, and blood biomarkers in autosomal dominant 

AD indicate the pathophysiologic cascade begins up to 20 

years before the expected age of onset. 

The pathophysiologic changes of AD can be specifically 

measured and targeted for clinical trials. Autosomal dominant 

AD prevention trials offer a unique opportunity to 

lead the way to effective treatments for all AD. 

Study supported by: NIH K-23-AG03094601, NIH R- 

01-NS065667, NIH 3P-01-AG02627603S1 (FACS), NIH 

1U-01-AG03243801 (DIAN), ADRC (P50 AG05681-22), 

HASD (P01 AG03991-22), WU CTSA award (UL1 

RR024992), Mass Spectrometry Resource (NIH 

RR000954), Eli Lilly research collaboration 

R.J.B. is a co-founder of a company (C2N Diagnostics) 

that has licensed a Washington University patent on some 

of the technology described in this abstract. 

T1502. Integrating Genome-Wide Association and 

Functional Validation To Understand Susceptibility for 

Alzheimer’s Pathology 

Joshua M. Shulman, Portia I. Chipendo, Lori B. Chibnik, 

Brendan T. Keenan, Dong Tran, Matthew A. Huentelman, 

Julie A. Schneider, Eric M. Reiman, Denis A. Evans, David 

A. Bennett, Mel B. Feany and Philip L. De Jager; Boston, 

MA; Chicago, IL and Phoenix, AZ 

Gene discovery in Alzheimer’s disease (AD) is limited by etiologic 

heterogeneity of dementia in cases and subclinical 

disease in controls. We have implemented a novel strategy 

using quantitative AD pathology as an outcome for genome-wide 

association (GWA) analysis. Candidate susceptibility 

genes were subsequently tested for functional 



validation in a Drosophila model, based on transgenic 

expression of human Tau. In a pilot study of 227 subjects, 

6 out of 15 genomic regions associated with AD pathology 

contained genes showing interactions with Tau neurotoxicity 

in vivo. In the full cohort of 651 autopsies, GWA identified 

a variant in PFTK1 (rs11563839) associated with neurofibrillary 

tangle burden (p ¼ 6 10 6 ), and this polymorphism 

was also related to episodic memory decline (p ¼ 

0.004) in 1600 prospectively-followed, elderly subjects. Significantly, 

in Drosophila, we find that both gain- and lossof-function 

of the PFTK1 ortholog, Eip63E, is associated 

with suppression and enhancement of Tau toxicity, respectively. 

Interestingly, PFTK1 encodes a cell cycle-related kinase 

strongly expressed in adult brain. Our strategy of integrating 

a GWA scan for pathologic phenotypes with 

functional validation in a model organism is likely to be a 

powerful approach for gene discovery in AD. 

Study supported by: National Institutes of Health/National 

Institute on Aging, Massachusetts Alzheimer’s Disease Research 

Center, the Harvard NeuroDiscovery Center, and the Clinical 

Investigator Training Program: Beth Israel Deaconess Medical 

Center–Harvard/MIT Health Sciences and Technology, in collaboration 

with Pfizer Inc. and Merck & Co. 

T1503. Brain Expression Genome-Wide Association 

Study (eGWAS) and Alzheimer’s Disease 

Nilufer Ertekin-Taner, Fanggeng Zou, High Seng Chai, Curtis 

S. Younkin, Julia Crook, V Shane Pankratz, Mariet Allen, 

Minerva M. Carrasquillo, Christopher N. Rowley, Otto 

Pedraza, Morad Ansari, Caroline Hayward, Igor Rudan, 

Harry Campbell, Ozren Polasek, Nicholas D. Hastie, Asha A. 

Nair, Sumit Middha, Sooraj Maharjan, Thuy Nguyen, Li 

Ma, Kimberly G. Malphrus, Ryan Palusak, Sarah Lincoln, 

Gina Bisceglio, Constantin Georgescu, Christopher P. Kolbert, 

Jin Jen, Zbigniew Wszolek, Maria Barcikowska, Sigrid B. 

Sando, Jan Aasly, Kevin Morgan, Clifford Jack, Ronald C. 

Petersen, Neill R. Graff-Radford, Alan Wright, Dennis W. 

Dickson and Steven G. Younkin; Jacksonville, FL; Rochester, 

MN; Edinburgh, United Kingdom; Split, Croatia; Zagreb, 

Croatia; Warsaw, Poland; Trondheim, Norway and 

Nottingham, United Kingdom 

Genetic variants that modify brain gene expression may also 

influence risk for CNS diseases, including Alzheimer’s disease 

(AD). We performed an eGWAS of 24,526 transcript 

levels measured in the cerebella of 197 ADs and 177 non- 

ADs with 313,330 SNP genotypes. After elimination of 

probes with a known SNP within their sequence, cis-SNP/ 

transcript level associations were sought. Corrections were 

made for technical and biological variables. Significant cerebellar 

associations were also tested in the temporal cortex of 

198 ADs and 193 non-ADs. Cerebellar expression was detectable 

for 69% of all transcripts. After accounting for 

technical variance, cis-SNPs explained 5–85% of the variance 

of >1,600 transcripts. There was an excess of significant 

associations. Substantial number of cis-SNP/transcript 

associations were significant in both ADs and non-ADs 

with similar direction and magnitude of effects. Many top 

cerebellar cis-SNPs were also detectable in the temporal cortex 

with similar effect sizes. We demonstrate that genetic 

factors influence brain expression for many genes, replicably 

across disease pathologies and tissue regions. eGWAS may 

be an excellent approach to identify candidate functional 

genetic variants implicated in AD. Top cis-SNPs are investigated 

for association with AD and its endophenotypes 

including plasma amyloid-b. 

Study supported by: R01 AG018023, P50 AG016574 

70 

T1504. Insulin-Like Growth Factor 1 (IGF-1) and Risk 

of Alzheimer’s Disease: The Framingham Study 

Andrew J. Westwood*, Alexa S. Baiser, Ramachandran S. 

Vasan, Tamara B. Harris, Ronenn Roubenoff, Aleksandra 

Pikula, Rhoda Au, Charles DeCarli, Philip A. Wolf and 

Sudha Seshadri; Boston, MA; Framingham, MA; Bethesda, 

MD and Sacremento, CA 

Objective: To relate serum IGF1 to risk of Alzheimer’s disease 

(AD) and to brain volumes in a dementia-free community 

sample. 

Background: IGF1 may be a biological link between lifestyle 

and risk of AD; exercise elevates and hyperinsulinemia 

depresses circulating IGF1. IGF1 levels correlate positively 

with cognition in older men and lower levels are reported in 

mouse models of AD. 

Method: Framingham participants (N ¼ 648, mean age 

79 6 4 yrs, 63% women) who had serum IGF1 measured 

(radioimmunoassay, coefficient of variation 4%) in 1990-94 

were followed prospectively for incident AD. Dementia-free 

survivors (N ¼ 161) underwent brain MRI in 1999–2005. 

Results: Mean IGF1 levels were 143 6 60lg/L. We 

observed 105 cases of incident AD over a mean follow-up 

of 9 6 4 years. In multivariable models adjusted for age-, 

sex-, APOE, plasma homocysteine and waist-hip ratio, each 

standard deviation increase in baseline IGF1 was associated 

with a 23% lower risk of AD (HR ¼ 0.77,95%CI:0.62- 

0.96;p ¼ 0.019). Persons with IGF1 levels in the lowest 

quartile had lower brain volumes (b 6 SD ¼ 0.6 6 0.28; 

p ¼ 0.033) cross-sectionally, and a 80% greater risk of 

developing AD (HR ¼ 1.80,95%CI:1.19–2.71;p ¼ 0.005) 

compared to others. 

Conclusions: Higher IGF1 levels may protect from clinical 

AD and subclinical brain atrophy. 

Study supported by: This work was supported by the Framingham 

Heart Study’s National Heart, Lung, and Blood 

Institute contract (N01-HC-25195) and by grants from the 


(R01 NS17950 to P.A.W.) and from the National Institute 

on Aging (R01 AG16495 to P.A.W., AG08122 to P.A.W., 

AG033193 to S.S., AG031287 to S.S., AG033040 to 

P.A.W., P30AG013846 to S.S.). Dr. Roubenoff is an employee 

of Biogen Idec, Inc, but reports no conflict of interest 

with the subject of this paper 

T1505. Components of Blood Pressure and Progression 








Background: The contribution of blood pressure (BP) components 

to the burden and progression of cerebral white 

matter hyperintensity (WMH) is poorly understood. We 

evaluated these associations in the population-based Atherosclerosis 

Risk in Communities (ARIC) cohort. 

Methods: 983 participants each underwent 2 brain MRIs 

10 years apart. Systolic (SBP) and diastolic BP (DBP) were 

measured at 4 study visits. Four BP components were examined 

as predictors of WMH progression: 1) mean arterial 

pressure (MAP); 2) pulse pressure (PP); 3) orthostatic hypotension 

(OH); and 4) 10-year change in BP. 

Results: Baseline (preceding MRI #1) MAP value predicted 

WMH progression (OR 1.39 (1.20–1.62), per 10 

mm Hg increase), but PP did not. Presence of OH did not 



predict WMH progression, but OH severity did (OR 1.21 

(1.02–1.42) per 10 mm Hg SBP decrease). Change in DBP 

over 10 years had a U-shaped relationship with WMH progression: 

extreme increases and decreases, independent of 

antihypertensive use, were both associated with greater 

WMH progression (p ¼ 0.007). 

Discussion: WMH progression is significantly predicted 

by MAP and extent of orthostatic SBP reduction. Significant 

changes over time in DBP, whether positive or negative, predict 

WMH progression, cautioning against simplified interpretations 

of blood pressure associations. 

Study supported by: The Atherosclerosis Risk in 

Communities Study is carried out as a collaborative study 

supported by National Heart, Lung, and Blood Institute 

contracts (HHSN268201100005C, HHSN268201100006C, 

HHSN268201100007C, HHSN268201100008C, HHSN2 

68201100009C, HHSN2682011000010C, HHSN2682011 

000011C, HHSN2682011000012C). 

T1506. Therapeutic and Preventive Effects of a Novel 

AD Vaccine 

Carmen Vigo, Ivan Cuevas, Lucia Fernandez, Valter 

Lombardi, Richard Manivanh and Ramon Cacabelos; 

Sunnyvale, CA and Bergondo, La Coruna, Spain 

A vaccine consisting of b-amyloid peptide (Ab) delivered in 

a liposomal matrix composed of phosphatidylcholine: phosphatidylglycerol: 

cholesterol: sphingosine-1-phosphate 

(EB101) was administered intraperitoneally for seven 

months to double transgenic mice (B6C3-Tg (APPswe,P- 

SEN1dE9)), before or after the AD pathology was developed. 

Ab plaques and neurofibrillary tangles were quantified 

by ELISAs and brain histology using specific antibodies. Basal 

immunological interaction between the T-cells in the 

affected hippocampal area and other immune activation 

markers, including glial fibrillary acidic protein (GFAP) 

(astroglia) and CD-45 (B-cells) were also studied. Both preventive 

and therapeutic vaccination with EB101 resulted in 

a marked inhibition of Ab deposits (from 60 to 90%), a 

reduction of neurofibrillary tangles (70 to 90%) and almost 

completely suppression of reactive gliosis as measured by 

GFAP immunoreactivity, consistent with a marked decrease 

in amyloidosis-induced inflammation. No external neurological 

deficits were observed as a result of EB101 immunization 

(limb paralysis or brain atrophy). Cognitive tests in 

these animals treated with EB101 also show a dramatic 

improvement in learning and psychomotor activity. The 

present results indicate that the immunization with EB101 

prevents and reverses AD neuropathology and underlined 

inflammation, thus warranting further studies. 

Study supported by: Euroespes Foundation 

T1507. MoCA vs. MMSE: Patterns of Cognitive 

Performance across Adult Lifespan in a Non-Clinical 

Sample 

Shea Gluhm, Charles Van Liew, Jody Goldstein, Guerry Peavy, 

Mark Jacobson, Stephanie Lessig and Jody Corey-Bloom; La 

Jolla, CA and San Diego, CA 

The Mini Mental State Examination (MMSE) is the most 

commonly used brief cognitive screening instrument; however, 

the Montreal Cognitive Assessment (MoCA) may be 

more sensitive to early cognitive dysfunction. The present 

study sought to compare age-related decline on these measures 

across the adult lifespan in a non-clinical sample. Performance 

on the MMSE and MoCA of 202, presumably 

normal, community-dwelling participants ranging in age 

from 20–89, divided by decade, were compared with 

71 

Wilcoxon Signed Ranks Tests. We found that, for the total 

sample, there were significant differences between MoCA 

and MMSE with regard to total score (p


neuronal excitability and synaptic transmission. Here, we 

used the glutamate sensing fluorescent reporter (Super- 

GluSnFR) developed by Roger Tsien’s laboratory to perform 

sensitive optical measurements of glutamate release in 

response to Ab. This probe provides both a temporal and 

quantitative fluorescent readout of glutamate concentration 

by imaging FRET-dependent changes. We transfected HEK- 

293T cells with SuperGluSnFr, and then co-cultured these 

‘glutamate-sensing cells’ with astrocytes. Nanomolar concentrations 

of oligomerized (but not non-oligomerized) Ab 

induced release of several hundred-micromolar glutamate 

from astrocytes. These levels of glutamate can cause synaptic 

loss and excitotoxic damage to neurons. Taken together with 

prior studies, our work suggests that the neurotoxic effects 

of Ab may be mediated at least in part by local release of 

glutamate from astrocytes. Additionally, we found that 

newer congeners of memantine (called NitroMemantines) 

are able to prevent toxic glutamatergic effects of Ab to a 

greater degree than memantine itself. 

Study supported by: NIH and ADAMAS Pharmaceuticals, 

Inc. 

Stuart Lipton is the named inventor on patents for memantine 

and NitroMemantine for neurodegenerative disorders 

that are licensed to Forest Laboratories, Inc. and ADAMAS 

Pharmaceuticals, Inc., respectively. Concerning memantine, 

Dr. Lipton participates in a Royalty Sharing Agreement 

administered by his former institution, Harvard Medical 

School/Children’s Hospital, Boston. 

T1510. Is Poststroke Dementia Related to Amyloid 

Deposition and Microglia Activation? 

Wolf-Dieter Heiss, Basia Radlinska, Jean-Paul Soucy, Ralf 

Schirrmacher, Alexander Thiel and Vladimir Hachinski; Cologne, 

Germany; Montreal, QC, Canada and London, ON, Canada 

Amyloid deposition typical for Alzheimer’s Disease might be 

a predisposing factor for poststroke dementia and might be 

aggravated by inflammation accompanying ischemic changes. 

The relationship between amyloid deposition, inflammatory 

reaction and development of poststroke dementia is studied in 

5 patients with first supratentorial ischemic stroke by MRI (at 

2 weeks and 5 – 7 months) and PET with 11 C-PIB for amyloid 

imaging and with 11 C-PK 11195 for inflammation at 6 

months, the clinical course is followed for 12 months. 

Preliminary results indicate a significant increase in global 

PIB uptake (SUVR > 1.5) in the entire cortex compared to 

cerebellum. This amyloid deposition usually was asymmetric 

with peak values in prefrontal (SUVR ¼ 2.29 6 0.325) or 

parieto-temporal areas (SUVR ¼ 2.02 6 0.202). Increased 

PK-uptake as a marker of microglia activation was found in 

areas around the infarct, but also in connecting fiber tracts 

and distant from the ischemic lesion. 

These preliminary data demonstrate amyloid deposition 

and increased microglia activation in patients after stroke. 

The relationship between these two pathological processes 

and their impact on the development and progression of 

cognitive impairment will be further investigated. 

T1511. Cycad Methylazoxymethanol Linked to DNA 

Damage, Cancer and Neurodegeneration 

Glen E. Kisby, Rebecca C. Fry, Michael R. Lasarev, Theodor 

K. Bammler, Richard P. Beyer, Mona I. Churchwell, Daniel R. 

Doerge, Lisiane B. Meira, Valerie S. Palmer, Ana-Luisa 

Ramos-Crawford, Xuefeng Ren, Robert S. Sullivan, Terrance J. 

Kavanagh, Leona D. Samson, Helmut Zarbl and Peter S. 

Spencer; Portland, OR; Seattle, WA; Cambridge, MA; 

Jefferson, AR; Guildford, Kent, United Kingdom; Buffalo, NY; 

Piscataway, NJ and Chapel Hill, NC 

72 

Guam ALS-PD Complex (tauopathy) is linked epidemiologically 

to cycasin, methylazoxymethanol (MAM) glucoside, a 

neurotoxin, carcinogen and genotoxin. MAM produces O6methylguanine 

(O6-mG) DNA lesions that are repaired by 

O6-mG methyltransferase (MGMT). We asked whether 

MAM-induced DNA damage in MGMT-deficient 

(Mgmt / ) mouse brain (a model of the young adult 

human brain) activates signal transduction pathways perturbed 

in neurodegenerative disease. Brain transcriptional 

profiles of young adult Mgmt / and wild-type mice were 

determined at intervals after a single systemic dose of MAM 

or vehicle. Mgmt / mice treated with MAM vs. vehicle 

showed 362 differentially expressed genes, of which 57 were 

highly correlated with O6-mG levels. Sixty of 153 modulated 

genes correlated with O6-mG when the response of 

wild-type vs. Mgmt / mice to MAM vs. vehicle was 

determined. Top associations were with neurological disease, 

psychological disorders, cancer, and genetic disorders. Prominent 

MAM-modulated brain KEGG pathways included 

Wnt-beta-catenin, which is perturbed in Alzheimer Disease 

(AD) and in MAM-induced colorectal carcinoma. Thus, 

MAM modulates pathways common to cancer (in cycling 

cells) and cell degeneration (in non-cycling neurons). Exposure 

to environmental MAM-like genotoxins (e.g., nitrosamines) 

may have relevance to the etiology of other tauopathies, 

notably AD. 

Study supported by: National Institutes of Environmental 

Health Sciences: ES11384, ES11399, ES011387 and 

ES07033. 

T1512. Genetic Associations between VPS10 Receptor 

Genes and Late-Onset Alzheimer’s Disease 

Christiane Reitz, Joseph Lee, Lindsay Farrer, Margaret Pericak- 

Vance, Jonathan Haines, Ekaterina Rogaeva, Peter St. George- 

Hyslop and Richard Mayeux; New York; Boston; Miami; 

Nashville and Toronto, Canada 

Background: Genetic and functional studies showed that 

genetic variation in SORL1 and SORCS1 is associated with 

LOAD risk by modulating secretase processing of APP. 

Consequently, underexpression of SORL1 or SORCS1 leads 

to Ab overexpression and increased LOAD risk. We 

hypothesized that also genetic variation in the homologs 

SORCS2 and SORC3 is associated with LOAD. 

Methods: First, we analyzed associations between genetic 

variation in SORCS2 and SORCS3 and LOAD risk in several 

independent datasets. Then, we compared SORCS2 

and SORCS3 expression levels in brain regions from LOAD 

cases and controls, and performed cell biological experiments 

exploring the effect of both genes on APP processing. 

Results: Consistent with SORL1 and SORCS1, inherited 

variants in SORCS2 and SORCS3 were associated with 

LOAD risk. In addition, both genes were underexpressed in 

amygdala tissue from LOAD brains compared to control 

brains. Finally, we found evidence that - similar to SORL1 

and SORCS1- both SORCS2 and SORCS3 may influence 

APP processing by modulating gamma secretase processing 

of APP. 

Conclusions: In addition to SORL1 and SORCS1, also 

variants in SORCS2 and SORCS3 may play a role in the 

pathogenesis of LOAD through an effect on APP processing. 

Study supported by: R37-AG15473 (Mayeux), P01- 

AG07232 (Mayeux), R01-AG09029 (Farrer), R01- 

AG025259 (Farrer), P30-AG13846 (Boston University Alzheimer’s 

Disease Research Center grant), K23AG034550 

(Reitz). R01-AG027944 (Pericak-Vance), R01-AG019757 

(Pericak-Vance) 



T1513. Cardiac Ejection Fraction, Cognitive Function 

and Leukoaraiosis in an Elderly Cohort: The 

Cardiovascular Health Study 

Rebecca F. Gottesman, Salvador Cruz-Flores, Annette 

Fitzpatrick, John Gottdiener, Traci Bartz, Richard Kronmal 

and W.T. Longstreth, Jr.; Baltimore, MD; St. Louis, MO and 

Seattle, WA 

Background: Heart failure has been associated with cognitive 

dysfunction. To explore mechanism, we examined associations 

among ejection fraction (EF), cognition, and MRIdefined 

white matter hyperintensity (WMH) in the Cardiovascular 

Health Study (CHS), a longitudinal cohort study 

of participants 65 years old. 

Methods: We included 1972 CHS participants who underwent 

echocardiography, 2 brain MRIs 5 years apart, and cognitive 

assessments concurrent with the MRIs. Linear regression 

models including vascular risk factors, demographics, and EF 

(normal or impaired) were used to evaluate these outcomes: 

WMH grade on a 10-point scale, progression of WMH by 

1 grade, and change in cognitive performance on the 100point 

modified Mini-Mental State examination (3MS) and 

Digit Symbol Substitution test (DSST). 

Results: EF independently predicted WMH grade at the second 

MRI (p ¼ 0.05) and WMH progression (OR 1.61, 95% 

CI 1.07–2.43). Worse EF independently predicted more decline 

in 3MS (p ¼ 0.02) and DSST (p ¼ 0.02), but these relationships 

were partially attenuated with WMH grade as a covariate. 

Conclusions: In an elderly population, EF predicts volume 

and progression of WMH. EF also predicts cognitive 

performance, but this may be partially mediated through 

effects on the white matter. 

Study supported by: NIH/ NHLBI (not to the first / corresponding 

author directly) 

T1514. Predicting MCI Outcome with Clinically 

Available MRI and CSF Biomarkers 

David S. Heister, James B. Brewer, Sebastian Magda, Kaj 

Blennow and Linda K. McEvoy; La Jolla, CA and Mölndal, 

Sweden 

Cerebrospinal fluid (CSF) measures and medial temporal 

lobe atrophy (MTLA) on structural magnetic resonance 

images (MRIs) each predict decline to Alzheimer’s disease 

(AD), but how such measures can be used clinically to 

improve predictive prognosis is unclear.193 MCI participants 

were separated into risk groups (high/low) based on MTLA, 

quantified from FDA-approved software for volumetric imaging 

of MRIs, or based on cerebrospinal fluid (CSF) levels of 

total tau and Ab 1–42. Participants were also stratified into 

groups based on the combination of MTLA and CSF risk 

measures. Cox hazards models were used to assess group hazard 

ratios (HR) of converting to AD. MCI individuals with 

high atrophy or high CSF risk showed significantly greater 

hazard of AD conversion than those with low risk for each 

measure (HR: 3.52–4.20). Combining atrophy and CSF risk 

information substantially improved predictive ability. Individuals 

with both risk factors showed a significantly higher HR 

(13.68) than those with neither risk factor. Using both measures, 

80% of high-risk individuals developed AD in 3 years 

compared to 10% of low-risk individuals. Clinically available 

CSF and MRI measures can be combined to significantly 

improve predictive prognosis in MCI. 

Study supported by: L.K.M. is supported by NIA 

K01AG029218; J. B.B. is supported by NINDS 

K02NS067427. Data collection and sharing for this project 

was funded by the Alzheimer’s Disease Neuroimaging Initiative 

(ADNI) (National Institutes of Health Grant U01 

73 

AG024904). ADNI is funded by the National Institute on 

Aging, the National Institute of Biomedical Imaging and Bioengineering, 

and through generous contributions from the following: 

Abbott, AstraZeneca AB, Bayer Schering PharmaAG,- 

Bristol-Myers Squibb, Eisai Global Clinical Development, 

Elan Corporation, Genentech, GE Healthcare, GlaxoSmithKline, 

Innogenetics, Johnson and Johnson, Eli Lilly and Co., 

Medpace, Inc., Merck and Co., Inc., Novartis AG, Pfizer Inc, 

F. Hoffman-La Roche, Schering-Plough, Synarc, Inc., and 

Wyeth, as well as non-profit partners the Alzheimer’s Association 

and Alzheimer’s Drug Discovery Foundation, withparticipation 

from the U.S. Food and Drug Administration. Private 

sector contributions to ADNI are facilitated by the Foundation 

for the National Institutes of Health (www.fnih.org). The 

grantee organization is the Northern California Institute for 

Research and Education, and the study is coordinated by the 

Alzheimer’s Disease Cooperative Study at the University of 

California, San Diego. ADNI data are disseminated by the 

Laboratory for Neuro Imaging at the University of California, 

Los Angeles. This research was also supported by NIH grants 

P30 AG010129, K01 AG030514, and the Dana Foundation. 

None of the study sponsors had any role in the design and 

conduct of the current study, nor in data analysis, interpretation, 

preparation, review or approval of the manuscript. 

L. McEvoy’s spouse is President, CorTechs Labs, Inc. La Jolla, 

CA; Sebastian Magda is an employee of CorTechs Labs, Inc. 

T1515. Light and Electron Microscopic Analysis of FUS 

Immunoreactivity in 3 Variants of Tau and TDP-43 

Negative Frontotemporal Lobar Degeneration 

Keith A. Josephs, Wen-Lang Lin, Joseph E. Parisi, Neil Graff- 

Radford, Ronald C. Petersen and Dennis W. Dickson; 

Rochester, MN and Jacksonville, FL 

Background: The majority of frontotemporal lobar degenerations 

(FTLD) can be classified as tau or TDP-43 immunoreactive. 

Three rare variants were unclassified until recent 

evidence demonstrated FUS immunoreactivity: neuronal intermediate 

filament inclusion disease (NIFID), atypical 

FTLD with ubiquitin-immunoreactive inclusions (aFTLD- 

U) and basophilic inclusion body disease (BIBD). 

Aim: To assess the immunohistochemical profile of 

NIFID, aFTLD-U and BIBD. 

Methods: Light, electron and immunoelectron microscopic 

analysis of 15 cases of NIFID (n ¼ 5), aFTLD-U (n 

¼ 8) and BIBD (n ¼ 2). 

Results: All aFTLD-U and BIBD, and 3/5 NIFID cases 

were FUS immunoreactive. One FUS-negative NIFID case 

showed TDP-43 immunoreactivity. Electron microscopic 

examinations revealed FUS-immunoreactive granulofilament 

cytoplasmic inclusions in all aFTLD-U and BIBD cases, 

and in the FUS-positive NIFID cases. Granulofilament 

inclusions in the TDP-43 positive NIFID case were FUS 

negative, yet TDP-43-immunoreactive. Compact hyaline 

inclusions in all 5 NIFID cases were FUS-negative. 

Conclusions: Unlike in aFTLD-U and BIBD, FUS immunoreactivity 

in NIFID is variable and is limited to only certain 

types of inclusions, arguing against the notion that FUS is the 

primary pathological process in NIFID. Furthermore, TDP- 

43 immunoreactivity and NIFID are not mutually exclusive. 

Study supported by: NIH R01-AG037491 

T1516. Effects of Once-Daily, Extended-Release 

Memantine (28 mg/day) on Cognitive Domains in 







In this post hoc analysis of a 24-week, randomized, placebocontrolled 

trial (MEM-MD-50, NCT00322153) in ChEItreated 

patients with moderate to severe Alzheimer’s disease 

(AD), we examined the effects of a new, extended-release 

(ER) formulation of memantine (28 mg, once daily) on 

individual SIB domains, as well as on aggregated domains 

defined previously (Schmitt, 2006). Treatment groups were 

compared in terms of mean change from Baseline at Endpoint 

for nine SIB domains (Social Interaction, Memory, 

Orientation, Language, Attention, Praxis, Visuospatial Ability, 

Construction, and Orienting to Name) and combinations 

of domains aggregated using a face-valid approach 

into three higher-order subscales: MEMORY, LANGUAGE, 

and PRAXIS. Significant advantage of memantine ER over 

placebo was observed for the domains of Memory (OC, P 

¼ 0.021; LOCF, P ¼ 0.016), Language (OC, P ¼ 0.003; 

LOCF, P ¼ 0.004), Attention (OC, P ¼ 0.014; LOCF, P 

¼ 0.003), Praxis (OC, P ¼ 0.015; LOCF, P ¼ 0.002), Orientation 

(LOCF, P ¼ 0.043), and Construction (OC, P ¼ 

0.042), and for all three higher-order subscales (MEMORY: 

OC, P ¼ 0.002; LOCF, P ¼ 0.003; LANGUAGE: OC, 

LOCF, P ¼ 0.003; PRAXIS: OC, P ¼ 0.012; LOCF, P ¼ 

0.004). In conclusion, memantine ER treatment may be 

associated with significant improvements on several cognitive 

domains, including memory, language, praxis, and 

attention. 

Study supported by: Forest Research Institute 

Michael Tocco and Stephen M. Graham are employees of 

Forest Research Institute, Inc. Suzanne Hendrix is an employee 

of Pentara Corporation, an independent contractor 

to several pharmaceutical companies, including Forest 

Research Institute, Inc. Michael L. Miller and Vojislav 

Pejovic are employees of Prescott Medical Communications 

Group, an independent contractor to several pharmaceutical 

companies, including Forest Research Institute, Inc. 

T1517. Efficacy of Memantine by Baseline Disease 

Severity: A Pooled Post-Hoc Analysis of Trials in Mild to 

Moderate Alzheimer’s Disease 




Several randomized, placebo-controlled trials have demonstrated 

benefits of memantine in patients with moderate to 

severe Alzheimer’s disease (AD); trials in mild to moderate 

AD have yielded less consistent results. The dementia severity 

range in which memantine may provide benefits should 

be determined with greater precision. In this post-hoc analysis, 

we pooled 713 memantine-treated (20 mg/day) and 559 

placebo-treated patients from three randomized trials in 

mild to moderate AD (MMSE range: 10–23). For each 

baseline MMSE value, mean change from baseline was estimated 

for measures of cognition (ADAS-cog), function 

(ADCS-ADL23), global status (CIBIC-Plus), and behavior 

(NPI). Furthermore, data (intent-to-treat population, 

observed cases) were analyzed by means of a mixed model 

with quadratic terms for time and baseline MMSE. Memantine 

treatment was associated with significant benefits versus 

placebo within the MMSE range of 12–20 for the ADAScog, 

15–18 for the ADCS-ADL, and 10–17 for the CIBIC- 

Plus; no significant differences between groups were 

observed for the NPI. In conclusion, memantine treatment 

may be associated with significant cognitive, functional, and 

global benefits in patients with MMSE scores corresponding 

to the early moderate stage of AD. 

Study supported by: Forest Research Institute 

74 

Drs. Stephen Graham and Michael Tocco are employed 

by Forest Research Institute. Dr. Suzanne Hendrix is an employee 

of Pentara Corporation, an independent contractor 


Research Institute, Inc. Drs. Michael L. Miller and Vojislav 




T1518. Clinical Gait Abnormalities and Hippocampal 

Morphometry in MCI: Preliminary Results from the 

ADNI Study 

Vincent S. DeOrchis and Joe Verghese; Bronx, NY 

Objective: To examine whether clinical gait abnormalities 

could identify aMCI subjects with reduced hippocampal 

volume and increased risk of cognitive decline. 

Methods: The population for this study included subjects 

with aMCI diagnosed using established criteria and with 

neuroimaging enrolled in the Alzheimer’s Disease Neuroimaging 

Initiative (ADNI) study. Hippocampal volume was 

assessed in 370 aMCI subjects using semiautomated voxel 

based morphometry available on the ADNI website. The 

association of gait abnormalities, as diagnosed by study 

clinicians, with hippocampal volume was assessed using linear 

regression adjusted for age, gender, education and total 

brain volume. 

Results: Subjects mean age was 72.8 years, average education 

was 15.4 years and 62.3% were women. The mean 

hippocampal volume was 3188.77 þ/ 562.27mm3 on the 

right and 3145.28 þ/ 549.78mm3 on the left. Clinical 

gait abnormalities were diagnosed in 35 (9.3%) subjects and 

were associated with right hippocampal volume (estimate 

264.646, 95% CI: 443.4 to 85.8, p ¼ 0.004), but 

not the left (p ¼ 0.053). Adding MMSE or memory test 

scores to the final model did not change the significance of 

association. 

Conclusions: Our preliminary study supports gait abnormalities 

as a clinical marker of worse hippocampal morphology 

in aMCI. 

T1519. Topography of Cortical Thinning in PIB- 

Negative Subcortical Vascular Dementia Versus 


Chi Hun Kim, Sang Won Seo, Sung Tae Kim, Jae-Hong Lee, 

Jae Seung Kim, Seung Jun Oh, Suk-Hui Kim, Hae Kwan 

Cheong, Jong-Min Lee, Seun Jeon and Duk L. Na; Seoul, 

Korea and Suwon, Korea 

Objective: To determine the existence of cortical atrophy in 

Subcortical Vascular Dementia (SVaD) with negative 11 C- 

Pittsburgh compound B (PIB) PET scan and to compare 

the topography of cortical thinning between PIB (-) SVaD 

and Alzheimer’s disease (AD). 

Methods: Cortical thickness in 24 patients with PIB (-) 

SVaD, 81 clinically probable AD subjects with minimal ischemia, 

and 72 normal cognitive controls (NC) was measured 

using a surface-based morphometric method. The 

results were compared and mapped onto a 3D brain 

surface. 

Results: Compared with NC, significant cortical thinning 

of PIB (-) SVaD was noted in a widespread area including 

frontal, temporoparietal, medial frontal, posterior cingulate 

cortices, and ligual gyri. Compared with AD, PIB (-) SVaD 

demonstrated cortical thinning in bilateral perisylvian area, 

ligual gyri, and right medial frontal lobe. Compared with 

PIB (-) SVaD, AD showed significant cortical atrophy in 

bilateral medial temporal lobes and precuneus. 



Interpretation: Small vessel disease without amyloid burden 

may lead to substantial cortical atrophy. PIB (-) SVaD 

demonstrated characteristic cortical thinning in bilateral 

perisylvian area, ligual gyri, and the right medial frontal 

lobe. 

Study supported by: The Korea Healthcare Technology 

R&D Project, Ministry for Health, Welfare & Family 

Affairs, Republic of Korea (A050079), grants from the Asan 

Institute for Life Sciences (2006-159), and the Conversing 

Research Center Program through the National Research 

Foundation of Korea (NRF) funded by the Ministry of 

Education, Science and Technology (2009-0081959) 

T1520. The First Nationwide Survey of Bardet-Biedl 

Syndrome in Japan 

Makito Hirano, Toshihide Yamashita, Yasushi Ikuno, Hiromi 

Iwahashi, Mitsuru Ohishi, Toshiyuki Mano, Ryu Ishihara, 

Ichiro Tanaka, Keiko Yanagihara, Yusaku Nakamura and 

Susumu Kusunoki; Sakai, Osaka, Japan; Suita, Japan; Izumi, 

Japan; Osaka, Japan; Kashihara, Japan and Osaka Sayama, 

Japan 

Bardet-Biedl syndrome (BBS) is an autosomal recessive disorder 

characterized by mental impairment, rod-cone dystrophy, 

polydactyly, central obesity, hypogonadism, and renal 

abnormalities. The causative genes have been identified as 

BBS1-14 genes encoding proteins that maintain cilia function, 

but more than 20% of patients have no mutations 

identified. This disease is extremely rare in Japan, where 

only a few patients have been reported. We conducted the 

first nationwide survey of this disease in 2010. We summarized 

clinical and genetic information of newly identified 

patients and previously reported patients. We found that 

rare liver fibrosis was detected in 20% of patients, while 

only 30% of patients had apparent renal abnormalities, 

thought to be a universal symptom. DNA array analysis 

(BBS1-10,12) was performed in 9 patients with clinically 

definite or possible BBS, but no known mutations were 

identified. However, cultured fibroblasts from two patients 

had reduced protein levels of BBS6 without any coding 

mutations, suggesting abnormalities in transcription/translation 

or protein instabilities affected by other regulatory proteins. 

In conclusion, we speculate that clinical symptoms 

and genetic background of BBS in Japan may differ from 

those in the Western countries. 

Study supported by: Health and Labour Science Research 

Grants in Japan (Research in intractable diseases) 

T1521. P600 Word Repetition Effect Amplitude 

Correlates with Left Hippocampal Volume 

John M. Olichney, Rawi Nanakul, Alireza K. Javan, Patrick 

E. Adams, Andrea Schneider, Andreea Seritan, Randi J. 

Hagerman, Paul J. Hagerman and Susan M. Rivera; Davis, 

CA and Sacramento, CA 

Background: Prior work has shown that hippocampal volume 

and certain ERP components (e.g. P600 repetition 

effect) can be predictors of memory ability. However, the 

relationship between hippocampal volume and electrophysiological 

measures of human memory remains unclear. 

Objective: To define the relationship between P600 word 

repetition effect amplitude and hippocampal volume. We 

hypothesized they would be moderately correlated, with 

each variable independently predicting verbal memory. 

Methods: 32 channel ERP data was recorded during a 

category decision task. Structural 3T MRI was acquired 

(Siemens Trio) and hippocampal volumes measured on 3D 

MPRAGE sequences. 

75 

Results: Across our mixed sample of subjects (n ¼ 33; 

21 with Fragile X-associated Tremor Ataxia Syndrome, 12 

normal controls, mean age ¼ 65, mean MMSE ¼ 27), a 

significant correlation (r ¼ 0.38, p ¼ 0.029) was present 

between P600 repetition effect amplitude and left, but not 

right, hippocampal volume. Linear regression models found 

that subsequent cued recall was predicted by left hippocampal 

volume and age. In contrast, free recall correlated with 

P600 repetition effect amplitude (rho ¼ .49, p ¼ 0.004) 

only. 

Conclusion: P600 word repetition effect correlates with 

left hippocampal volume. These two measures appear complementary 

and predict different aspects of memory 

performance. 

Study supported by: National Institutes of Health Roadmap 

Interdisciplinary Research Consortium Grant [Grant 

Numbers UL1DE019583 (NIDCR), RL1AG032115], NIH 

Grant R01-AG18442, California Alzheimer’s Disease 

Program 

Randi Hagerman receives research support from Neuropharm, 

Seaside therapeutics, Forest, Johnson and Johnson 

and Roche and consultation with Novartis for fragile X 

research studies. 

T1522. Visuospatial Construction Measures and Their 

Utility in Identifying Dementia of the Alzheimer’s Type 

Bonnie M. Scott, G. Duncan, H. Carlson, Matthew Nance, 

Michele K. York, Angela Larery, Josephine Stouter and Adriana 

M. Strutt; Houston, TX 

Objectives: To examine the psychometric properties and 

clinical utility of three visuospatial-construction (VC) measures 

in differentiating Mild Cognitive Impairment (MCI) 

and Alzheimer’s disease (AD). 

Background: Assessment of VC deficits aid clinicians in 

distinguishing AD from other forms of dementia and determining 

the patient’s functional status. However, there is currently 

no research comparing the psychometric properties of 

commonly employed VC measures with MCI subtypes and 

varying stages of AD. 

Methods: 37 MCI and 126 AD patients completed the 

Beery-Buktenica Developmental Test of Visual Motor Integration 

(Beery), Rey-Osterrieth Complex Figure Test (Rey- 

O), and the Block Design subtest of the WAIS-III. 

Results: Age and education significantly differed between 

groups, and were therefore utilized as covariates. ANCOVAs 

revealed significant between group differences (p


measure (SIB, ADCS-ADL 19, NPI, CIBIC-Plus), 5 response 

levels were defined, based on change scores attained at Endpoint 

by the 10 th ,25 th ,50 th ,75 th , and 90 th percentile of 

placebo-treated patients. The proportions of memantineand 

placebo-treated patients attaining each response level 

(or better) were compared using Fisher’s exact test. For each 

outcome measure, the proportion of memantine-treated 

patients numerically exceeded that of placebo-treated 

patients at all response levels. On the SIB, the memantine 

group was significantly superior at the 90 th percentile level 

(improvement >11 points; P ¼ 0.003), on the NPI at the 

90 th percentile level (improvement 17 points; P ¼ 0.018) 

and the 75 th percentile level (improvement 8 points; P ¼ 

0.016), and on the CIBIC-Plus at the 75 th percentile level 

(score 18 yrs) and 

low education (


employee of Pentara Corporation, an independent contractor 


Research Institute, Inc. Michael L. Miller and Vojislav 




T1527. Neurophysiologic Markers of Aging-Related 

Muscle Weakness 

Ela B. Plow, Dina Gohar, Mehmed B. Bayram, Jing Hou, 

Alexandria Wyant, Yin Fang, Vlodek Siemionow and Guang 

H. Yue; Cleveland, OH 

Age-related muscle weakness is usually ascribed to peripheral 

factors, such as loss and change of muscle fibers. Recent evidence 

demonstrates that central neural drive diminishes 

with age, but its relation to strength remains unclear. We 

investigated the cortical neurophysiologic markers of agerelated 

muscle weakness using Transcranial Magnetic Stimulation 

(TMS). Ten healthy elderly (76.3 6 2.6yrs) and 15 

young individuals (22.3 6 0.9yrs) were enrolled. Measures 

included left elbow flexion strength, size of muscle evoked 

potential (MEP) following suprathreshold TMS delivered to 

Right Motor Cortex (RM1), size of RM1 map of left biceps 

brachii (BB) muscle, and inter-hemispheric inhibition (IHI) 

exerted upon RM1. Results demonstrate that elderly, compared 

to the young, were weaker (83.4 6 8.4N vs.105.9 6 

8.3N, p ¼ 0.04), possessed smaller RM1 map of BB (717.8 

6 150.8sq. mm vs.1034.4 6 116.1sq. mm, p ¼ 0.05) and 

showed stronger IHI (79.9 6 6.3% vs. 63.5 6 6.2%, p ¼ 

0.04), although their size of MEP was larger (1.1 6 0.2mV 

vs. 0.4 6 0.1mV, p ¼ 0.02). Stronger IHI was associated 

with smaller RM1 map of BB (r ¼ 0.38, p ¼ 0.05) and 

poor strength (r ¼ 0.36, p ¼ 0.06). Thus, although compensatory 

processes ensue in the muscle, age-related weakness 

persists. Reduced motor cortical maps and stronger inhibition 

from ipsilateral motor areas may explain weakness 

in the elderly; interventions modulating these cortical factors 

may improve strength. 

Study supported by: National Institutes of Health R01 

NS035130 

T1528. The Purkinje Cell of the Cerebellar Cortex in 


and Stavros J. Baloyannis; Thessaloniki, Greece 

Alzheimer’s disease is a heterogeneous neurodegenerative disorder, 

characterized by progressive memory loss, affective 

and behavioural changes.We studied the morphological findings 

of the cerebellar cortex, in twenty early cases of Alzheimer’s 

disease using electron microscopy and silver impregnation 

techniques. The Purkinje cells showed morphological 

alterations concerning dendritic profiles and axonic collaterals. 

Loss of dendritic branches was marked in Purkinje cells 

of the vermis. Large number of spines was lost in most of 

Purkinje cells. Axonic collaterals were also lost. The thick 

axonic network around the initial part of Purkinje cell 

axons, disappeard. The electron microscopy revealed morphological 

alterations of the mitochondria in the perikaryon, 

the dendritic processes and the axon of Purkinje cells. Fragmentation 

of the cisternae of Golgi apparatus was prominent.The 

loss of dendritic spines resulted in a dramatic 

decrease of the synapses in the molecular layer. However a 

tendency for regeneration of the spines was noticed, through 

a limited number of unattached spines. The synaptic alteration 

of the Purkinje cell emphasizes the importance of synaptic 

loss for the clinical and pathological profile of the 

disease, 

77 

T1529. A Translational Program of BDNF Gene 

Delivery for Alzheimer’s Disease 

Mark H. Tuszynski, Alan Nagahara, Adrian P. Kells, J. 

Bringas, John Forsayeth and Krystopf S. Bankiewicz; La Jolla, 

CA and San Francisco, CA 

Nervous system growth factors have extensive effects on neuronal 

function and survival. Nerve growth factor (NGF) prevents 

the death and stimulates the function of basal forebrain 

cholinergic neurons in correlational models of Alzheimer’s disease 

(AD), and is in a Phase 2 multi-center clinical trial using 

AAV2 gene delivery. Separately, Brain-Derived Neurotrophic 

Factor (BDNF) influences the survival and function of entorhinal 

cortical and hippocampal neurons in several animal 

models of AD, including transgenic mutant APP-expressing 

mice; aged rats and lesioned rats; and aged and lesioned primates. 

These effects occur independent of beta amyloid load. 

We are examining the safety and tolerability of BDNF gene 

delivery to the entorhinal cortex in rodent and primate dose 

escalation, safety and tolerability studies. Successful completion 

of these studies will lead to a Phase I trial of AAV2- 

BDNF gene delivery to target short term memory loss in Alzheimer’s 

disease. The translation of this clinical program will 

require development and utilization of real-time, MRI-guided 

AAV2 vector delivery with gadoteridol co-infusion to track 

and confirm vector distribution to the intended target. 

Study supported by: NIH, VA, Alz Assoc, Shiley Family 

Foundation 

T1530. Imaging Signatures of Pathology in Behavioral 

Variant Frontotemporal Dementia 

Jennifer L. Whitwell, Clifford R. Jack, David S. Knopman, 

Bradley F. Boeve, Ronald C. Petersen, Joseph E. Parisi, Dennis 

W. Dickson and Keith A. Josephs; Rochester, MN and 

Jacksonville, FL 

Background: Behavioral variant frontotemporal dementia 

(bvFTD) is pathologically heterogeneous. The most common 

pathologies underlying bvFTD are Pick’s disease (PiD), 

corticobasal degeneration (CBD), and FTLD-TDP type 1. 

We aimed to determine whether patterns of atrophy on 

imaging differed across these pathologies in bvFTD. 

Methods: We identified 15 bvFTD subjects that had a 

volumetric MRI and a pathological diagnosis of PiD (n ¼ 

5), CBD (n ¼ 5) or FTLD-TDP type 1 (n ¼ 5). Voxelbased 

morphometry was used to assess patterns of atrophy 

in each group compared to 20 controls. 

Results: All groups showed frontal grey matter loss, 

although specific patterns of atrophy differed across groups. 

The PiD group showed widespread frontal loss with involvement 

of the anterior temporal lobes. The CBD group 

showed less severe loss predominantly involving posterior lateral 

and medial superior frontal lobe. The FTLD-TDP group 

showed widespread loss in frontal, temporal and parietal 

lobes. Greater parietal loss was observed in FTLD-TDP compared 

to both other groups, and greater anterior temporal 

loss was observed in PiD compared to CBD. 

Conclusions: Atrophy patterns in bvFTD vary according 

to pathology and may therefore be helpful in predicting 

these underlying pathologies. 

Study supported by: NIH grants R01 DC10367, R01 

AG037491, R21 AG38736, R01 AG11378 and P50 AG16574. 

T1531. Improved Statistical Power To Detect Treatment 

Effects on Functional Outcomes in Alzheimer’s Disease 

(AD) Clinical Trials by Item-Response Theory (IRT) 

M. Colin Ard, Douglas R. Galasko and Steven D. Edland; La 

Jolla, CA 



The Alzheimer’s Disease Cooperative Study-Activities of 

Daily Living (ADCS-ADL) scale assesses an individual’s 

ability to perform a range of everyday tasks and is frequently 

used as an endpoint in clinical trials for AD. While 

the ADCS-ADL has excellent face validity as a measure of 

the functional impact of AD, the manner in which it is constructed 

presents several challenges for longitudinal data 

analysis, including the use of different rating scales across 

items, item-specific gender and/or lifestyle biases, differences 

in the complexity or difficulty of indicated activities, and 

missing data at the item level. IRT constitutes a family of 

quantitative models that assume that responses on specific 

items are jointly probabilistically determined by item characteristics 

and underlying subject-level trait(s), and are well 

suited to handling item heterogeneity and missing data. 

Using data from an ADCS clinical trial of 409 mild to 

moderate AD patients we found that ADCS-ADL scores 

constructed from IRT-based re-weighted item scores displayed 

increased sensitivity to change and improved statistical 

power as an outcome measure in clinical trials. 

Study supported by: NIH/NIA AG010483 (SDE), 

AG005131 (SDE, DG), and AG034439 (MCA, SDE). 

T1532. Neuropathologic Basis of Age-Associated Brain 

Atrophy 

Deniz Erten-Lyons, Randy Woltjer, Hiroko Dodge, Lisa Silbert 

and Kaye Jeffrey; Portland, OR 

Objective: To examine the association between postmortem 

neuropathologic measures and antemortem brain atrophy in 

aging. 

Background: Better understanding of the neuropathologic 

basis of age-related brain atrophy is needed to differentiate 

disease processes from normal aging, and to optimize 

use of brain volume as an outcome in Alzheimer disease 

prevention studies. 

Methods: Seventy-three participants of a longitudinal aging 

study were followed until death. All subjects had 2MRI 

scans, with the last scan within 36 months of death, and cognitive 

evaluations within 24 months of death. The association 

between antemortem rate of ventricular volume expansion over 

time and three postmortem measures:neurofibrillary tangles 

(NFTs), neuritic plaques (NPs) and infarcts (large vessel and 

lacunar) was examined using a mixed-effects model adjusting 

for age at death, APOE e4 presence and cognitive status. 

Results: Presence of infarcts, high NFT and NP scores 

were significantly associated with higher rate of ventricular 

enlargement regardless of cognitive status. e4 carriers also 

had a significantly higher rate of ventricular enlargement. 

Conclusion: These results suggest that brain atrophy, as 

indexed by ventricular enlargement, is a marker of accruing 

age-associated neuropathology independent of presence of 

cognitive impairment. 

Study supported by: Merit Review Grant & Research Career 

Development Award, Office of Research and Development, 

Department of Veterans Affairs, National Institutes of 

Health (AG08017, MO1 RR000334) 

T1533. A Long-Term, Open-Label Extension Study 

Evaluating the Safety of Extended-Release Memantine 

(28 mg) in Patients with Moderate to Severe 


Stephen M. Graham and James Perhach; Jersey City, NJ 

This study (MEM-MD-82) was a 52-week, multicenter, 

open-label extension (OLEX) of two previous trials of a 28mg, 

once-daily, memantine extended-release (ER) formulation: 

MEM-MD-51, a 52-week, open-label dosing trial in 

78 

patients with AD (MMSE 3–18; N ¼ 166) who were 

titrated or switched immediately to memantine ER, and 

MEM-MD-54, a 28-week OLEX of a pivotal, 24-week, 

randomized, placebo-controlled trial of memantine ER 

(MEM-MD-50; NCT00322153) in patients with AD 

(MMSE 3–14; N ¼ 677). All patients from Study MEM- 

MD-54 and most patients from MEM-MD-51 were taking 

a ChEI at Baseline, which continued throughout this study. 

Sixty-six patients received memantine during this trial; 44 

(66.7%) completed the study, and 8 (12.1%) discontinued 

due to an AE. A total of 50 (75.8%) patients experienced a 

treatment-emergent AE, including UTI (13.6%), agitation 

(12.1%), aggression (10.6%), exacerbation of AD (9.1%), 

and anemia, constipation, weight decrease, and back pain 

(7.6% each); 17 (25.8%) experienced a serious AE. In conclusion, 

treatment with once-daily, memantine ER (28 mg) 

for up to two years is well tolerated in patients with moderate 

to severe Alzheimer’s disease. 

Study supported by: Forest Laboratories, Inc. 

Drs. Stephen Graham and James Perhach are employed 

by Forest Research Institute. 

T1534. A Retrospective Analysis Using Data-Monitoring 

Algorithms: What Are the Logical Relationships between 

the ADAS-Cog and MMSE? 

Christian Yavorsky, Guillermo DiClemente, Mark Opler, Sofija 

Jovic, Brian Rothman and Ashleigh DeFries; New York, NY 

Background: The ADAS-Cog and MMSE are standard 

instruments in Alzheimer’s disease trials. The Critical Path 

Institute Online Data Repository (CODR) includes results 

of AD trials submitted by 11 sponsors. However, reliability 

and validity problems exist with clinician administered 

measures. Data-monitoring uses relationships between 

instruments to address this. We assessed the likelihood of 

error in assessments if they fell outside expected correlations 

between the ADAS-Cog and MMSE, and examined whether 

errors could have been detected by data-monitoring. 

Methods: Correlations of total ADAS-Cog and MMSE 

scores from AD patients in 11 trials were compared with 

known correlations. Assessments that fell out of previously 

reported ranges were evaluated using data-monitoring 

algorithms. 

Results: The correlation between MMSE and ADAS-Cog 

was .780 (p

dementia. Denied images, mentioned spirits, paranoid about 

witchcraft to make him look mad and get him locked up, implying 

pension would be stolen. He was hydrated, given Aricept, 

Seroquel, became calmer. He was discharged with diagnosis of 

Alzheimer’s dementia, bimodal CBS (Charles Bonnet Syndrome), 

possible resolved delirium and dehydration. 

Discussion: CBS involves complex visual hallucinations 

with partial or full insight; auditory variant rarely reported 

with visual, both associated with sensory deprivation. 

Patients often hide hallucinations, as our patient did. Management 

includes anticonvulsant, antipsychotic, and antidepressant 

trials, also reassurance, increasing sensory stimulation 

and socialization. 

CBS was suggested as early marker for dementia, particularly 

Alzheimer’s and Lewy Body dementias. 

Conclusion: Suggest screening blind or deaf patients for 

CBS; closely follow for signs of dementia if positive. Further 

studies to ascertain positive predictive value and if earlier diagnosis 

leads to improved outcomes. 

T1537. Lead Exposure Up-Regulated Autophagy 

Response in Neuroblastoma SH-SY5Y Cells Via mTOR 

Kinase Signaling Pathway 

Shun-Sheng Chen, Chueh-Tan Chen and Jiin-Tsuey Cheng; 

Kaohsiung, Taiwan 

Objective: To study the signal transduced interaction 

between b-amyloid accumulation and autophagy response of 

lead exposured neuroblastoma SH-SY5Y cells. 

Background: Cellular necrosis, apoptosis, and b-amyloid 

deposition frequently occur after chronic lead exposure, resulted 

in the amyloid-b formed during autophagic turnover of APPrich 

organelles supplied by both autophagy and endocytosis. 

Therefore, the new perspective wastriedtoprovetheroleof 

autophagy on amyloidogensis disorders of lead exposure. 

Methods: SH-SY5Y neurons were exposed with low concentration 

lead, and enhanced autophagy process was 

observed by western blot. The APP-mRNA levels, intracellular 

and secreted isoforms of APP, the mRNA levels, cell 

viability and b-amyloid production were also measured. 

Results: After lead exposure, SH-SY5Y cells were enhanced 

by their autophagy responses through increased LC3II from 

LC3I cleavage. Lead exposure also induced neuronal death by 

b-amyloid deposition. During autophagy process, neuronal 

death was accompanied with other autophagic characteristics 

as accumulation of the autophagosome and protein degradation 

regulated negatively by the mTOR kinase signaling. 

Conclusion: The autophagy processes have shown that 

enhancing chronic lead exposure in neurons is accompanied 

by accumulation of the autophagosome and protein degradation 

which regulated negatively via mTOR kinase signaling. 

Study supported by: National Science Council, Taiwan 


T1601. Withdrawn 


T1602. OnbotulinumtoxinA for the Treatment of 

Chronic Migraine: Long-Term Outcome 

Hanlon T. Christopher, Silvia M. Weibelt, Diane C. Andress- 

Rothrock and John F. Rothrock; Birmingham, AL 

Background: The long-term clinical outcomederived from 

treatment of chronic migraine (CM) with onabotulinumtoxinA 

(onabotA) is unknown. 

Methods: From a series of CM patients treated twice 

with onabotA who experienced a 50% or greater reduction 

79 

in headache days/month, we continued to treat and follow 

123 consecutive patients for at least 2 years (mean 2.76 

years; range 24 months-61 months). 

Results: Ten subjects (8%) eventually worsened despite 

continued treatment and relapsed to CM. Thirty-one subjects 

(25%) were able to stop onabotA therapy and remain 

largely free of headache for >6 months (mean sets of injections 

required: 4.8; range 2–8). Eighty-two (67%) remained 

responders but required ongoing injection therapy throughout 

the study period (at intervals ranging from 3 months to 

6 months; mean 3.4 months). Out of 1,142 sets of injections, 

there was one serious adverse event (status migrainosus, 

requiring brief hospitalization). 

Conclusion: Most CM patients who initially respond to 

treatment with onabotA will maintain that response for at 

least 2 years, and a substantial minority will be able to discontinue 

treatment and do well without prophylactic therapy. 

Long-term therapy with onabotA for CM is associated 

with a very low incidence of SAEs. 

Member, Allergan Physician Advisory Board; Consultant, 

Allergan 

T1603. Utility of Orally-Inhaled Dihydroergotamine 

When Early Intervention Is Impractical 

Shashidhar Kori, Stewart Tepper, Peter J. Goadsby, Paul 

Winner, Min Wang and Stephen Silberstein; Mountain View; 

Cleveland; San Francisco; West Palm Beach and Philadelphia 

Background: Well-controlled studies have demonstrated 

substantial reductions in triptan efficacy with delayed migraine 

treatment, and surveys have revealed some patient reluctance 

to treat migraines early, leading to treatment failure 

and dissatisfaction. 

Methods: This post-hoc analysis of a randomized, double-blind, 

placebo-controlled phase 3 study of orally-inhaled 

DHE compared 2-hour pain-relief(PR) and pain-free(PF) 

rates among patients treating migraine within 1 hour, 1–4 

hours, 4–8 hours, or >8 hours of its start. 

Results: Of 903 patients randomized, 771 treating a single 

attack were included in the efficacy analysis. Two-hour PF 

and PF rates were: 66% and 38% (inhaled DHE) and 41% 

and 13% (placebo) when treated within 1 hour of migraine 

start; 60% and 28% (inhaled DHE) and 35% and 10% (placebo) 

when treated within 1–4 hours; 53% and 22% (inhaled 

DHE) and 30% and 8% (placebo) when treated within 4–8 

hours; and 49% and 19% (inhaled DHE) and 24% and 9% 

(placebo) when treated >8 hours after start. 

Conclusions: This analysis demonstrated the efficacy of 

orally inhaled DHE in moderate/severe acute migraine, even 

when administered >8 hours after migraine start. Inhaled 

DHE may help many migraineurs who are unable to treat 

migraine early. 

Study supported by: This study was sponsored by MAP 

Pharmaceuticals. Dr Kori is a full-time employee of MAP 

Pharmaceuticals. Dr Tepper, Dr Goadsby, Dr Winner and 

Dr Silberstein have consulted for MAP Pharmaceuticals. 

Shashi Kori, MD is a full-time employee of MAP 

Pharmaceuticals. 

T1604. Characterization of Intraepidermal Nerve Fiber 

Morphology in Pain Associated with Diabetic 

Neuropathy and Impaired Glucose Tolerance 

Hsinlin T. Cheng, Jacqueline R. Dauch, Gordon A. Smith, 

Robinson J. Singleton, Brandon M. Yanik and Eva L. 

Feldman; Ann Arbor, MI and Salt Lake City, UT 

Neuropathic pain is a common symptom associated with diabetes 

and. Measurement of intraepidermal nerve fiber 



density (IENFD) in distal skin samples is a standard 

method for the diagnosis of diabetic neuropathy (DN). 

IENFD, however, is not considered a good indicator for the 

diagnosis of painful diabetic neuropathy (PDN). To evaluate 

the use of skin biopsy to study neuropathic pain in PDN, 

we studied the IENFD and nerve morphology in skin samples 

from: normal control, diabetic control, PDN, and DN. 

Skin samples from both the distal leg and proximal thigh 

were examined. Quantification of IENFD demonstrated no 

significant difference between PDN and DN. Morphological 

studies on these skin samples demonstrated the presence of 

axonal swellings or microneuromas, round shape nerve segments 

in PDN. The microneuroma densities in the proximal 

thigh of PDN are significantly higher than that of the 

DN. In summary, IENFD does not have a diagnostic value 

for PDN. In contrast, the microneuroma densities in proximal 

skin samples could be used as an indicator for PDN. 


T1605. Heavily T2-Weighted Magnetic Resonance 

Myelography for Post-Lumbar Puncture Headache: 

A Pilot Study 

Yen-Feng Wang, Jong-Ling Fuh, Jiing-Feng Lirng and 

Shuu-Jiun Wang; Taipei, Taiwan 

Objective: To investigate the utility of heavily T2-weighted 

magnetic resonance myelography (HT2W MRM) in postlumbar 

puncture (LP) headache (PLPH). 

Background: PLPH occurs in about one third of patients 

receiving diagnostic LPs. HT2W MRM was proved comparable 

to computed tomographic myelography in localizing 

CSF leaks in spontaneous intracranial hypotension. 

Methods: We prospectively enrolled inpatients with indications 

for diagnostic LPs. Whole-spine axial HT2W 

MRMs were carried out after LPs. PLPH was diagnosed 

according to the ICHD-2 criteria. 

Results: Sixteen patients (3M/13F, age 45.9 6 14.6 

years, range 24–82) were recruited. Five patients (31.3%) 

(1M/4F) developed PLPH. Twelve patients (75%) had post- 

LP CSF leakage, and all of them had CSF leaks along the 

nerve roots. Of these patients, four (33.3%) had epidural 

CSF collections, and five (41.7%) had lumbosacral retrospinal 

CSF collections. All of the patients with PLPH had 

CSF leaks along the nerve roots, but only 41.7% of the 

patients with CSF leakage developed PLPH. 

Conclusion: HT2W MRM was sensitive in detecting 

CSF leaks after LP. Although CSF leakage after LPs was 

common, it was not the only determinant in the development 

of PLPH. 

Study supported by: Taipei Veterans General Hospital 

The authors are employees of Taipei Veterans General 

Hospital. 

T1606. Angioplasty and Stenting for the Treatment of 

Idiopathic Intracranial Hypertension Associated with 

Dural Venous Sinus Stenosis 

Parisa P. Javedani**, Jeremy D. Fields, Kenneth C. Liu, 

Stanley L. Barnwell and Bryan Petersen; Portland, OR and 

Charlottesville, VA 

Introduction: Lumboperitoneal shunt, ventriculoperitoneal 

shunt, and optic nerve sheath fenestration are accepted surgical 

therapies for medically refractory idiopathic intracranial 

hypertension (IIH). Stenting of stenotic venous sinuses has 

emerged as a potential therapy. 

Methods: We retrospectively reviewed all cases of dural 

stents for IIH at our institution. Eligibility criteria included 

medically refractory IIH with documented papilledema and 

80 

dural venous sinus stenosis of the dominant venous outflow 

system (gradient 10 mmHg). 

Results: Thirteen cases were identified (all female; mean 

39 years); all failed medical therapy and 5/13 failed surgical 

intervention. Technical success was achieved in 13/13 without 

major peri-procedural complications. The mean pre-procedural 

gradient across the venous stenosis was reduced from 

23mmHg to 4mmHg post-procedure. Headache resolved or 

improved in 9/13. Papilledema resolved in 12/13 and 

improved in 1/13. Among the 9 patients with angiographic 

follow-up, there were no instances of restenosis. 

Conclusions: Venous sinus stenting can achieve a high 

degree of technical success and safety with excellent clinical 

outcomes. Our series suggests that angioplasty and stenting 

may be considered as an alternative to current surgical therapies 

in patients with IIH and dural venous sinus stenosis. 

Study supported by: This study was unfunded and no 

individuals other than the authors have contributed to the 

preparation of this manuscript. 

T1607. Adrenal Insufficiency Presenting as Postural 

Tachycardia Syndrome 


Objectives: To report a case of adrenal insufficiency presenting 

as Postural Tachycardia Syndrome (POTS). 

Background: POTS is a disorder of unknown etiology 

characterized by orthostatic intolerance and excessive 

tachycardia. 

POTS is believed to be caused by central hypovolemia. 

Adrenal insufficiency has not been described before as an 

etiology for POTS. 

Methods: Case report 

Case report: We report a case of a 20 year-old woman 

who presented for migraine headache. Headaches improved 

after hydration and treatment. She returned to emergency 

room 2 weeks after discharge, with dizziness and recurrent 

falls associated with loss of consciousness that occurred 

while she attempted to stand. 

Physical examination showed orthostatic tachycardia. Tilt 

table test was positive. A diagnosis of POTS was made. 

No major improvement was noticed with fluid hydration 

and treatment with fludrocortisone. Additional work up 

showed low morning cortisol level. A diagnosis of adrenal 

insufficiency was made and patient was placed on prednisone 

with relief of her symptoms. 

Conclusion: POTS remains a diagnosis of exclusion. Here 

we report the first case of POTS associated with adrenal insufficiency, 

which was successfully treated with prednisone. 

Given the right clinical setting, checking cortisol level should 

be a consideration in evaluation for an etiology for POTS. 

T1608. Prevalence of Chronic Migraine (CM), 

Headache-Related Disability and Sociodemographic 

Factors in the US Population: Results from the American 

Migraine Prevalence and Prevention (AMPP) Study 

Dawn C. Buse, Michael L. Reed, Kristina Fanning, Aubrey N. 

Manack, Catherine C. Turkel and Richard B. Lipton; Bronx, 

NY; Chapel Hill, NC and Irvine, CA 

Objective: Estimate the prevalence of CM in the US population 

by sociodemographics and headache-related disability. 

Methods: In 2004 surveys were mailed to 120,000 US 

households; 162,756 individuals aged 12 returned surveys; 

28,621 reported severe headache. CM was defined as 

ICHD-2 migraine with headache frequency 15 headache 

days/month. Crude and sociodemographically adjusted prevalence 

ratios (PRs) were generated. 



Results: 19,189 individuals (11.8%) met ICHD-2 criteria 

for migraine (17.3% of females; 5.3% of males); 0.9% met 

criteria for CM (1.3% of females; 0.5% of males). Prevalence 

was highest in males and females aged 40–49. When 

compared with persons aged 12–17, adjusted PRs in the 

40–49 age group were as follows: females 4.71 (95% CI 

3.24–6.83), males 3.31 (95% CI 1.99 ¼ 5.49.) Rates of 

CM were inversely correlated with annual household 

income. Severe-headache related disability was reported by 

38.0% of CM vs. 9.5% of EM respondents. 

Conclusions: Prevalence of CM in the US was 0.9%, 

and was highest in adjusted models among females, in midlife, 

and households with the lowest income. Severe headache-related 

disability was most common among persons 

with CM. 

Study supported by: The AMPP was funded through a 

grant to the National Headache Foundation by Ortho- 

McNeil Neurologics, Inc., Titusville, New Jersey with supplemental 

funding by Allergan Inc., Irvine, CA. 

Drs. Buse, Lipton, Serrano, and Reed have received consulting 

funds and/or research support from Allergan. Drs. 

Manack and Turkel are full time employees of Allergan. 

T1609. Chronic Low Dose Methadone for the 

Suppression of Treatment-Refractory Chronic Migraine 

Keyvani Madjid and John F. Rothrock; San Diego, CA and 


Objective: to determine the safety and effectiveness of 

methadone administered chronically to patients with treatment-refractory 

chronic migraine (TRCM). 

Methods: We administered low dose (range 2.5–10 mg 

TID) methadone to a series of patients with TRCM. A positive 

treatment response was defined as a 50% or greater 

reduction in headache days/month during the 3rd treatment 

month relative to the baseline pre-treatment month, with 

that response maintained for at least 6 months. At 6 months 

we attempted to taper all responders off methadone. 

Results: We treated 130 subjects, and 57 (44%) achieved 

a positive response. We followed all responders for a mean 

of 27 months (range 13–57 months). Only 5 patients (4% 

of the total and 7% of responders) eventually were able to 

discontinue methadone without relapsing to CM within 30 

days. There was one serious adverse event (death due to 

overdose of oxycodone and alprazolam). 

Conclusions: While chronic administration of low dose 

methadone may achieve remission of TRCM to episodic 

migraine in a significant minority of patients so treated, discontinuation 

of treatment typically is associated with rapid 

relapse to CM. 

T1610. Relationship between High Frequency Nausea 

and Treatment Satisfaction in Episodic Migraine (EM): 

Results of the American Migraine Prevalence and 

Prevention (AMPP) Study 

Richard B. Lipton, Michael L. Reed, Kristina M. Fanning and 

Dawn C. Buse; Bronx, NY and Chapel Hill, NC 

Objective: Report headache symptomology and satisfaction 

with medications among persons with EM by headacherelated 

nausea status. 

Methods: Respondents to the 2009 AMPP survey who 

met criteria for EM (ICHD-2 criteria and


Objective: Compare patterns of medical consultation and 

headache-impact in persons with migraine in the general 

population. 

Methods: The AMPP study is a longitudinal, population 

study of persons with severe headache. 2009 survey respondents 

who met ICHD-2 criteria for migraine (CM [ 15 

headache days/month] or EM [


Results: 17 patients entered 30 days of data (192 headache 

attacks [mean ¼ 5.5]). Lower PSS was associated with 

higher odds ratios (ORs) for headache within 12 hours (OR 

[95% CI] ¼ 1.81 [1.13, 2.91] per 4-unit difference on 

PSS). Improvement in mood was significantly associated 

with headache occurrence within 12, 18, and 24 hours for 

happy (ORs ¼ 1.20, 1.20, and 1.18, and relaxed ORs ¼ 

1.26, 1.17, and 1.14. Increases of >10 points were associated 

with headache occurrence within 12 hours for happy 

OR ¼ 2.73 and relaxed OR ¼ 2.31. 

Conclusions: Reduced perceived stress and increased feelings 

of happiness and relaxation were associated with 

increased odds of headache. 

Study supported by: This study was supported by an investigator 

initiated grant from ENDO Pharmaceuticals, 

Chadds Ford, PA. 

Drs. Buse and Lipton have acted as consultants and/or 

received research support from Endo Pharmaceuticals. 

T1616. Assessing the Consistency of LEVADEX TM 

(MAP0004, Orally Inhaled Dihydroergotamine) 

Pharmacokinetic Parameters in Healthy Volunteers: 

Results from 3 Clinical Studies 

Amy Forst, Julie Iwashita, Don Kellerman, Shashidhar Kori, 

Tracy Thomas and Glyn Taylor; Mountain View; Merthyr 

Tydfil, United Kingdom and Radyr, United Kingdom 

Background: Rapid, consistent drug absorption is important 

for effectively treating migraine acutely. MAP0004, a 

novel, orally inhaled dihydroergotamine (DHE), has shown 

efficacy in treating migraine acutely. Data from pharmacokinetic 

studies were analyzed for consistency, adequacy, and 

speed of DHE absorption through inhalation. 

Methods: Three studies compared MAP0004 1.0mg 

nominal with 1.0mg of intravenous (IV) DHE, assessing 

pharmacokinetic differences between smokers and nonsmokers, 

effect of co-administration of ketoconazole on 

MAP0004, and acute effects of MAP0004 on pulmonary artery 

pressure. 

Results: MAP0004 delivered DHE rapidly in all studies 

(mean Tmax, 7–11 minutes). Peak plasma concentrations of 

DHE were consistent across studies in non-smokers (Cmax geometric mean, 2475–2551 pg/mL). MAP0004 Cmax values 

were substantially lower than IV values (average, 

45,000pg/mL) but higher than intranasal (1,004pg/mL). 

Cmax and clearance did not vary significantly based on lung 

function, age, or weight across studies. Co-administration of 

ketoconazole did not significantly impact DHE pharmacokinetics 

after MAP0004 administration. 80-hydroxy-dihydroer gotamine concentrations after MAP0004 administration 

were low (average Cmax,


Introduction: Dejerine Roussy Syndrome(DRS) usually 

results from damage to sensory pathways from thalamus to 

spinal cord. It may occur after stroke, multiple sclerosis, after 

trauma or in brain tumor involving thalamus. Occurrence 

of DRS in a patient with Sneddon’s syndrome has not 

been reported. We report a patient diagnosed with Sneddon’s 

syndrome presenting with central pain syndrome. 

Case Report: 69 year-old man with hypertension, hepatitis 

C, right posterior cerebral artery stroke presented with 

left hemi body severe pain. He was diagnosed with Sneddon’s 

syndrome nine months ago. His pain was severe, 

burning in nature, tearing and excruciating deep pressure 

pain involving left face, and left hemibody. He had left 

sided spastic hemiparesis and hyperesthesia and tenderness 

to touch. He was treated with all neuropathic medications 

and narcotic medications without significant success. 

Conclusion: DRS can be long lasting or even life long 

occurs immediately or years after a stroke. The exact pathogenesis 

is unknown, it could be related with inflammatory 

mediators and pain peptides by denervated nerves. To the 

best of our knowledge, occurrence of DRS in Sneddon’s 

syndrome has not been reported in the literature. 

T1620. Valproate-Responsive Subclinical Rhythmic 

Electrographic Disharges (SREDA) in a Migraineur 

Umer Akbar, Bhavpreet Dham, Evren Burakgazi and John 

Kelly; Camden, NJ and Washington 

Objective: To report SREDA and its response to valproate 

in a patient with migraine headache. 

Background: The electroencephalogram (EEG) findings 

in migraine are typically non-specific, such as ictal and 

inter-ictal diffuse and focal slowing. More specific EEG 

findings in migraine are diffuse and focal slowing in theta 

and delta frequencies and decreased alpha activity with 

increased response to photic stimulation. 

Design: We report the case of SREDA during a migraine 

headache, improving after valproate administration. 

Case: A 45 year-old Caucasian female with catamenial 

migraines was admitted for dizziness, fogginess, and refractory 

migraines. Magnetic resonance imaging revealed deep white matter 

changes consistent with history of recurrent migraines. An 

otherwise unremarkable EEG showed intermittent episodes of 

SREDA in wakefulness and drowsiness. Valproate was initiated 

and repeat EEG two-weeks later showed no evidence of SREDA. 

Discussion: SREDA is an infrequent EEG pattern which 

occurs predominantly in adults after hyperventilation. It is 

generally thought to be benign with little clinical significance 

and has been noted in conditions as diverse as vascular 

events, syncope, transient global amnesia and epilepsy. 

Response to valproate confirms the electrical mechanism of 

migraines and suggests SREDA’s potential causal relationship 

with migraines. 

T1621. Utriculo-Ocular Counterroll Reflex Disruption 

in Skew Deviation 

James A. Sharpe, Manokaraananthan Chandrakumar, Alan 

Blakeman, Herbert C. Goltz and Agnes M. Wong; Toronto, 

ON, Canada 

Lateral head tilt activates the utricles to evoke a static ocular 

counterroll reflex (OCR) of torsional eye motion. Damage 

to utriculo-ocular reflex pathways with an abnormal static 

OCR might be the mechanism of skew deviation, a vertical 

strabismus caused by supranuclear lesions. 

OCR gains, the change in torsional eye position/change 

in head position after sustained passive lateral head tilt of 

20 , were recorded using search coils in 18 patients with 

84 

skew from brainstem or cerebellar lesions, and 18 controls. 

Patient group mean OCR gain was significantly reduced in 

both torsional directions. OCR gains were asymmetric 

between eyes and between directions in 16 patients. The 

hypotropic eye incyclotorting gain was lower than the 

hypertropic eye excyclotorting gain after head roll toward 

the hypotropic eye in 17 patients. 

The reduced static OCR gain and interocular and directional 

gain asymmetries provide evidence of disruption of 

utriculo-ocular pathways in the pathogenesis skew deviation. 


Research (CIHR) 

T1622. Superior Semicircular Canal Dehiscence (SSCD) 

and Osteoporosis in Elderly Asian Women 

Alexander Yu, Douglas L. Teich and Eric T. Wong; Boston, 

MA 

SSCD is associated with vertigo caused by the absence of bone 

overlying the SSC. This opening acts as a third window for 

the vestibular system, resulting in vertigo that is triggered by 

sound (Tullio phenomenon) or external pressure exerted on 

the canal (Hennebert sign). Four elderly Asian women were 

evaluated for dizziness/vertigo in a community health clinic. 

Their age, 57–85, was significantly older than the median age 

of 40 originally reported for SSCD. They all complained of 

motion-induced vertigo without nausea or vomiting. Their 

cerebellar examinations were normal. Only one had inducible 

vertigo on Dix-Hallpike maneuver, and none had Tullio phenomenon 

or Hennebert sign. SSCD was confirmed in all cases 

by high-resolution CT, with longitudinal areas of dehiscence 

along the long axis of SSC, ranging from 0.4 to 3.0 mm, as 

seen on Poschl view. They also suffered from osteopenia or 

osteoporosis in the axial skeleton, as confirmed by bone density 

tests. SSCD may be associated with osteoporosis in elderly 

Asian woman without Tullio phenomenon or Hennebert sign. 

Further research is needed to determine the relationship of 

age, race, osteoporosis risk, and the development of SSCD. 

Study supported by: Chinese Center of Long Island 


T1701. Antibodies to Metabotropic Glutamate Receptors 

in Ophelia Syndrome and Cerebellitis 

Eric Lancaster, Eugenia Martinez-Hernandez, Maarten J. 

Titulaer, Sarah Wong, Jian Xu, Anis Contractor, Rita Balice- 

Gordon and Josep Dalmau; Philadelphia, PA and Chicago, IL 

Objective: The Ophelia syndrome is a presumed autoimmune 

disorder characterized by severe but treatment-responsive 

limbic encephalitis, in association with Hodgkin’s lymphoma. 

We aimed to determine the target autoantigen. 

Methods: Imunohistochemistry on brain tissue and cultures 

of rat hippocampal neurons was used to demonstrate 

antibodies. Immunoprecipitation, mass spectrometry, and 

mGluR5 knock outs served to identify the antigen. 

Results: Patient’s serum had antibodies that reacted with 

the hippocampus and cell surface of live, cultured neurons, 

immunoprecipitated mGluR5, and specifically labeled cells 

transfected with mGluR5. Reactivity of patient’s serum, but 

not control individuals, was abrogated in brain of mGluR5null 

mice, further confirming the specificity of the antibodies. 

Parallel studies with mGluR1, a receptor closely related 

to mGluR5, lead us to identify a case with cerebellar ataxia 

and mGluR1 antibodies. Despite the high homology of 

these receptors, each patient’s antibodies were specific for 

one type of receptor and a distinct syndrome, resembling 

the phenotype of genetic deletion of each receptor. 



Conclusions: Antibodies to mGluR1 and mGluR5 should be 

considered in patients with cerebellitis and the Ophelia syndrome. 

Recognition is important because these disorders respond 

to immunotherapy. Our findings facilitate this diagnosis. 

Study supported by: Dr. Lancaster has a training grant 

from Talecris; his contribution to the current work was supported 

by a Dana Foundation Neuro-immunology Award. 

Dr. Martinez-Hernandez has a grant from the Fondo de 

Investigaciones Sanitarias, FIS, Spain (FI08/00285). Dr. Titulaer 

is supported by a KWF fellowship 2009–4451 of the 

Dutch Cancer Society. Dr. Dalmau receives royalties from 

the editorial board of Up-To-Date; from Athena Diagnostics 

for a patent for the use of Ma2 as autoantibody test. Dr. 

Dalmau has received a research grant from Euroimmun, and 

his contribution to the current work was supported in part 

by grants from the National Institutes of Health and 

National Cancer Institute (grant RO1CA89054). Drs. Dalmau 

and Balice-Gordon were supported by 1RC1NS068204- 

01, and a McKnight Neuroscience of Brain Disorders award. 

T1702. Mortality Outcomes for Interferon Beta-1b 

Versus Placebo 21 Years Following Randomization 

Douglas S. Goodin, Anthony T. Reder, George Ebers, Gary 

Cutter, Marcelo Kremenchutzky, Joel Oger, Mark Rametta, 

Karola Beckmann and Volker Knappertz; San Francisco, CA; 

Chicago, IL; Oxford, United Kingdom; Birmingham, AL; 

London, ON, Canada; Vancouver, BC, Canada; Wayne, NJ; 

Berlin, Germany and Montville, NJ 

Objective: To assess the effect of interferon beta-1b (IFNB- 

1b) 250 lg on mortality outcomes in multiple sclerosis 

(MS). 

Methods: Originally 372 patients were randomized to either 

IFNB-1b 50 lg (n ¼ 125), IFNB-1b 250 lg (n ¼ 

124), or placebo (n ¼ 123), and remained on assigned 

treatment for a median 3.8 years (maximum 5.1 years), 

before licensed treatment became available. The primary 

outcome variable in this 21 year follow-up study was allcause 

mortality and the secondary cause-specific mortality. 

Results: At 21.1 years (median), vital status information 

was available for 366/372 (98.4%) of patients; of whom, 81 

(22.1%, 81/366) were deceased. Original randomization to 

IFNB-1b 250 lg showed a significant reduction in all-cause 

mortality versus placebo (log-rank test, P ¼ 0.0272) and 

risk of death was reduced by 39.3%. Cause of death data 

was assessed for 75% of deaths; of which, 50/61 (82%) 

were MS-related. Most of the excess mortality rate in placebo 

was MS-related. 

Conclusions: With near-complete ascertainment and over 

21 years of observation time, a substantial survival advantage 

was seen for patients receiving early IFNB-1b treatment 

versus placebo. Most of this striking difference appears to be 

due to MS-related deaths. 

Study supported by: Bayer HealthCare Pharmaceuticals, 

Montville, NJ, USA. 

Prof Douglas Goodin has received honoraria from Bayer 

HealthCare Pharmaceuticals for consulting fees and honoraria 

for speaking. 

T1703. A Recombinant Human Neuron-Binding IgM 

Protects Spinal Cord Axons and Improves Motor 

Function in a Murine Model of Multiple Sclerosis 

Aleksandar Denic*, Slobodan Macura, Arthur E. Warrington, 

Istvan Pirko, Brandon R. Grossardt, Larry R. Pease and Moses 

Rodriguez; Rochester, MN 

Our laboratory demonstrated that a natural human serum antibody, 

sHIgM12, binds to neurons in vitro and promotes neurite 

85 

outgrowth. We generated a recombinant form, rHIgM12, with 

identical properties. Intracerebral infection with Theiler’s Murine 

Encephalomyelitis Virus (TMEV) of susceptible mouse 

strains causes chronic demyelinating disease with progressive 

axonal loss and neurologic dysfunction similar to progressive 

forms of multiple sclerosis. We studied the effects of rHIgM12 

on motor function of TMEV-infected mice by recording spontaneous 

nocturnal activity continuously over several weeks using 

AccuScan activity boxes. Compared to control IgM- and salinetreated 

mice, rHIgM12-treated mice showed improved motor 

function which became statistically significant at 9 days post 

treatment (p ¼ 0.038 and p ¼ 0.043, respectively) until the 

end of experiment (56 days). To assess axonal integrity we performed 

retrograde-labeling, a technique that relies on anatomically 

continuous and functionally preserved axons. rHIgM12treated 

mice had more retrograde-labeled brainstem neurons, as 

compared to saline-treated controls. This study supports the hypothesis 

that peripheral treatment with a neuron-binding IgM 

not only protects spinal cord axons in vivo but also influences 

functional motor improvement. 

Study supported by: This work was supported by grants from 

the NIH (R01 GM092993, R01 NS024180, R01 NS03219, 

R01 NS048357) and the National Multiple Sclerosis Society 

(CA 1011-A8). We also acknowledge with thanks support from 

the Applebaum and Hilton Foundations and the Minnesota 

Partnership Award for Biotechnology and Medical Genomics. 

A patent for the use of rHIgM12 antibody has been issued 

to the Mayo Clinic and Foundation. Therefore some of the 

authors may receive financial compensation in the future. 

T1704. Neuroprotection Mediated through Estrogen 

Receptor Alpha in Astrocytes 

Rory Spence, Mary Hamby, Elizabeth Umeda, Noriko Itoh, 

Sienmi Du, Galyna Bondar, Michael Sofroniew and Rhonda 

Voskuhl; Los Angeles, CA 

Estrogen has well documented neuroprotective effects in 

central nervous system (CNS) disorders such autoimmune 

inflammation, traumatic injury, stroke, and neurodegenerative 

diseases, but the underlying mechanisms are uncertain, 

because diverse cell types express estrogen receptors in the 

immune system and CNS. We selectively deleted estrogen 

receptor alpha (ERa) from either astrocytes or neurons using 

well-characterized Cre-loxP systems for conditional gene 

knockout in mice. We found that signaling through ERa in 

astrocytes, but not in neurons, is essential for the neuroprotective 

effects of systemic treatment with ERa ligand on 

clinical function, CNS inflammation and axonal loss during 

experimental autoimmune encephalomyelitis. Our findings 

reveal a novel cellular mechanism for estrogen-mediated 

neuroprotective effects by signaling through astrocytes and 

have implications for understanding the pathophysiology of 

sex hormone effects in diverse CNS disorders. 

Study supported by: National Multiple Sclerosis Society 

(RRV), Adelson Medical Research Foundation (RRV and 

MVS), NIH NINDS grants NS 062117 (RRV) and 

NS057624 (MVS), Skirball Foundation, Hilton Foundation, 

and Sherak Family Foundation. 

T1705. Genesis of Astrogliosis in an Autoimmune Model 


Fuzheng Guo, Yoshiko Maeda, Joyce Ma, Monica Delgado and 

David Pleasure; Sacramento, CA 

Astrogliosis is a prominent feature of multiple sclerosis 

(MS). We employed genetic fate-mapping and bromodeoxyuridine 

(BrdU) labeling to evaluate the cellular origins of reactive 

astroglia in spinal cords of mice with experimental 



autoimmune encephalomyelitis (EAE) induced by immunization 

with myelin oligodendrocyte glycoprotein peptide 35–55 

(MOG peptide). We found that oligodendroglial progenitor 

cells (OPCs) were stimulated to proliferate early in the course 

of MOG peptide EAE, and that many of these OPCs differentiated 

to oligodendroglia, but that OPC generation of 

astroglia was a rare event. Spinal cord ependymal cells, which 

have been reported to give rise to astroglia following spinal 

cord trauma, did not generate either OPCs or astroglia during 

MOG peptide EAE. Instead, virtually all reactive astroglia, 

identifiable by their expressions of nestin and vimentin 

in addition to glial fibrillary acidic protein, arose from resident 

spinal cord astroglia. In spinal cord white matter, astroglial 

proliferation increased overall astroglial numbers. In gray 

matter, in contrast, reactive astrogliosis was not accompanied 

by astroglial hyperplasia. Our study provides the first comprehensive 

view of the cellular origins of astrogliosis during 

an autoimmune demyelinative disorder. 

Study supported by: This work was supported by the 

Shriners Hospitals for Children, the California Institute for 

Regenerative Medicine (CIRM), NMSS and NINDS 

T1706. Distinct Features of Neuromyelitis Optica 

According to Anti-Aquaporin-4 Antibody IgG Subclass 

Noriko Isobe, Tomomi Yonekawa, Takuya Matsushita, Yuji 

Kawano, Katsuhisa Masaki, Satoshi Yoshimura and Jun-ichi 

Kira; Fukuoka, Japan and Matsuyama, Japan 

Objective: To clarify the clinical features of neuromyelitis 

optica (NMO) according to anti-aquaporin-4 (AQP4) antibody 

IgG subclass. 

Methods: We developed a quantitative flow cytometric 

assay for each anti-AQP4 antibody IgG subclass, and 

assessed 142 patients with multiple sclerosis (MS), 29 with 

neuromyelitis optica (NMO) fulfilling the 1999 criteria, 19 

with recurrent/longitudinally extensive myelitis, 86 with 

other inflammatory or non-inflammatory neurological diseases, 

and 28 healthy controls. 

Results: Anti-AQP4 antibody IgG was detected in 46 

patients, among whom 35 fulfilled either the 1999 or 2006 

NMO criteria, 9 had recurrent/longitudinally extensive spinal 

cord lesions, and two showed MS-like features. IgG1, 2, 

3 and 4 anti-AQP4 antibodies were detected in 97.8, 39.1, 

13.0 and 8.7% of patients, respectively. In patients not 

receiving corticosteroids, the levels of IgG1 anti-AQP4 antibody 

correlated positively with disease duration, while those 

of IgG2 antibodies correlated negatively with maximum spinal 

cord lesion length. IgG2 anti-AQP4 antibody carriers 

showed a younger age of onset, longer disease duration and 

lower Progression Index than those of other subclasses. 

Conclusion: IgG1 anti-AQP4 antibody levels increase 

with disease duration, while IgG2 antibodies are seen in relatively 

benign cases with a younger onset age. 

Study supported by: The Health and Labour Sciences 

Research Grant on Intractable Diseases (H20-Nanchi-Ippan- 

016) from the Ministry of Health, Labour and Welfare, Japan, 

and the grant-in-aid (B; no. 22390178) from the Ministry of 

Education, Culture, Sports, Science and Technology, Japan. 

T1707. Effect of Fingolimod on Relapse Rate by Prior 


FREEDOMS Subgroup Analyses 

Marcelo Kremenchutzky, Paul O’Connor, Reinhard Hohlfeld, 

Ernst-Wilhelm Radue, Ludwig Kappos, Lixin Zhang-Auberson, 

Dieter A. Häring, Philipp von Rosenstiel, Xiangyi Meng and 

Augusto Grinspan; London, ON, Canada; Toronto, ON, 

Canada; Munich, Germany; Basel, Switzerland and East 

Hanover, NJ 

86 

Fingolimod (FTY720) is a sphingosine-1-phosphate receptor 

modulator for the treatment of relapsing-remitting multiple 

sclerosis. In the phase 3 FREEDOMS study, fingolimod 

0.5-mg once daily reduced annualized relapse rate (ARR) by 

56% vs. placebo at 24 months (p


companies and by grants from the Swiss MS Society, the 

Swiss National Research Foundation, the European Union, 

the Gianni Rubatto, Novartis and Roche Research Foundations. 

Dr. Zhang-Auberson, Dr. Häring, Dr. von Rosenstiel, 

Dr. Meng, and Dr. Grinspan are employees of Novartis. 

T1708. Effect of Fingolimod on Relapse Rate by Prior 


TRANSFORMS Subgroup Analyses 

Bhupendra O. Khatri, Jean Pelletier, Ludwig Kappos, Hans- 

Peters Hartung, Giancarlo Comi, Frederik Barkhof, Jeffrey 

Cohen, Tracy Stites, Xiangyi Meng and Augusto Grinspan; 

Milwaukee, WI; Marseille, France; Basel, Switzerland; 

Dusseldorf, Germany; San Raffaele, Milan, Italy; Amsterdam, 

Netherlands; Cleveland, OH and East Hanover, NJ 

In the phase 3 TRANSFORMS study of relapsing–remitting 

multiple sclerosis patients, fingolimod (FTY720) 0.5-mg 

once-daily significantly reduced annualized relapse rate 

(ARR) vs. once-weekly intramuscular interferon (IFN)b-1a 

30 lg by 52% at 1 year (p


payments for all these activities have been exclusively used 

for funding of research of his department. Research and the 

clinical operations (nursing and patient care services) of the 

MS Center in Basel have been supported by non-restricted 

grants from one or more of these companies and by grants 

from the Swiss MS Society, the Swiss National Research 

Foundation, the European Union, the Gianni Rubatto, 

Novartis and Roche Research Foundations. Dr. Francis, Dr. 

Collins, Dr. Zhang-Auberson, and Dr. Tang are employees 

of Novartis. 

T1710. Fingolimod Mechanism of Action (MOA) in 

Multiple Sclerosis (MS) 

Jerold Chun and Jeffery A. Cohen; La Jolla, CA and 

Cleveland, OH 

Fingolimod, the first oral MS therapy, is thought to mediate 

its effects through actions of its metabolite, fingolimodphosphate, 

on sphingosine-1-phosphate (S1P) receptors on 

T-lymphocytes. This interaction produces ‘‘functional antagonism’’ 

of the receptor subtype S1P1 on CCR7-positive Tcells 

(naïve, activated, central memory), whereby S1P1 is 

internalized and degraded, receptor signalling is lost, and 

egress from lymphoid organs is inhibited. Fingolimod exposure 

leaves CCR7-negative effector memory T-cells comparatively 

unaffected. In MS, the net effect is a relative preservation 

of immune surveillance, while proinflammatory 

subsets are prevented from reaching the CNS. Additionally, 

accumulating data support a distinct, nonmutually exclusive 

mechanism involving direct S1P-receptor modulation on 

CNS cells. To address this issue, studies in EAE-challenged 

mice have been pursued using a conditional-null mutation 

resulting in a lack of S1P1 in neurons and glia. This mutant 

demonstrated attenuated disease severity and loss of fingolimod 

efficacy despite maintaining immunologic effects on 

peripheral blood lymphocytes. Loss of fingolimod efficacy 

was apparent in astrocyte, but not neuronal, S1P1-null mutants. Additional data in this model will be discussed 

towards considering fingolimod as having a dual MOA 

involving S1P-receptor modulation in not only the immune 

system but also the CNS. 

Study supported by: NIH and Novartis Pharma AG, 

Basel, Switzerland 

Dr. Chun has received consulting fees from Novartis and 

Ono; lecture fees from Novartis; and research support from 

Novartis and Pfizer. Dr. Cohen has received consulting fees 

from Biogen Idec, Elan, Lilly, Novartis, and Teva. 

T1711. Antibodies to the VGKC-Complex Proteins LGI1 

and CASPR2 in Acquired Neuromyotonia 

Camilla Buckley, Philippa Pettingill, Rosie Pettingill, Matthew 

Kiernan, Osamu Watanabe, Sarosh Irani, Patrick Waters and 

Angela Vincent; Oxford, United Kingdom; Sydney, Australia 

and Kagoshima, Japan 

VGKC-complex-Abs are found in neuromyotonia, Morvan’s 

syndrome, or limbic encephalitis. It is now clear that 

VGKC-complex-Abs are mainly directed against proteins 

that are complexed with VGKCs in the brain, particularly 

LGI1 (mainly limbic encephalitis) and CASPR2 (mainly 

Morvans syndrome and neuromyotonia), but these antibodies 

have not been studied in detail in neuromyotonia 

patients. 68 sera from patients with neuromyotonia from 

centers in Kagoshima and Sydney (29 females, 39 males,12 

to 85 years) were assayed using cell-based assays for antibodies 

to LGI1 and CASPR2. 20/68 (30%) sera were positive 

for VGKC-complex-Abs (106–1560 pM) including three of 

the four with Morvans syndrome. 14 of these sera were pos- 

88 

itive with the cell based assays: eight for both CASPR2-Abs 

and LGI1-Abs, four only for CASPR2-Abs, two only for 

LGI1-Abs. In addition, ten of the 48 VGKC-complex-Ab 

negative sera had CASPR2-Abs. These results indicate that 

patients with neuromyotonia can have both CASPR2 and 

LGI1 antibodies. Moreover, the presence of CASPR2 

antibodies in 20% of the patients negative for VGKC-Abs 

by radioimmunoassay suggests that the cell-based assays 

may make a useful addition to the investigation of 

neuromyotonia. 

Study supported by: Medical Research Council of Great 

Britain and NIHR Oxford Biomedical Research Centre 

Angela Vincent, Sarosh Irani and Patrick Waters may in 

the future receive royalties from use of the LGI1 and 

CASPR2 antibodies for diagnosis of neuromyotonia and 

other diseases 


T1713. Association of MS Susceptibility Variants and 

Early Attack Location 

Ellen M. Mowry, Jean Pelletier, Maria R. Blasco, Pierre 

Duquette, Pablo Villoslada, Pierre-Antoine Gourraud, Irina 

Malikova, Christophe Picard, Jamie McDonald, Elaine Roger, 

Stacy Caillier and Emmanuelle Waubant; San Francisco; 

Marseille, France; Madrid, Spain; Montreal, Canada and 

Pamplona, Spain 

Background: The anatomic location of early MS relapses 

within the central nervous system is predicted by the first 

attack location. We sought to determine if genetic polymorphisms 

associated with multiple sclerosis (MS) susceptibility 

are associated with attack location. 

Methods: 17 well-documented MS susceptibility polymorphisms 

were genotyped in 354 white, non-Hispanic 

patients seen within a year of MS onset. Their association 

with the CNS location of the first two MS attacks was 

assessed. 

Results: The IL12A polymorphism was associated with 

increased odds of both attacks involving the spinal cord 

(OR ¼ 2.4, 95% CI 1.3, 4.3, p ¼ 0.003). The IL7R polymorphism 

was associated with reduced odds of both attacks 

involving the brainstem/cerebellum (OR ¼ 0.2, 95% CI 

0.0, 0.9, p ¼ 0.042). There was a strong trend for an association 

of EVI5 with reduced odds of both attacks featuring 

optic neuritis. Several other genes showed trends for association 

with attack locations. 

Conclusions: Some of the MS susceptibility genes predict 

MS attack location. This finding may lead to improved 

understanding of MS pathogenesis and treatment. 

Study supported by: National Multiple Sclerosis Society 

T1714. Osmotic Demyelination Syndrome: Lack of 

Association between Outcome and Severity of 

Hyponatremia 

Jennifer E. Fugate*, Jonathan Graff-Radford and Alejandro A. 

Rabinstein; Rochester, MN 

Objective: Little is known about outcome predictors in osmotic 

demyelination syndrome (ODS), though one study 

identified that severity of hyponatremia is an independent 

predictor. We aimed to characterize clinical and radiographic 

features of patients with ODS and identify variables that 

predict outcome. 

Methods: A retrospective study of patients with ODS 

identified by a search of Mayo Clinic medical records from 

1999–2010. Patients were diagnosed by clinical and 



radiographic features. Medical charts were reviewed. Favorable 

outcome was defined by Modified Rankin Scale (mRS) 

2. 

Results: Of 24 patients, 14 (48%) had central pontine 

myelinolysis and 10 (42%) extrapontine myelinolysis. Hyponatremia 

was documented in 18 (75%) with mean sodium 

nadir of 114 mmol/L. Favorable outcome was seen in 

15 (63%) at mean follow-up 3 years. Initial brain MRI was 

normal in 21%. The following variables were assessed: age 

(p ¼ 0.40), gender (0.68), sodium level (p ¼ 0.30), albumin 

(p ¼ 0.12), extrapontine involvement (p ¼ 1.00), 

GCS (p ¼ 0.33), alcoholism (p ¼ 1.00), and sepsis (p ¼ 

0.17). None were predictive of outcome. 

Conclusions: Functional outcome in ODS is not associated 

with severity of hyponatremia. Serial brain imaging is 

of value as a substantial proportion of patients have normal 

initial MRIs. 

T1715. Correlation of Brain Atrophy, Disability and 

Spinal Cord Atrophy in a Murine Model of MS 

M Mateo Paz Soldan, Jeffrey D. Gamez, Aaron J. Johnson, 

Anne K. Lohrey, Yi Chen and Istvan Pirko; Rochester, MN 

and Cincinnati, OH 

Disability progression in MS remains incompletely understood. 

Unlike lesional measures, CNS atrophy has strong 

correlation with disability. TMEV infection in SJL mice is 

an established model of progressive MS. We utilized in vivo 

MRI to quantify brain and spinal cord atrophy in this 

model and analyzed the temporal relationship between atrophy 

and disability. 

Infected and control mice were followed for 12 months. 

Disability was assessed monthly using rotarod assay. Volumetric 

MRI datasets were acquired at 7 Tesla. Ventricular 

volume and C4-5 cord cross-sectional area measurements 

were performed using Analyze 10. At three months, brain 

atrophy reached statistical significance (p ¼ 0.009). In contrast, 

disability did not differ until four months post infection 

(p ¼ 0.0032). Cord atrophy reached significance by 

eight months (p ¼ 0.0088). By 12 months, brain atrophy 

resulted in 111.8% increased ventricular volume (p ¼ 

0.000031), while spinal cord cross-sectional area was 25% 

reduced (p ¼ 0.0013) in cases. 

Our results suggest that significant brain atrophy precedes 

and predicts the development of disability, while spinal cord 

atrophy occurs late and correlates with severe disability. The 

observed temporal relationship establishes a framework for 

mechanisms of disability progression and enables further 

investigations of their underlying substrate. 

Study supported by: National MS Society Pilot Project 

Grant, NIH/NINDS R01 NS 058698 

T1716. Anti-NMDA-Receptor Encephalitis: Clinical 

Analysis of 457 Patients 

Maarten J. Titulaer, Eugenia Martinez-Hernandez, Lindsey 

McCracken, Rita Balice-Gordon and Josep Dalmau; 

Philadelphia, PA and Barcelona, Spain 

Introduction: Anti-NMDA-receptor encephalitis is the 

most common and best characterized antibody-mediated encephalitis. 

We provide the clinical features and follow-up of 

457 patients. 

Methods: Analysis of demographics, onset, syndrome, 

treatment, and outcome at 24 months. 

Results: 84% were female. Median age was 21 years 

(range 1–85; 39% 18 and 4.3% 45 years). 45% had a 

tumor (94% teratomas, 5.7% other). 62% of females >18 

years had ovarian teratoma(s), versus 35% 18 years. 84% 

89 

of patients had 3 of the following: psychiatric symptoms, 

speech disorder, seizures, dyskinesias, decreased consciousness, 

autonomic instability, or hypoventilation. Treatment 

included tumor removal when appropriate, and immunotherapy 

(91%). At the time of analysis, full recovery/minimal 

symptoms (back to all activities) have occurred in 

57.3% of the patients, mainly in those with teratoma 

(66.7% vs 48.8%); 6% died. The frequency of relapses varied 

according to the response to initial immunotherapy; 

87% of relapses occurred in patients without teratoma. 

Conclusions: Anti-NMDA-receptor encephalitis is a 

severe but treatable disorder of predominantly young individuals. 

Prompt diagnosis and treatment are important to 

improve outcome. In addition to the above, detailed analysis 

of the frequency of relapses, immunotherapy, timing of recovery, 

and prognostic factors will be provided. 

Study supported by: The current work is supported in 

part by grants from the National Institutes of Health and 

National Cancer Institute RO1CA89054 (Dalmau), 

1RC1NS068204-01 (Balice-Gordon and Dalmau), and a 

McKnight Neuroscience of Brain Disorders award (Balice- 

Gordon and Dalmau). 

Dr. Titulaer is supported by a KWF fellowship 2009– 

4451 of the Dutch Cancer Society; Dr. Martinez-Hernandez 

has a grant from the Fondo de Investigaciones Sanitarias, 

FIS, Spain (FI08/00285); Dr. Dalmau receives royalties 

from the editorial board of Up-To-Date; from Athena Diagnostics 

for a patent for the use of Ma2 as autoantibody test. 

Dr. Dalmau holds a patent application for the use of 

NMDA receptor as autoantibody test. Dr. Dalmau has 

received a research grant from Euroimmun. 

T1717. Cigarette Smoke Induces Inflammation and 

Oxidative Stress in Brains of Lewis Rats 

Ashwani Khanna and Walter Royal, III; Baltimore, MD 

Cigarette smoke (CS) exposure is a risk factor for developing 

multiple sclerosis. We examined this effect by analyzing 

brains from Lewis rats exposed to CS from 4 cigarettes / 

day for 30 days using a system developed to simulate 

human smoking behavior. Using quantitative PCR, we 

observed an increase in IFN-c, TNF-a, TGF-b, IL-10,IL- 

6, IL-17, IL-18 and IL-23 and in MCP-1/CCL2, MIP-1a/ 

CXCR3 and SDF-1a/CXCL12 gene expression in the rat 

brains. Also gene expression for p22 phox , NOX-4, dual oxidase 

and Nfr2 was increased whereas thioredoxin gene 

expression was decreased. Immunocytochemical analysis of 

the brains from the rats showed, compared to rats not 

exposed to CS, increased staining for CD4, TNF-a, IFNc, 

class II MHC, ED1, Nrf2 and GFAP. Double immunofluorescence 

staining demonstrated cytoplasmic localization 

of Nrf2 in astrocytes from rats exposed to CS whereas nuclear 

localization was observed for non-exposed rats. These 

studies, therefore, demonstratethatCSexposurecanresult 

in increased brain immune cell trafficking, pro-inflammatory 

responses and oxidative stress. This model may be 

useful for elucidating mechanisms related to the link 

between CS and MS and for developing effective treatment 

strategies for the diseases. 


T1719. Differential Sensitivity of Human PBMC Subsets 

to Alemtuzumab-Mediated Cytotoxicity 

William Siders, Sambasiva Rao, Juanita Campos-Rivera, Jose 

Sancho, Paula Boutin, Peter Severy, Johanne Kaplan, Bruce 

Roberts and Srinivas Shankara; Framingham, MA 



Alemtuzumab(monoclonal antibody)currently in clinical 

trials for treatment of MS,recognizes CD52antigen 

expressed on lymphocytes&most myeloid cells. Its cytotoxic 

effects are reportedly influenced by density ofC- 

D52antigen on target cells. Using alemtuzumab in polychromatic 

flow cytometry assay,we examined levels 

ofCD52expression on 20distinct subsets of lymphoid 

&myeloid cells in peripheral blood mononuclear 

cells(PBMCs)from 11 healthy donors. Data demonstrated 

subsets of PBMCs express differing levels ofCD52. Quantitative 

analysis showed memory B-cells&myeloid dendritic 

cells(mDCs)display highest number while natural killer 

(NK) cells&plasmacytoid DCs have lowest number of CD52 

molecules per cell amongst lymphoid&myeloid cell populations,respectively. 

To test significance of differential CD52expression 

on sensitivity of PBMC subsets to alemtuzumabmediated 

cytotoxicity, we developed flow cytometry-based, 

complement-dependent cytotoxicity(CDC) assay allowing for 

simultaneous assessment of total number of dead cells&identification 

of phenotypes of surviving cells amongst PBMCs. 

Results from 4donors indicated, amongst lymphocytes, alemtuzumab-mediated 

profound cytotoxic effects on B-and Tcells 

with minimal effect on NKcells, correlating with density 

ofCD52 on these cells. Despite CD52 levels comparable to 

lymphocyte subsets, mDCs&monocytes were less susceptible 

to alemtuzumab-mediated CDC indicating antigen density 

alone doesn’t define susceptibility. Additionally, mDCs&monocytes 

expressed significantly higher levels of complement 

inhibitory proteins(CIPs) compared to lymphocyte subsets 

which may contribute to their resistance to alemtuzumabmediated 

CDC. Overall, results show while density of CD52 

antigen can influence cell susceptibility to alemtuzumabmediated 

CDC, factors such as, level of CIP expression, may 

also have an impact. 

Study supported by: Genzyme 

All authors received financial compensation (including 

stock) as employees of Genzyme. 

T1720. Transcriptional Profiles Uncover Population 

Structure among Multiple Sclerosis Patients 

Linda Ottoboni, David Hafler, Howard Weiner and Philip De 

Jager; Boston, MA and New Haven, CT 

There is extensive heterogeneity among subjects with multiple 

sclerosis (MS) in terms of their disease course and treatment 

response. Here, we explore the structure of the MS subject 

population using RNA expression profiles derived from peripheral 

blood mononuclear cells of 398 subjects. These subjects 

were untreated (n ¼ 148), treated with glatiramer acetate 

(GA) (n ¼ 101) or treated with interferon beta (IFNb) (n ¼ 

143). We find the expected expression profile associated with 

IFNb treatment but find no significant difference in gene 

expression with GA treatment. Using unsupervised clustering, 

we define 2 subsets of untreated subjects, with enhanced type 

I interferon response gene expression in the smaller group and 

enhanced lymphocyte activation pathway gene expression in 

the larger group. This population structure is preserved in the 

GA subjects (Kappa coefficient ¼ 0.83) and IFNb subjects 

(Kappa ¼ 0.82). Retrospective data suggest that the smaller 

group is more likely to have an clinical or MRI event after 

sampling (p ¼ 0.02). We therefore report the identification of 

a gene expression profile discriminating 2 subsets of MS subjects 

and further report that this architecture captures a previously 

reported T cell gene expression signature associated with 

high-risk CIS subjects (Corvol et al. PNAS 2008). 

Study supported by: Affymetrix Inc. - donation of microarrays 

and processing 

90 

T1721. Analysis of Immune Competence Following 

Alemtuzumab Treatment in huCD52transgenic Mice 

William Siders, Nathalie Chretien, Michael LaMorte, Bruce 

Roberts and Johanne Kaplan; Framingham, MA 

Alemtuzumab (humanized monoclonal antibody) currently 

in clinical trials for treatment of MS, results in the overall 

depletion of lymphocyte subsets followed by extended period 

of repopulation. The goal, to evaluate functionality of 

lymphocytes following treatment, was accomplished by 

treating human CD52 (huCD52) transgenic mice w/alemtuzumab 

& evaluating ability of remaining T-lymphocytes 

to respond to polyclonal stimulation. While lymphocytes 

from animals treated with alemtuzumab are present in 

lower numbers, they retain ability to proliferate in response 

to T-cell receptor (TCR) ligation & produce similarTh1, 

Th2, & Th17 family cytokines compared to lymphocytes 

from untreated animals. Comparison of TCR Vbeta families 

represented in alemtuzumab-treated & untreated animals 

demonstrated no differences in diversity of T-cell repertoire. 

We next evaluated ability of alemtuzumab-treated 

animals to generate de novo & recall immune responses to 

adenovirus serotype2( Ad2). Animals were injected with 

Ad2 at various timepoints relative to treatment &evaluated 

for Ad2-specificT-cell frequency by IFNgamma ELIspot & 

development of antibodies to Ad2. This demonstrated animals 

immunized as early as 7 days developed detectable 

frequencies of antigen specific T-cells. Recall responses 

were unaffected in animals which memory responses were 

allowed to develop prior to treatment. These studies demonstrate 

alemtuzumab therapy doesn’t impact functional 

activity of remaining T-cells &doesn’t severely affect ability 

of host to generate functional T- and B-cell responses to 

foreign antigen. 

Study supported by: Genzyme 

All authors receive financial compensation from Genzyme 

(including stock) as employees. 



T1724. A Single Dose Escalation Study of the Safety, 

Pharmacokinetics, and Pharmacodynamics of 

Subcutaneous Pegylated Interferon-Beta in Healthy 

Volunteers 

Kenneth Grabstein, Aijun Wang, Natalie Winblade Nairn and 

Daniel J. Burge; Seattle, WA 

Purpose: A single-ascending dose study was conducted in 

healthy volunteers (HV) to establish the safety, tolerability, 

pharmacokinetic (PK) and pharmacodynamic (PD) profile 

of AZ01 (PEGylated interferon-beta). Methods: Eight HV 

in each of 5 escalation cohorts received one subcutaneous 

dose of AZ01 or placebo (6:2) at doses from 100mg to 

10mg. Safety, PK and PD parameters were evaluated. 

Results: The AE profile of AZ01 was consistent with that 

expected with IFN-beta therapy, especially in the 10mg 

group. Events included injection site reactions, headache, 

myalgia, chills, fatigue, nausea, and fever. No SAEs or AEs 

leading to discontinuation occurred. No laboratory parameter, 

vital sign measurement, or ECG was determined by the 

Investigator to be clinically significant. PK and PD analyses 

indicate a sustained exposure and response to single doses of 

AZ01, lasting about 14 days. Conclusion: Single subcutaneous 

AZ01 doses of up to 10mg were well tolerated by HV. 



The prolonged PK and PD may allow dosing at greater 

intervals than with currently available interferon therapies. 

Less frequent injections are likely to decrease injection site 

reactions and flu-like symptoms and improve compliance. 

Study supported by: Allozyne Inc. 

Salaries, stock options, consulting fees 

T1725. Regulation of IL-12 by IL-23 in Bone Marrow 

Dendritic Cells 

Farinaz Safavi, Patricia Gonnella, Bogojub Ciric, 

Abdolmohamad Rostami and Farinaz Safavi; Philadelphia, PA 

Th1 and Th17 cells play important roles in the pathogenesis 

of Experimental Autoimmune Encephalomyelitis and other 

autoimmune models. IL-12 and IL-23 are heterodimeric 

cytokines produced mostly by dendritic cells (DCs) that 

promote development of Th1 and Th17 cells, respectively. 

Understanding the mechanisms that regulate production of 

these cytokines should advance our knowledge of the pathogenesis 

of Multiple Sclerosis (MS) and could lead to novel 

treatments for this disease. To test the effect of IL-23 on IL- 

12 production, Bone Marrow Dendritic cells(BMDCs) from 

IL-23p19 KO and WT mice were used. BMDCs were 

stimulated with LPS and mouse recombinant IL-23 (5 ng/ 

ml) and IL-12p70 and IL-12p35 expression were measured 

after 6 and 24 hrs. WT DCs stimulated with both LPS and 

LPSþIL-23 secreted significantly larger quantities of IL- 

12p70 (p< 0.01) compared to IL-23p19 KO cells. RT-PCR 

analysis also showed significantly higher expression of IL- 

12p35 mRNA in WT DCs treated with either LPSþIL-23 

or LPS (p < 0.01 ). Our observations indicate that the 

expression of IL-12 is regulated by IL-23 in vitro.Finding 

similar regulatory mechanisms in vivo should increase our 

understanding of the role of these cytokines in the pathogenesis 

of autoimmunity. 

Study supported by: NIH grant number RO1NS046782 

T1726. Clinically Apparent MRI Activity Predicts 2-Year 

Outcomes in Patients with RRMS 

Thomas Scott, Xiaojun You and Pamela Foulds; Pittsburgh, 

PA and Weston, MA 

Background: Several studies report clinically apparent and 

clinically silent MRI lesions predict disease worsening in 

patients on IFNb-1a. The magnitude of risk, especially with 

clinically silent activity, remains uncertain. 

Methods: SENTINEL enrolled patients with > ¼ 12 

months of prior IFNb-1a treatment who continued with or 

without addition of natalizumab. MRI scans at baseline and 

year 1 were compared for new lesions. New lesions as a risk 

for clinical activity (relapses or progression) between years 1 

and 2 were examined. 

Results: New MRI and clinical activity in year 1 was 

associated with increased risk for relapse or progression in 

year 2 (P

T1729. Are Anti-TAG-1 Autoantibodies Markers in 

Autoimmune Demyelinating Disorders of the PNS and 

CNS? 

Harry Alexopoulos, Pantelis P. Pavlakis, Domna Karagogeos, 

Clementine E. Karageorgiou and Marinos C. Dalakas; Athens, 

Greece and Heraklion, Greece 

There is evidence of anti-TAG-1 autoantibodies (TAG-1 

is involved in the development of axonal connections), 

being associated with autoimmune syndromes of the 

CNS. Anti-TAG-1 antibodies have been found both in 

multiple sclerosis and in autoimmune limbic encephalitis. 

Also, polymorphisms in the TAG-1 gene influence 

treatment response in chronic inflammatory demyelinating 

polyradiculoneuropathy. We wanted to investigate 

whether anti-TAG-1 autoantibodies are present in 

CIDP. 

We tested serum samples from 12 patients with autoimmune 

demyelinating neuropathy (8 with CIDP, 2 with 

CIDP and CNS demyelination, 2 with MMN), 9 with 

Sjögren’s syndrome-associated axonal polyneuropathy and 

50 with relapsing–remitting MS. We established a cell-based 

assay previously used to detect anti-TAG-1 autoantibodies 

in LE. Briefly, either the FNIII or the IgC2 domains of 

TAG-1 were expressed in HEK cells and were incubated 

with patient sera. Antibody binding was visualized using a 

secondary anti-human fluorescent antibody. 

No positive staining was detected in any of the patients 

with autoimmune peripheral neuropathies or with RRMS. 

Our results suggest that neither anti-TAG-1 autoantibodies 

are a marker for demyelination in PNS or CNS nor TAG-1 

is a candidate autoantigen in CIDP or MS. 

Study supported by: Special Research Account, National 

and Kapodistrian University of Athens 

T1730. Introduction and Diffusion of Multiple Sclerosis 

in the United States 

Mitchell T. Wallin and John F. Kurtzke; Washington, DC 

Epidemiological evidence suggests that multiple sclerosis has 

spread from an origin in northwestern Europe. How and 

when it came to the US has not been well defined. Nationwide 

morbidity data here have arisen primarily from the 

military-veteran populations of our several wars. Prevalence 

rates for draftees rejected because of MS for military service 

in World War I provide its earliest distribution. Small numbers 

of cases required combination of states of residence 

into 23 areas. Prevalence rates for the 255 cases in these 

areas showing a strikingly high rate in area P, comprising 

Wisconsin and Minnesota. These states were settled in the 

mid-19 th century mainly by immigrants from south-central 

Norway and Sweden. Their original Scandinavian residences 

were principally in the high-risk areas for MS in both countries. 

When compared with two later military-veteran series, 

one from World War II and the Korean Conflict, the other 

from Vietnam and later service to 1994, a continuing spread 

from this north mid-western focus was seen. MS appears to 

have been introduced into the US in the mid-19 th by immigrants 

from Scandinavia to Wisconsin and Minnesota, with 

later diffusion throughout the land. 

Study supported by: VA Merit Review Grant Program & 

National MS Society 




92 

T1733. The Relationship between Conduction Block 

and Clinical Characteristics in Guillain-Barré Syndrome 

with Anti-GM1/GalNAc-GD1a Antibodies 

Go Ogawa, Ken-ichi Kaida, Susumu Kusunoki, Motoi 

Kuwabara, Fumihiko Kimura and Keiko Kamakura; 

Tokorozawa, Saitama, Japan and Osaka-Sayama, Osaka, 

Japan 

Patients with Guillain-Barré syndrome (GBS) with antibodies 

to a ganglioside complex GM1/GalNAc-GD1a frequently 

show motor conduction block (CB) at the intermediate 

portion of peripheral nerves. Clinical data of 57 

patients with anti-GM1/GalNAc-GD1a-IgG were studied to 

elucidate the association between CB and clinical and electrophysiological 

characteristics. Thirty patients (male/female, 

19/11) had CB, classified as CB(þ) (mean age, 43.1 6 

17.7 y/o), and 27 patients (male/female, 10/17) did not, 

classified as CB(-) (mean age, 46.8 6 16.5 y/o). Males are 

significantly more in the CB(þ). Both groups presented frequent 

preceding respiratory infection, infrequent cranial and 

sensory nerve disturbances, and were equal in the disability 

at the nadir. Twenty of 25 non-ambulatory patients could 

walk within three months, with no difference between both 

groups. The 17 CB(þ) patients were electrodiagnostically 

classified into acute inflammatory demyelinating polyneuropathy, 

one of whom changed to acute motor axonal neuropathy 

later. Serial electrophysiological results suggested 

Wallerian degeneration only in one and no evident remyelination. 

In view of the rapid recovery and lack of remyelination, 

axonal conduction failure with little pathological 

changes may be major mechanism in anti-GM1/GalNAc- 

GD1a antibody-associated nerve dysfunction. 


T1735. Randomized, Open-Label Study To Evaluate 

Patient-Reported Outcomes (PRO) with Fingolimod 

after Changing from Prior Disease-Modifying Therapy 

(DMT) for Relapsing Multiple Sclerosis (MS): EPOC 

Study Rationale and Design 

Luigi M. Barbato, Lesley Schofield, Kevin McCague, Linda 

Pestreich, Kathy Tobias and Manoj Malhotra; East Hanover, 

NJ 

The efficacy and safety profiles of fingolimod (FTY720) 

have been well characterized in phase 2 and 3 studies; however, 

limited data exist on PRO. The EPOC study is evaluating 

PRO and safety in relapsing–remitting MS patients 

who have received 6 months’ treatment with a single 

approved DMT and are randomized 3:1 to receive oncedaily 

fingolimod 0.5 mg with no washout or to continue 

standard DMT (interferon-b-1a, interferon-b-1b, or glatiramer 

acetate) for 6 months, followed by a 3-month openlabel 

extension, in which the DMT group can change to 

fingolimod with no washout. The primary endpoint is 

change in patient-reported treatment satisfaction using the 

global satisfaction subscale of the Treatment Satisfaction 

Questionnaire for Medication (TSQM). Secondary endpoints 

are safety/tolerability, changes in physician-reported 

Clinical Global Impression-Improvement, and changes in 

the following PRO: Fatigue Severity Scale; Beck Depression 

Inventory-II; activities of daily living using the Multiple 

Sclerosis Activities Scale (PRIMUS-Activities); effectiveness, 

side effects, and convenience subscales of the TSQM; and 

the Short-Form 36 Health Survey-v2-acute. The study is 

enrolling at approximately 175 US centers. 



Study supported by: Novartis Pharma AG, Basel, 

Switzerland 

Dr. Barbato, Dr. Schofield, Dr. McCague, Dr. Pestreich, 

Dr. Tobias, and Dr. Malhotra are employees of Novartis. 

T1736. Novel Diagnostic Tool for MS 

Nancy L. Monson, Ann J. Ligocki, William H. Rounds, Diane 

Xiang, Lindsay G. Cowell, Doug Bigwood, Eric Eastman, 

Jeffrey L. Bennett, Scott D. Boyd, Andrew Z. Fire, Elliot M. 

Frohman and Benjamin M. Greenberg; Dallas, TX; 

Gaithersburg, MD; Denver, CO and Palo Alto, CA 

Multiple Sclerosis (MS) is the leading disease of the central 

nervous system and a significant, costly cause of disability in 

young adults. Patients at risk for MS often present with a 

single demyelinating event, typically called a ‘‘clinically isolated 

syndrome’’ (CIS) and present a challenging diagnostic 

and therapeutic dilemma. The community has an urgent 

need to develop tools that would assist in identifying 

patients who would develop MS in the future, since treatment 

with the appropriate immunomodulatory agents early 

in the disease course can delay long-term disability. Our laboratory 

has identified a completely novel type of biomarker, 

based on a pattern of antibody gene mutations (i.e. antibody 

gene signature or ‘‘AGS’’) that is exclusive to MS 

patients. In fact, the AGS demonstrated utility as a molecular 

diagnostic tool in a small cohort of patients who presented 

with optic neuritis (and were thus at risk to develop 

MS) with a sensitivity of 100%, specificity of 67%, positive 

predictive value of 89%, negative predictive value of 100%, 

and accuracy of 91%. Our current cohort, which includes 

both patients who presented with optic neuritis and patients 

that presented with transverse myelitis, has an increased accuracy 

of 94%. 

Study supported by: National MS Society 

T1737. Efficacy of Combination Therapy in Marburg 

Variant Type of Multiple Sclerosis 

Jennifer Gabbard, Yasmin Bilal and Dipak Pandya; Paterson, 

NJ 

Background: Marburg variant type of MS is a very rare 

form of disease which commonly consider fatal after weeks 

to months. The variant forms of multiple sclerosis are very 

rare and tend to have a rapid deteriorating course. We 

report an unusual case of Marburg variant MS, who 

responded to various combination therapy favourably. 

Results: 18-year-old male presented with imbalance, 

wide based gait and dizziness. His physical examination 

showed nystagmus, no signs of optic neuropathy, dysmetria, 

hyperreflexia and sustained clonus. The MRI brain 

showed extensive demyelinating plaques in periventricular 

white matter, brainstem and various areas of brain. The 

imaging findings were not consistent with Neuromyelitis 

optica. The patient was initally treated with pulse doses 

methylprednisone and plasmapheresis. Because of persisting 

clinical symptoms and imaging findings, patient was 

treated with induction doses of mitoxanthrone and interferone 

beta-1b. The patient had near complete resolution 

of his clinical symptoms and significant MRI 

improvement. 

Conclusions: Combination acute therapies like plasmapheresis 

and mitoxanthrone may reduce inflammatory cytokins 

in acute course of disease and may result in improvement. 

More multicenter clinical trial may help to 

understand nature of disease and management options. 

93 

T1738. Coma as an Inital Manifestation of Acute 

Disseminated Encephalomyelitis 

Megan McGarry, Natasha Tilluckdharry and Dipak P. 


Background: Acute Disseminated Encephalomyelitis 

(ADEM) is a monophasic autoimmune demyelinating disease. 

Clinical manifestations include an altered behavior, 

headache, seizures and coma. We report a unique case which 

was presented as a coma an initial manifestation of ADEM. 

Case Report 21 year old woman was found unconscious 

with severe metabolic acidosis, renal failure requiring urgent 

dialysis and ventilatory support. Magnetic Resonance Imaging 

(MRI) of the brain showed extensive T2 signal in the 

periventricular area, deep white matter and bilaterally in the 

basal ganglia and hippocampus with enhancement. Her 

CSF studies were unremarkable. She was treated with pulse 

dose of solumedrol. Because of lack of initial response, she 

was treated with eight cycles of plasmapheresis. She recovered 

completely from illness. She had several follow up 

MRIs with significant improvement. Her neuropsychological 

evaluations showed significant improvement and currently 

she is pursuing higher studies without any difficulties. 

Conclusion: Based on our literature, this is the first case 

of coma patient as an initial manifestation of ADEM. Our 

patient has complete recovery of physical and neurcognitive 

symptoms which make onliest clinical features and outcome. 

We think that prompt and aggressive management may 

improve outcome from catastrophic neurological sequel. 

T1739. NMO-IgG in a Patient with Neurosarcoidosis 

Lena Derani** and Elham Bayat; Washington, DC 

Neurosarcoidosis is a rare manifestation of sarcoidosis in 

which inflammation and abnormal deposits occur in the 

nervous system. Neuromyelitis optica (NMO) is an inflammatory 

demyelinating disease that primarily targets the spinal 

cord and optic nerves. NMO-IgG (anti-aquaporin-4) is 

considered to be a sensitive and specific marker for NMO. 

However, NMO-IgG has also been found in patients with 

NMO spectrum disorders (NMOSD), paraneoplastic disease 

and systemic lupus erythematosus. We present the first 

reported case of NMO-IgG positivity in a patient with neurosarcoidosis 

but no evidence of NMO. We discuss autoimmunity 

in neurosarcoidosis, the specificity of NMO-IgG 

and its likelihood in autoimmune diseases, and the possibility 

of concomitant neurosarcoidosis and neuromyelitis 

optica. We suggest the possibility that NMO-IgG is a nonspecific 

indicator of autoimmunity present in patients with 

other autoimmune disorders, including neurosarcoidosis. We 

remind neurologists that patients who test positive for 

NMO-IgG do not necessarily carry the diagnosis of NMO 

or NMOSD, despite the documented specificity and sensitivity 

of NMO-IgG for these diseases. Further studies are 

needed to investigate the positive predictive value of NMO- 

IgG and the prevalence of positive NMO-IgG in the normal 

population and in patients with autoimmune diseases. 

T1740. Correlates of Dietary Intake in Individuals with 

Multiple Sclerosis 

Matthew A. Plow, Marcia Finlayson and Chi Cho; Cleveland, 

OH and Chicago, IL 

Since the multiple sclerosis (MS) population has a similar 

life expectancy as the general population and is characterized 

by impairments that can interfere with healthy eating habits, 

malnutrition, vitamin deficiencies, and obesity are prevalent 

problems among MS patients. However, few studies have 

been conducted to provide clinicians with evidence-based 


approaches to support MS patients in developing healthy 

eating habits. Thus, the purpose of this study was to identify 

modifiable factors that predict dietary intake. Using 

data from an on-line validated survey of 271 MS patients, a 

stepwise multiple regression model was used to determine 

the relative importance of personal characteristics, symptoms, 

functional limitations, and coping skills in influencing 

dietary intake. Over 50% of the sample was overweight/ 

obese. Four variables made the model: self-efficacy (b ¼ 

0.51), physical activity (b ¼ 0.14), pessimism (b ¼ 0.13), 

age (b ¼ 0.12), and coping skills (b ¼ 0.10), which 

explained 40.5% of the variance in intake. Neither symptoms 

nor functional limitations significantly predicted 

intake. This is the first study to document a relationship 

between self-efficacy, coping skills, and nutritional habits in 

a large generalizable sample of MS patients. Future research 

should examine the effectiveness of efficacy-enhancing nutritional 

interventions that focus on improving coping skills. 

Study supported by: This work was supported through 

the National Multiple Sclerosis Society (NMSS) post-doctoral 

training grant. The information presented in this 

abstract does not necessarily reflect the position, ideas, or 

opinions of the NMSS 

Neurooncology 


T1801. Comparative Uptake and Cytotoxicity of Anti- 

Hu and Anti-Ri Antibodies in Rat Cerebellar Slice 

Cultures 

John E. Greenlee, Susan A. Clawson, Blair Wood, Kenneth E. 

Hill and Noel G. Carlson; Salt Lake City, UT 

Anti-Hu and anti-Ri antibodies label different neuronal antigens 

in western blots. However, both recognize intracellular 

antigens present in essentially all neurons. At autopsy, brains 

of patients with anti-Hu antibody show neuronal destruction. 

In contrast, anti-Ri antibody is less clearly associated with 

neuronal death, and patients with anti-Ri antibodies may 

respond to treatment. To study the effects of these two antibodies 

on living neurons, we incubated rat cerebellar slice 

cultures with either anti-Hu or anti-Ri antibodies and followed 

these cultures over time using TUNEL and FLICA 

methods to detect apoptosis, and uptake of SYTOX green to 

detect non-apoptotic cell death. Both anti-Hu and anti-Ri 

antibodies accumulated in essentially all neurons. Anti-Hu 

antibodies caused apoptosis within 72–96 hours in neurons 

throughout the cerebellum. In contrast, cells accumulating 

anti-Ri antibodies remained viable for over 200 hours, without 

evidence of apoptosis or necrotic cell death. Neurons in 

general may be able to internalize antibodies. Anti-Hu 

appears to be specifically cytotoxic, whereas anti-Ri appears 

less able to affect neuronal viability and may predominantly 

cause neuronal dysfunction rather than death. 

Study supported by: United States Department of Veterans 

Affairs 

T1802. Cerebrospinal Fluid Chemokine/Cytokine 

Biomarkers for Melanoma Brain Metastasis 

Edwin Lok, Amy S. Chung, Szexian Lee, Tamar Melman, 

Kenneth D. Swanson and Eric T. Wong; Boston, MA 

Because immunoregulatory chemokines and cytokines may 

modulate the biology of melanoma brain metastasis, we 

measured 26 of them in the CSF from 22 patients using 

multiplexed ELISA. Clustergram revealed segregation of 

non-disease controls from 5 identifiable clusters of patient 

94 

CSF with distinct chemokine/cytokine profiles. Cluster 1 

had the shortest time from initial melanoma diagnosis to 

brain metastasis (mean 7.5 months, P ¼ 0.0278). MIP-1b 

and IP-10 were elevated while GRO-a, IL-8, and Eotaxin 

were suppressed. Cluster 3 was associated with a shortened 

time from brain metastasis to death (mean 5.5 months, P ¼ 

0.0714). Prior biologics treatment also correlated with the 

shortest time interval from brain metastasis to death (mean 

5.9 months, P ¼ 0.0396). In cluster 3, MIP-1a was elevated, 

while GRO-a, MPIF-1, MIG, and IL-1b were suppressed. 

Cluster 4 was associated with a trend for prolonged 

overall survival (mean 78.0 months, P ¼ 0.0847). This cluster 

also had the longest survival from date of biologics treatment 

to death (mean 58.5 months, P ¼ 0.0132). Cluster 4 

had elevated MPIF-1 while GRO-a, IL-8, IP-10, MDC, IL- 

5, MIG, and IL-1b were suppressed. Specific cytokine/chemokine 

profiles measured in the CSF of melanoma patients 

may have prognostic and predictive value for melanoma 

brain metastasis. 

Study supported by: A Reason To Ride research fund. 

T1803. Role of p75NTR and Its Signaling Pathways in 

Fenretinide (4-hydroxyphenyl Retinamide – 4HPR) 

Induced Apoptosis in Neuroblastoma Cells 

Veena R. Ganeshan and Nina F. Schor; Rochester, NY 

Objective: To understand the role of p75NTR and its signaling 

pathways in Fenretinide(4-hydroxyphenyl retinamide- 

4HPR)-induced apoptosis and determine if JNK phosphorylation 

mediates this effect in neuroblastoma cells. 

Background: 4HPR is well tolerated in clinical trials and 

causes apoptosis by increasing oxidative stress(OS) in cancer 

cells. p75NTR has pro and anti-apoptotic functions 

depending on the cell milieu and type. 

Methods: S-type neuroblastoma cells (SH-EP1) transfected 

with varying amounts of p75NTR are used in the 

4HPR-concentration experiments. 4HPR treatment results 

in changes in JNK phosphorylation and OS, which are 

recorded using western blotting and fluorescent microscopy. 

Results: 4HPR treatment reduces viability in SH-EP1 

and SK-N-SH cells within 24(15%)-72 hrs(40%) and causes 

an increase in mitochondrial O2- production. A sustained 

increase in Akt phosphorylation occurs in SH-EP1 cells 

with lower p75NTR levels. Within 1hr of 4HPR treatment, 

JNK phosphorylation increases by 100–150% in SH-EP1 

cells with lower levels of p75NTR. 

Conclusion: JNK phosphorylation appears to be protective 

against 4HPR-induced apoptosis. This will facilitate the 

development of combination chemotherapeutics for residual 

disease treatment and recurrence by increasing OS and 

enhancing response to conventional chemotherapeutics. 

Study supported by: Studies presented in this abstract 

were funded by grants to Nina F. Schor from the 

National Cancer Institute (CA07429), the National Institute 

of Neurologic Disorders and Stroke (NS038569), 

and the William H. Eilinger Endowment for Pediatric 

Research of the Golisano Children’s Hospital at the University 

of Rochester Medical Center. These organizations 

played no role in the interpretation of the data or the 

substance of the review presented herein. Veena R. Ganeshan 

is a doctoral student in the Integrated Graduate Program 

in Neuroscience of the University of Rochester 

School of Medicine and Dentistry. Neither of the authors 

have conflicts of interest of relevance for this abstract. No 

additional individuals played any role in the production 

of this abstract. 



T1804. Relationship between Brain MRI Imaging 

Parameters and Molecular Biomarkers as Prognostic 

Indicators in Glioblastoma Multiforme 

Xiao-Tang Kong, Senxi Du, Beverly D. Fu, Mark E. Linskey 

and Daniela Bota; Orange, CA and Irvine, CA 

The purpose of this study is to explore the relationship 

between the potential MRI prognostic indicators and the 

molecular biomarkers in order to better evaluate the prognosis 

of the patients with GBM. 

Molecular biomarkers were determined using Immunohistochemistry. 

MIPAV software was used to quantify the volume 

of MRI parameters. 

Of the seventeen specimens that eventually included, 10 

had high and 6 low levels of MGMT expression; 5 were 

negative and 11 positive for EGFR wt amplification; 12 

negative and 5 positive for EGFRvIII expression; 14 had 

PTEN loss and 3 had intact PTEN. The vasogenic edema 

were more significant in the low MGMT expression group 

(P ¼ 0.04). The necrosis were larger in the group of non 

EGFR wt amplification (P ¼ 0.03) and the total volume of 

tumor involvement was significantly more in EGFRvIII variant 

expression group (P ¼ 0.0072). Our data suggest that 

the volume of vasogenic edema, necrosis and total tumor 

involvement are related to low MGMT level, lack of EGFR 

wt amplification, and increased EGFRvIII expression as 

prognostic indicators. Definitely, more cases will be added 

to the study to confirm the findings and to evalute the clinical 

prognosis. 

T1805. Secondary Intramedullary Spinal Cord Non- 

Hodgkin’s Lymphoma 

Eoin P. Flanagan, Brian P. O’Neill, Thomas M. Habermann 

and B. Mark Keegan; Rochester, MN 

Intramedullary spinal cord metastases are rare and associated 

with poor long-term survival. We present the clinical characteristics 

and outcome of secondary intramedullary spinal 

cord non-Hodgkin’s lymphoma (NHL) in 7 patients seen at 

Mayo Clinic from 1996–2010. All patients had myelopathy 

and imaging evidence of intramedullary spinal cord involvement 

of pathologically confirmed systemic NHL (B-cell, 6; 

T-cell, 1). Spinal cord NHL was diagnosed by: CSF cytology, 

4; PET hypermetabolism, 2; or MRI features alone, 1. 

In two, myelopathy was the initial presentation of systemic 

NHL and in the remainder the median time from NHL diagnosis 

to intramedullary involvement was 8 months (range, 

1–58). Median age was 60 years (range, 41–81) and 4 were 

wheelchair-dependent at diagnosis. MRI spine lesions (Thoracic, 

3; Cervical, 3; or both, 1) were typically gadolinium 

enhancing, expansile and associated with PET hypermetabolism. 

Two had leptomeningeal involvement and 4 had coexisting 

brain lesions. Six of the seven were treated: intravenous 

methotrexate, 3; radiation, 2; and R-CHOP, 1. Four 

improved (mild, 3; marked, 1) following treatment. The 

median survival was 11.5 months (range, 1–28). Secondary 

intramedullary spinal cord NHL appears to have longer survival 

than intramedullary metastases from most other 

cancers. 

T1806. Case Report: Optic Neuropathy in a Patient with 

Glioblastoma Receiving Bevacizumab 

Robert A. Fishman, Lara Kunschner and Erik Happ; 

Pittsburgh, PA 

Bevacizumab is a humanized monoclonal antibody developed 

to target and subsequently block vascular endothelial 

growth factor received FDA approval for single agent treatment 

of recurrent Glioblastoma multiforme in 2009. Estab- 

95 

lished Bevacizumab side effects include hypertension, 

delayed/impaired wound healing, gastrointestinal perforation 

and proteinuria. Recently a small number of case reports 

have reported six cases of optic neuropathy following Bevacizumab 

treatment for GBM. 

We report here a case of a 24 year old woman with Neurofibromatosis 

type I (NF-1) and right Thalamic GBM 

(WHO grade IV), who developed a severe, subacute optic 

neuropathy during treatment with Bevacizumab and Irinotecan 

for recurrent GBM following standard initial chemo 

radiotherapy with temozolamide. Following 4 cycles of salvage 

chemotherapy using Bevacizumab/Irinotecan, the 

patient developed diffuse blurring of vision, which progressed 

over 3-4 weeks-eventually resulting in total 

blindness. 

Six weeks following cessation of Bevacizumab the patient’s 

vision improved with return of light perception, and eventual 

return of baseline vision. Our completed poster will 

include a summary of our case, MRI images of the tumor, 

and results of ophthalmologic examination during and following 

Bevacizumab treatment. 

Study supported by: Residency program 

T1807. Anti-Ri-Associated Paraneoplastic Brainstem 

Cerebellar Syndrome with Medically-Intractable Nausea 

in a Patient with Large Cell Neuroendocrine Lung 

Carcinoma: A Case Report 

Amber N. Mitchell, Jessica Levesque and Earl Zimmerman; 

Albany, NY 

Paraneoplastic neurologic syndromes are rarely the first 

manifestations of occult malignancies, which if left 

untreated, often lead to significant morbidity and mortality. 

The presence of onconeural antibodies supports the paraneoplastic 

diagnosis, but is not absolutely essential. Anti-Ri 

autoantibodies are commonly associated with breast and 

small cell lung cancers. There are cases of anti-Ri paraneoplastic 

cerebellar degeneration reported, but none describe 

associated severe nausea and emesis as the major presenting 

and enduring symptoms. Here, we report a case of a 75year-old 

female with medically-intractable nausea, vomiting, 

diplopia, and vertigo for three months. Physical exam 

revealed rotary nystagmus, ocular skew deviation, limb dysmetria, 

and gait ataxia. After two courses of intravenous immunoglobulin, 

there was minimal improvement. Serum was 

Anti-Ri antibody positive. Computed tomography identified 

a 2.5 cm by 2.2 cm lung mass, and histopathology revealed 

large cell neuroendocrine carcinoma with smaller portions 

of adenocarcinoma. Post-lobectomy, the patient clinically 

improved. This is the first report of paraneoplastic brainstem 

cerebellar syndrome involving anti-Ri positivity from 

neuroendocrine tumor of the lung with nausea and vertigo 

as the major features and with improvement after tumor 

resection. 

T1808. Glioblastoma Multiforme: A Rare Presentation 

of Pineal Tumor with Leptomeningeal Seeding and 

Future Directions 

Noor Yono, Tulika Ranjan and Rabih Kashouty; Manhasset, 

NY, United States Minor Outlying Islands and New York, NY 

Glioblastoma Multiforme (GBM) is the most common primary 

malignancy of the Central Nervous System. Pineal 

GBM with leptomeningeal seeding is an exceedingly rare tumor. 

Only 18 cases have been reported in the literature. We 

present a case of pineal gland GBM with leptomeningeal 

disease treated with combination chemotherapy. A 55-yearold 

man presented with progressive headache, peduncular 


hallucinations and perinaud syndrome. Brain MRI showed a 

heterogeneously enhancing pineal mass measuring 2.1 cm 

with hydrocephalus and infiltration into the midbrain and 

thalamus. The pathology was consistent with GBM with 

Ki-67 labeling index of 30–50%. Brain and total spine MRI 

showed diffuse linear leptomeningeal disease. Chemotherapy 

with Temozolomide was started on day 1st. Two high-volume 

lumbar punctures with intrathecal Methotrexate were 

performed. Within 24 hours following the first Methotrexate 

treatment, the patient showed significant improvement. 

Initial lumbar puncture showed markedly elevated protein 

count of 2856. He was further treated with Avastin every 2 

weeks and monthly Temozolomide. Recent CSF protein has 

improved to 315 and the pineal mass size has decreased to 

0.9mm after four months of starting treatment. 

T1809. Benign-Histology Meningioma with Extracranial 

Metastasis 

Umer Akbar, Bhavpreet Dham and Melissa Carran; 

Camden, NJ 

Objective: To recognize extracranial dissemination from a 

meningioma 

Background: Meningiomas are regarded as benign 

tumors, as malignant histology is uncommon and extracranial 

metastasis is an extremely rare occurrence. 

Design: We report a case of a histologically-benign meningioma 

with metastasis to the lungs. 

Case: A 36 year-old male with symptomatic epilepsy presented 

with a breakthrough seizure. Patient had a meningioma 

resection in 1998. Few months later, imaging revealed 

recurrent tumor which was treated with 48 Gy of radiation. 

Routine chest x-ray followed by computed tomography scan 

confirmed multiple scattered round nodules, averaging 

between 1 to 1.5 cm. Fine-needle aspiration showed clusters 

of meningothelial cells consistent with metastasis from the 

recurrent intracranial tumor. 

Discussion: Majority of meningiomas are benign (grade 

I), while few are atypical (grade II) and malignant (grade III) 

histology is rare. Aggressive behavior and metastatic potential 

is mainly seen in atypical and malignant histologic subtypes. 

With a metastasis rate of 0.1%, meningiomas are seldom 

seen extracranially. Very rarely, the metastasizing meningioma 

is of benign histology. Certain tumor tendencies for aggressive 

behavior have been observed and are discussed. 

Neurovirology 


T1901. HIV Associated Neurocognitive Disorder 

(HAND) Is Not Associated with Increased Fibrillar 

Amyloid Deposits Using 11 C-PiB in Middle-Aged HIVþ 

Participants 

Beau Ances, Jewell Thomas, Tammie Benzinger, Jon 

Christensen, Mengesha Teshome, Patricia Aldea, Anne Fagan, 

David Holtzman, John Morris and David Clifford; Saint 

Louis, MO 

Objectives: Diagnostic challenges exist for differentiating 

HIV associated dementia (HAD) from Alzheimer disease 

(AD). Similar abnormalities in brain amyloid-b42 (Ab42) 

metabolism occur for both. We evaluated if 11 C-Pittsburgh 

compound B ( 11 C-PiB) discriminates AD from HAND. 

Methods: In this case-control study 16 HIVþ (11 cognitively 

normal, 5 HAND) and 19 HIV- (8 cognitively normal, 

11 symptomatic AD) participants were assessed using 

11 

C-PiB, clinical exam, and cerebrospinal fluid (CSF) testing. 

v 2 and t-tests investigated differences in clinical and de- 

96 

mographic variables amongst groups. An ANOVA assessed 

11 C-PiBregional differences. 

Results: Symptomatic AD were older (p < 0.001), had 

lower CSF Ab 42 (p < 0.001), and had higher CSF tau levels 

(p < 0.001). Regardless of degree of impairment, HIVþ 

participants had no increased 11 C-PiB. Mean and regional 

binding potentials were elevated for symptomatic AD participants 

(p


Study Design: The regional virology database was 

screened to identify VZV positive CSF over 5years. Viral 

load was determined by quantitative-polymerase chain reaction 

(qPCR). 

Results: 608 patients were screened, 36 had VZV qPCR 

performed, 12 were positive and had encephalitis. There was a 

strong negative correlation between time to LP and viral load 

(tau b ¼ 0.59, p ¼ 0.036). There was no significant difference 

in viral load between those with good and poor outcome. 

Conclusions: CSF viral load appears to more closely 

relate to time to LP than outcome. Timing of LP should be 

included in future studies to investigate the role of the viral 

load in the pathophysiology and prognosis of encephalitis. 

Study supported by: Study funded by the National Institute 

for Health Research (NIHR-UK) Biomedical Research 

Centre. 

T1904. Acute Varicella Zoster Virus Encephalitis in 

Adults – Relationship between Viral Load, Time, 

Clinical Features and Outcome 

Benedict D. Michael, Michael Griffiths, Anna Stewart, 

Charlotte Buckley, Mark Hopkins, Ian J. Hart, Alistair Miller, 

Sareen E. Galbraith and Tom Solomon; Liverpool, United 

Kingdom 

Background: Varicella zoster virus (VZV) is a common 

cause of viral encephalitis with a wide spectrum of outcomes. 

The pathophysiology remains poorly understood and 

few prognostic markers exist. A few studies have used viral 

load, determined by quantitative polymerase chain reaction 

(qPCR) of cerebrospinal fluid (CSF), to investigate this, 

with contradictory results. However, these have not assessed 

this in the context of time. 

Objectives: To determine the viral load in VZV encephalitis 

in relation to time and outcome. 

Study Design: The Liverpool Specialist Virology laboratory 

was screened to identify patients with VZV-positive 

CSF over 5years. Viral load was determined. Poor outcome 

was defined as moderate disability-death (Glasgow outcome 

score [GOS]2-5) and high viral load as (>5 10 4 GEq/ml). 

Results: Of 608 screened; 36 had VZV-PCR; 12 were 

positive. There was a strong negative correlation between 

time from symptoms to LP and viral load (tau b ¼ 0.59, 

p ¼ 0.036). There was no difference in viral load between 

those with good and poor outcome. 

Conclusions: In VZV encephalitis, viral load appears to 

better relate to time. Timing should be included in future 

studies aiming to relate viral load to outcome. 

Study supported by: This study recieved support from 

the National Institute for Health Research Biomedical 

Research Centre on Infectious Diseases. The authors have 

no other conflicts of interest to declare 

T1905. TLR4 Expression Is Upregulated in HIV- 

Associated Neurocognitive Disorder (HAND) 

Amir H. Sabouri, Gursharan Chana, Amol Shah, Critian L. 

Achim, Ronald J. Ellis, Ian P. Everall and HNRC Group; La 

Jolla, CA; Melbourne, Australia and San Diego, CA 

Despite the introduction of highly active antiretroviral therapy 

(HAART), HIV associated neurocognitive disorders 

(HAND) remain a significant cause of morbidity. In a previous 

investigation, we have demonstrated that the gene 

expression of a number of proteins related to the innate 

immune system toll-like receptor (TLR) signaling pathway 

correlate strongly with HIV associated neurodegeneration, 

most significantly TLR3 and 4. In this study, we examined 

gene expression of TLR4, the major signaling receptor 

97 

for lipopolysaccharide (LPS), within post-mortem brain tissue 

from HIV positive subjects (n ¼ 15) and HIV negative 

subjects (n ¼ 10) via quantitative real time PCR (qRT- 

PCR) and immunohistochemistry (IHC). We also assessed 

cognitive brain functions in 7 domains and their correlation 

with TLR4 mRNA expression. We found TLR4 expression 

was significantly higher in HIV positive subjects than HIV 

negative controls (p


All patients recieved treatment with Amphotericine B and 

fluconazole.Only half completed the induction phase as 

recommended. 

Discussion: Clinical presentation, CSF findings of 

patients presenting with MC in our hospital do not differ 

from what has been reported in the medical literature. This 

findings confirms the need to mantain a high suspicion 

index of CM in immunocompromised or immunocompetent 

patients with suggestive clinical picture. 

Special attention must be paid to appropiate treatment schemes 

in the future to achieve better outcomes in this population. 

Study supported by: The authors declare no conflict of 

interest. None of the authors recieved any financial benefits 

from the study. 

T1908. The Diagnosis of Tuberculous Meningitis: A 

Current Review of the Clinical and Laboratory Methods 

Brian Chisulo, Shan H. Jiang and Yue S. Pan; Guangzhou, 

GZ, China 

This review traces our understanding of the clinical 

manifestations and the laboratory methods commonly 

98 

considered in the diagnosis of tuberculous meningitis 

(TBM). In compiling this review, we searched electronic 

data bases in PubMed, Science Direct, Biomedical Central 

and Google Scholar. Firstly, we evaluate the more 

traditional diagnostic methods which have been commonly 

applied in the diagnostics of tuberculosis (TB). 

The role of direct cerebrospinal fluid (CSF) examination 

for acid-alcohol fast bacilli, CSF culture for Mycobacterium 

tuberculosis, and detection of mycobacterial nucleic 

acid in the CSF is evaluated. We also consider the role 

of brain imaging and chest X-ray. Secondly, the review 

evaluates the current evidence on the role of some newly 

prospective diagnostic techniques and the coverage is 

given to the role of CSF adenosine deaminase activity, 

Gen Probe amplified Mycobacterium tuberculosis direct 

test, microscopic observation drug susceptibility 

(MODS) culture technique, ex vivo Mycobacterium tuberculosis-specific 

enzyme-linked immunospot assay 

(ELISpot assay) and enzyme-linked immunosorbent assay 

(ELISA) in the diagnosis of TBM. 

Study supported by: This was a self-financed study. 




Progress Poster Session Abstracts 












S141. Polymorphism of Ninjurin2 in Korean 

Atherothrombotic Stroke Patients 

Dae-il Chang, Sung Hyuk Heo and Hye Ok Kim; 

Seoul, Korea 

Recently, in a genome-wide association study, the singlenucleotide 

polymorphisms (SNPs) rs12425791 and 

rs11833579 of ninjurin2 (NINJ2) were reported to be associated 

with atherothrombotic stroke. However, this finding 

has not been validated in Korean population yet. We therefore 

investigated an association between atherothrombotic 

stroke and SNPs of NINJ2 in 200 patients and 295 control 

subjects. Allelic discrimination assay was used. The A allele 

of rs11833579 increased stroke risk significantly (p ¼ 

0.003, 1.50 (95% CI, 1.15 to 1.96), respectively). The A allele 

frequencies were higher in stroke patients with hypertension 

than in control subjects with hypertension (p ¼ 

0.042 and p ¼ 0.001, respectively) in both SNPs of NINJ2 

(rs12425791 and rs11833579). Also, the A allele was more 

frequent in stroke patients with hyperlipidemia than in control 

subjects with hyperlipidemia (p ¼ 0.010 and p ¼ 

0.005, respectively) in both. In addition, the A allele was 

more frequent in stroke patients with diabetes than in control 

subjects with diabetes (p ¼ 0.008, respectively) in SNPs 

rs11833579. In conclusion, SNPs of NINJ2 (rs12425791 

and rs11833579) confer a substantial genetic risk factor for 

atherothrombotic stroke in our population. 

S142. NMDA Receptor Biomarker for Acute Stroke 

Kerstin Bettermann and Svetlana Dambinova; Hershey, PA 

and Kennesaw, GA 

Background: The NR2 peptide, a breakdown product of 

brain specific NMDA receptors, is released into the bloodstream 

during cerebral ischemia and can be used as a marker 

for acute ischemic stroke (IS). Performance characteristics 

and critical values of the NR2 peptide in acute IS patients 

presenting within 24h of symptom onset are analyzed. 

Methods: NR2 peptide levels are correlated with neurological 

status and results of advanced neuroimaging studies. 

Plasma NR2 peptide concentrations are determined by plotting 

their absorbance values on a calibration curve constructed 

from the absorbance units of each calibrator and 

their known concentrations. 

Results: Controls and individuals with vascular risk factors 

have NR2 peptide concentrations below 0.25 and 0.3 

lg/l, respectively. Acute IS patients have significantly higher 

NR2 peptide levels (>0.5 lg/l) compared to all control 

groups (p75 cm/s was strongly predictive of symptomatic 

MCA vasospasm. The 3, 7, 10, and 14-day risks of symptomatic 

MCA vasospasm were 0.9%, 9.4%, 14.8%, and 

14.8% for those with initial MCA-MFV 75 cm/s (log-rank test: P ¼ 0.001). 

Elevated initial TCD velocity may provide a useful marker 

for those at high-risk for symptomatic vasospasm. The risk 

of vasospasm in those with elevated MCA velocity > 75 

cm/s was significantly higher, occurring earlier and for a 

longer period, than in those without. 

S144. Relative Change in Transcranial Doppler Velocities 

Is Inferior to Absolute Thresholds in Prediction of 

Vasospasm after Subarachnoid Hemorrhage 

Konark Malhotra, James Connors, Viven Lee and Shyam 

Prabhakaran; Chicago, IL 

We reviewed 350 consecutive SAH patients with aneurysmal 

source, who underwent serial TCD monitoring and survived 

at least 7 days. We recorded initial flow velocity(IFV) upto 

14 days, maximal flow velocity of middle cerebral artery 

(MCA), relative flow changes (MFV/IFV) >3, absolute 

maximal Lindegaard ratio (LR) >6, mean MCA maximum 

flow velocity (MFV) >175 cm/s, and MFV >200 cm/s. 

Among 211 patients selected, 48 (23%) developed symptomatic 

MCA vasospasm. Area under the ROC curve (AUC) 

was: MCA-MFV >175 ¼ 0.80, MFV >200 ¼ 0.73, LR 

>6 ¼ 0.71, and MFV/IFV >3 ¼ 0.60. The best characteristics 

were observed for MFV >175 cm/s or maximal LR 

>6 as: AUC ¼ 0.81; while sensitivity, specificity and negative 

predictive value were: 81%, 81% and 94%, respectively; 

the positive likelihood ratio was 4.1. MFV/IFV >3 performed 

the worst. MFV >175 cm/s and/or Lindegaard ratio 

>6 accurately predict symptomatic MCA vasospasm. Presence 

of either/both abnormalities in first 14 days after SAH 

increases the likelihood of symptomatic MCA vasospasm by 


4 fold. Other thresholds including relative change from 

baseline appear inferior to these absolute thresholds. 

Movement Disorder 


S238. Dopamine Transporter Imaging Predicts Long 

Term Outcomes in Parkinson’s Disease 

Bernard M. Ravina, Kenneth Marek, Shirley Eberly, David 

Oakes and Ira Shoulson; Rochester and New Haven 

Objective: Our objective is to measure the prognostic value 

of dopamine transporter (DAT) imaging for motor and 

non-motor outcomes in PD. 

Methods: We prospectively evaluated a PD cohort after 

enrollment in a de novo PD clinical trial with motor, cognitive, 

and behavioral measures. DAT imaging with [ 123 I][b]- 

CIT (b-CIT) and SPECT was performed at baseline and after 

22 months. 

Results: Four hundred and ninety-one (91%) of 537 subjects 

had evidence of dopamine deficiency consistent with 

PD. The cohort was followed for 5.5 (0.8) years and had a 

diagnosis of PD for 6.3 (1.2) years. Lower striatal binding 

of b-CIT at baseline was independently associated with significantly 

higher risk for important clinical milestones and 

measures of PD severity, including motor related disability, 

falling and postural instability, cognitive impairment, psychosis, 

and clinically important depressive symptoms. Subjects 

in the bottom quartile for DAT imaging compared to 

the top quartile had an odds ratio (95% CI) of 3.5 (1.7, 

6.9) for cognitive impairment and 12.8 (2.6, 61.8) for 

psychosis. 

Conclusions: Near the time of PD diagnosis, DAT imaging 

with b-CIT and SPECT is an independent predictor of 

clinically important motor and non-motor long term 

outcomes. 

Study supported by: This study was sponsored by the 


(NINDS) (5U01NS050095), Department of Defense 

(DOD) Neurotoxin Exposure Treatment Parkinson’s 

Research Program (W23RRYX7022N606), Michael J Fox 

Foundation (MJFF), Parkinson’s Disease Foundation (PDF), 

Cephalon Inc, and Lundbeck Inc. 

S239. Time to First Levodopa-Induced Motor 

Complication: Results from the STRIDE-PD Study 

C. Warren Olanow, Karl Kieburtz, Olivier Rascol, Werner 

Poewe, Anthony Schapira, Helena Nissinen, Mika Leinonen 

and Fabrizio Stocchi; New York, NY; Rochester, NY; Toulouse, 

France; Innsbruck, Austria; London, United Kingdom; Espoo, 

Finland; Kista, Sweden and Rome, Italy 

Introduction: The STRIDE-PD study compared initiation 

of levodopa/carbidopa (LC) to levodopa/carbidopa/entacapone 

(LCE) in PD patients. 

Objective: Determine the effect of levodopa dose on development 

of motor complications. 

Methods: A total of 745 patients participated in a 134– 

208-week, double-blind trial, with 3-monthly assessments of 

dyskinesia and wearing-off. Based on nominal levodopa 

dose per day, patients were divided into four groups: 400–600 mg (26.6%), 

and >600 mg (7.4%). Dose response was analyzed using 

log-rank test. Predictive factors for first motor complication 

were screened using multivariate analysis. 

Results: A dose response was observed between levodopa 

dose and first motor complication (p


Outcome measures: Dyskinesia score(primary); fingertapping 

and walking speed, tremor score and blood 

pressure(secondary). 

Analysis: Area Under Curve statistical paradigm 

Results: Single doses of 100mg of preladenant increased 

levodopa-induced dyskinesia compared to placebo(p ¼ 

0.003). Single doses of 10mg were not statistically significantly 

different from placebo(p ¼ 1.00). We found a trend 

suggesting a dose-response for dyskinesia, and observed dyskinesia 

in 4 of 12 subjects prior to levodopa infusion. Neither 

dose significantly improved any measure of parkinsonism. 

The drug was generally well-tolerated. 

101 

Conclusions: This proof of concept study does not support 

the hypothesis that A2A antagonists augment the 

effects of levodopa without increasing dyskinesia. Our 

results suggest that a supratherapeutic dose of preladenant 

may be pro-dyskinetic, perhaps independently of levodopa, 

in PD patients with pre-existing dyskinesias. 

Study supported by: Merck & Co, Inc 

Presenting author Hogarth and co-authors Nutt & Park 

received salary support from payments made by the sponsor 

to OHSU for contracted research design and execution services. 

Co-authors Troyer, Laguerre, Grachev, Xuan, and Tendolkar 

are/were employees of the sponsor. 















M622. New Approaches to Unraveling the Multi-Scale 

Neuroanatomical Changes in Williams Syndrome 

Ann Lam and Elan L. Ohayon; La Jolla 

In this study we explore novel multi-scale approaches to 

investigate the role of neuroanatomy in cognitive phenotypes 

by imaging the distribution of metalloproteins across 

scales in Williams Syndrome tissue. Synchrotron-based rapid 

scanning imaging was used at whole hemisphere levels and 

resolutions of up to 10 microns to co-localize elements in 

postmortem samples of Williams Syndrome and typicaldeveloping 

individuals in prefrontal, temporal and occipital 

cortex. Comparisons between- and within-subjects revealed 

differences between brain areas in elemental distributions. 

For example, initial scans showed the expected high levels of 

iron in nuclei such as the substantia nigra and prominent 

zinc florescence along white matter tracts. To increase the 

resolution by a factor of 20, microprobe imaging was then 

used attaining resolutions as high as 0.5 microns. This 

microprobe analysis of 50–100 micron thick cortical sections 

showed the co-localization of iron, zinc and calcium 

in and around cell-like structures. The combination of these 

two synchrotron-based techniques thus enables the study of 

biologically relevant elements in postmortem brain tissue 

across large areas, while allowing for the co-registering of 

elements at high resolutions at the cellular level in regions 

of interest. 


Epilepsy 


M725. HLA-B*1502 Genotyping in Carbamazepine and 

Phenytoin Induced Stevens-Johnson Syndrome 

Sivakumar M. Rajappa and Srinivasan A. Venkatesan; 

Chennai, Tamil Nadu, India 

Objective: To study the association of HLA-B* 1502 allele 

in epileptic patients with carbamazepine and phenytoininduced 

Stevens Johnson Syndrome (SJS) in South Indian 

patients. 

Background: SJS and Toxic Epidermal Necrolysis (TEN) 

are severe cutaneous adverse drug reactions caused by carbamazepine 

(CBZ) and phenytoin. A strong association has 

been reported between human leucocyte antogen HLA- 

B*1502 and carbamazepine-induced SJS in Han Chinese 

patients. 

Design/Methods: Single center, prospective cross sectional 

study. After an informed consent, 7 ml of EDTA 

blood were collected from 100 patients on CBZ and 50 on 

102 

phenytoin therapy. Purified DNA were used for HLA- 

B*1502 typing by Sequence Specific Oligopeptide method 

using Polymerase Cahin Reaction. 

Results: Out of 136 patients on CBZ, HLA-B*1502 

were detected in 22 and 19 developed SJS/TEN. In 50 

patients on Phenytoin, HLA-B*1502 were detected in six 

patients and four had SJS. The mean duration from exposure 

to SJS was 14 days. 

Conclusion: There is a high prevalence of HLA-B*1502 

in South Indians on Carbamazepine and Phenytoin 

(18.66%). There was a strong association of HLA-B*1502 

allele and the developmment of SJS in South Indians, with 

the Positive Predictive Value for Carbamazepine and Phenytoin 

being 86% and 66.67% respectively. 

M726. Filamin A Regulates Neural Progenitor 

Proliferation and Brain Size through wee1-Dependent 

cdk1 Phosphorylation 

Gewei Lian and Volney Sheen; Boston, MA 

Mutations in the actin binding filamin A (FlnA) gene cause 

the human disorder periventricular heterotopia (PH). FlnAdependent 

regulation of cytoskeletal dynamics is thought to 

direct neural progenitor migration and proliferation. Here 

we show that late embryonic FlnA null mice exhibit a 

reduction in brain size, impairments in neural progenitor 

proliferation, and decline in neural progenitor numbers over 

time. The drop in the progenitor population is not attributable 

to changes in cell fate specification through altered 

asymmetric versus symmetric cell divisions, but to prolonged 

cell cycle duration. Suppression of FlnA leads to 

impaired degradation of cyclin b1-related proteins, thereby 

delaying the onset and progression through mitosis. We find 

that the cdk1 kinase wee1 binds FlnA, demonstrates 

increased expressions levels after loss of FlnA function, and 

is associated with increased phosphorylation of cdk1. Phosphorylation 

of cdk1 inhibits activation of anaphase promoting 

complex degradation system, which is responsible for 

cyclin b1 degradation and progression through mitosis. Collectively, 

our results demonstrate a molecular mechanism 

whereby FlnA impairs G2 to M phase entry, leading to cell 

cycle prolongation, impaired neural progenitor proliferation, 

and reduced brain size. 

Study supported by: This work was supported in part by 

the National Institutes of Health NS063997-01 to VLS. 

This work was also supported in part by the Empire State 

Stem Cell Fund through the New York State Department of 

Health Contract #C024324 to VLS. The opinions expressed 

here are solely those of the author and do not necessarily 

reflect those of the Empire State Stem Cell Board, the New 

York State Department of Health, or the State of New York. 

VLS is a Doris Duke Clinical Scientist Developmental 

Award Recipient. 

M727. A Moderate-Throughput Screen for 

Antiepileptogenic Compounds 

Yevgeny Berdichevsky, Yero Saponjian, Michelle Mail and 

Kevin J. Staley; Boston, MA 

Epilepsy is a frequent complication of traumatic, infectious 

and ischemic brain injury. The time interval between injury 

and epilepsy provides a tremendous opportunity for diseasemodifying 

therapies. Development of these therapies is limited 

by inadequate knowledge of the pathophysiology of epileptogenesis, 

the long time intervals required to observe 

changes in experimental epileptogenesis, and difficulties 

quantifying epileptogenesis. 



To address these difficulties we have developed a moderate-throughput 

drug screen for antiepileptogenic agents 

comprised of arrays of organotypic hippocampal brain slices 

that develop robust spontaneous electrographic seizure activity 

within two weeks in vitro. Seizure activity is quantified 

either electrographically or metabolically as cumulative lactate 

production over two weeks in vitro. Neuroprotection is 

quantified as cumulative lactate dehydrogenase release over 

two weeks in vitro. 

Using these techniques we have screened 50 compounds 

from the NINDS Custom Compounds Collection in less 

than 4 months. Most anticonvulsants demonstrated neither 

antiepileptogenic nor significant chronic anticonvulsant 

effects, although acute anticonvulsant effects were readily 

observable. With other compounds we have resolved chronic 

anticonvulsant or antiepileptogenic activities (which are 

indistinguishable in the first-level screen) evidenced by 

decreased lactate with no increase in LDH, as well as neuroprotective 

activity, evidence by reduction in LDH. 

Study supported by: NIH (NINDS) 

M728. Network Structure and Sensitivity to the 

Geometry of Stimuli in Epilepsy and Cognition 

Elan L. Ohayon, Ann Lam and Terrence J. Sejnowski; La 

Jolla, CA 

What modulates a brain’s sensitivity to stimuli? This question 

is central to the study of reflex epilepsies and cognition. 

Previously we have shown that network structure can affect 

the persistence of complex activity. Here we use computational 

models to demonstrate how anatomical changes can 

modulate sensitivity to initial conditions such as perceptual 

stimuli. Models consisted of large spatial networks with up 

to 20,000 spiking units with inhibitory and excitatory populations. 

Network structure was adjusted by randomly adding 

or removing cells at 5% increments. Simulations showed 

that homogeneous networks (density ¼ 1.0) were least likely 

to maintain activity with either single or distributed stimuli. 

In contrast, heterogeneous networks became more sensitive 

to a variety of stimuli (e.g., over 95% persistence at density 

¼ 0.6). Interestingly, the self-propagating responses generated 

in heterogeneous networks could be ‘‘grafted’’ back 

onto the homogenous networks and continue propagating. 

These experiments illustrate that changes in anatomy 

can modulate responses to stimulus geometry thereby helping 

explain how neurodegeneration and trauma might lead 

to changes in sensitivity and the lowering of seizure threshold. 

The findings also illustrate how network structure 

might modulate responses to stimuli in attention and 

cognition. 


M729. Hypoglycemia Induced NMDA Receptor- 

Dependent Epileptiform Activity in the Hippocampal 

CA3 Area Causes Damage in CA1 

Carlos M. Florez, Jane Zhang, Peter Abdemalik, Liang Zhang 

and Peter L. Carlen; Toronto, ON, Canada 

Seizures are the most common clinical presentation of severe 

hypoglycemia in neonates. We characterized a new in vitro 

model for the study of hypoglycemia-induced seizures using 

mouse hippocampal thick slices. Changing the glucose concentration 

to 1mM produced seizures in 88% of the preparations. 

Isolation experiments revealed that CA3, less susceptible 

for hypoglycemia induced synaptic failure than CA1, is 

the epileptogenic area. The CA3 network showed decreased 

interneuronal activity and increased frequency and energy 

transfer of the pyramidal cell activity prior to the onset of 

103 

seizures. It was common to observe spreading depressionlike 

events (SD) and irreversible synaptic failure in the CA1 

region following the seizure activity originated in CA3 (8/ 

12). SD also occasionally occurred in the CA3 area (3/12) 

following a SD event in the CA1 region. Hypoglycemic 

seizures required glutamatergic activity and NMDA receptor 

blockade prevented the hypoglycemia-induced seizures and 

SDs. 

Conclusions: Hypoglycemia-induced seizures in the hippocampus 

start in the CA3 region and cause SD and synaptic 

failure in the CA1 region. Activation of NMDA receptors 

is the key factor for the initiation of seizures and 

development of brain failure during severe hypoglycemia. 

Study supported by: JDRF 

M730. Alzheimer Disease in Lafora Epilepsy 

Jesus Machado-Salas, Maria Rosa Avila-Costa, Patricia 

Guevara, Jorge Guevara, Reyna M. Duron, Dongsheng Bai, 

Miyabi Tanaka and Antonio V. Delgado-Escueta; Los Angeles, 

CA and Mexico, Mexico 

Here we describe temporal progression of tau and amyloid 

pathology and subtypes of Lafora bodies (LB) in a Laforindeficient 

(epm2a / ) mice model. Wild type and 

epm2a / mice were sacrificed at different ages. Histological 

and immuonocytochemical techniques were used. We 

found in epm2a / mice that neurofibrilary tangles appear 

at 11 days-old while senile plaques are abundant at 6 

months. Type I LB are abundant inside neurons, type II LB 

are confined to neuron somata. Type I LB appear at 1 

month while type II LB with an external rim staining for 

Neurofilament L, appear at 3 to 5 months. Both types of 

LB increase in size and number with age but show regional 

differences. Diencephalon has meager or no LB, cell death 

and neurofibrillary tangles. All three features are abundant 

in hippocampus, amygdala, cerebellum, and in greatest 

amount in pons. Our results indicate that Lafora Disease is 

both a neurodegenerative disease and a glycogen metabolism 

disorder. We hypothesize that laforin deficiency upregulates 

tau protein kinase I/glycogen synthase kinase 3beta forming 

tau, amyloid and polyglucosan bodies. Our findings are critical 

for future experiments on disease mechanisms and 

therapies for both Lafora disease and Alzheimer disease. 

Study supported by: National Institutes of Health 

[1RO1NS055057], Chelsea’s Hope Foundation for Lafora 

Disease and Programa de Apoyo a Proyectos de Investigación 

e Innovación Tecnológica-DGAPA UNAM 

[IN214609, and PAPCA 2010-2011] 

M731. Antiepileptic Activity of Intrapulmonary 

Midazolam 

Ashish Dhir, Dorota Zolkowska and Michael A. Rogawski; 

Sacramento and Davis 

Midazolam is a short-acting central/peripheral benzodiazepine 

receptor modulator. It is widely used as an intravenous 

sedative and a fast-acting anticonvulsant to abort status epilepticus. 

In the present study, we sought to characterize 

intrapulmonary route to deliver midazolam for providing 

fast and potent antiepileptic action. Further, we explored 

the involvement of both central and peripheral benzodiazepine 

receptors in its mechanism of anticonvulsant action. 

Intraperitoneal administration of midazolam at 250–5000 

lg/kg protected mice against pentylenetetrazol, picrotoxin 

and kainic acid-induced convulsions. Similarly, the intratracheal 

route of midazolam administration demonstrated anticonvulsant 

activity but at much lower doses (25–100 lg/ 

kg); the maximum anticonvulsant action was observed 


etween 5–15 minutes. The anticonvulsant action of intratracheal 

midazolam (100 lg/kg) in PTZ i.v. seizure model 

was reversed by pre-administration of flumazenil (5 mg/kg., 

i.p.), a centrally acting benzodiazepine receptor antagonist. 

Finasteride (100 mg/kg., i.p.), a 5-alpha reductase inhibitor 

and neurosteroid synthesis inhibitor partially blocked the 

anticonvulsant action of midazolam in PTZ seizure threshold 

model. Furthermore, the anticonvulsant action of intraperitoneal 

midazolam was reversed by PK-11195, a peripheral 

benzodiazepine receptor antagonist. In conclusion, the 

intrapulmonary midazolam provides fast and potent anticonvulsant 

action and involves both central and peripheral 

receptors in its mechanism of action. 

Study supported by: Epilepsy Laboratory 

M732. Do Brain Volumes in JME (juvenile myoclonic 

epilepsy) differ from normal controls? 

John T. Spitz, Long Vu, Lydia Su, Mark A. Mandelkern, 

Barbara E. Swartz; Berkely, CA, Irvine, CA, Los Angeles, CA, 

Irvine,CA and Newport Beach, CA 

Background: JME is a form of generalized epilepsy that 

accounts for 7% of all epilepsies. The natural history of 

JME and its brain morphology is explored in this study. 

Methods: We compared the volumes of 11 regions of interest 

(frontal and subcortical) and grey matter density 

between 17 JME subjects (33.8 þ/ 10.4 years) with sex 

and age-matched controls (35.4 þ/ 11.7) using automated 

and manual volumetric procedures and voxel based morphometry 

(VBM). Regional volumes were normalized to 

whole brain volumes prior to statistical analyses. 

Results: Initial analysis of the volumetric data using ttests 

with Bonferroni correction found no significant differences 

between the JME and controls. However, individual 

subjects with JME were more likely to have regions larger 

than control mean volumes than visa versa using VBM and 

volumetry (p ¼ .04, Binomial test, VBM). Additionally, 

both VBM and manual volumetric analysis found the left 

thalamus larger in JME subjects. These results will be 

expanded using ANCOVA and/or MANCOVA analyses to 

control for a number of covariates: duration of epilepsy, sex, 

seizure control and medications for final presentation. Discussion 

will emphasize the growing literature in this area. 

Study Supported By: Veteran’s Administration Merit 

Review Program 821/103. 



M836. Disrupted Expression of Myogenin in Inclusion 

Body Myositis 

Akatsuki Kubota, Jun Shimizu, Atsushi Iwata and Shoji Tsuji; 

Tokyo, Japan 

Background: Inclusion body myositis (IBM) is a chronic 

progressive inflammatory myopathy, with degenerative pathology. 

Immunosuppressive therapies are ineffective to 

IBM, unlike other idiopathic inflammatory myositis (IIM). 

Muscle regeneration is also impaired in IBM, but the mechanism 

remains undetermined. Myogenin is one of the myogenic 

regulatory factors, and plays an essential role in maturation 

of myogenic cells. 

Purpose: We evaluated expression of myogenin in biopsied 

muscles from IBM and IIM patients. 

Materials and methods. We analyzed biopsied samples 

from 11 IBM and 19 IIM patients. We quantified myogenin-positive 

cells by immunohistochemistry, myogenin protein 

by Western blot, and myogenin mRNA by real-time 

reverse transcription PCR (RT-PCR). 

104 

Results: Biopsied samples from IBM patients showed 

lower expression of myogenin than those from IIM patients, 

in immunohistochemistry (IBM 4.761.4 cells/100myofibers, 

IIM 7.363.7, p ¼ 0.0352), Western blot (IBM 

0.04860.030 (relative amount to actin), IIM 0.09060.056, 

p ¼ 0.0297), and real-time RT-PCR (IBM 10.668.5 (relative 

amount to b-actin), IIM 19.6611.7, p ¼ 0.0344). 

Immunohistochemistry of biopsied samples from IBM 

patients also revealed that myogenin was aberrantly 

expressed within degenerative myofibers, and was aggregated 

in Ab-positive inclusion bodies. 

Conclusion: Disrupted expression of myogenin may 

reflect impaired regeneration in IBM. 

Study supported by: Grants-in Aid for Scientific Research 

and Health and Labour Sciences Research Grants for 

Research on Intractable Diseases from the Ministry of 

Health, Labour and Welfare of Japan, and Grants-in Aid for 

Scientific Research from the Ministry of Education, Culture, 

Sports, Science and Technology of Japan. 

M837. Abnormalities of a Novel Autophagy-Associated 

Protein, NBR1, in Muscle Fibers of Sporadic Inclusion- 

Body Myositis (s-IBM) 

Carla D’Agostino, Anna Nogalska, Mafalda Cacciottolo, 

W.King Engel and Valerie Askanas; Los Angeles, CA 

Intra-muscle fiber accumulation of ubiquitinated protein 

aggregates containing several conformationally modified proteins, 

including amyloid-b and phosphorylated tau, is characteristic 

of the pathologic phenotype of s-IBM, the most 

common degenerative myopathy of older persons. Impaired 

protein-degradation, involving both the 26S proteasome and 

autophagic-lysosomal pathways, we previously demonstrated 

in s-IBM muscle. NBR1 is a ubiquitin-binding scaffold-protein 

importantly participating in autophagic degradation of 

ubiquitinated proteins. Abnormalities of p62, another ubiquitin-binding 

protein, were previously described in s-IBM. 

Abnormalities of NBR1 have not been reported in s-IBM. 

We have now identified in 14 s-IBM muscle biopsies 

that NBR1, by: a) immunohistochemistry, was strongly 

accumulated within s-IBM muscle-fiber aggregates, where 

it closely co-localized with p62 and phosphorylated tau; 

b) immunoblots, was increased 3x, p< 0.01; and c) immunoprecipitation, 

was associated with p62. By real-time PCR, 

NBR1 mRNA was increased 2x, p< 0.01. None of the 15 

disease- and normal-control muscle biopsies had any NBR1 

abnormality. 

This first demonstration of NBR1 abnormalities in s- 

IBM: a) further suggests that abnormalities of the autophagic-lysosomal 

pathway may be critically involved in the s- 

IBM pathogenesis, and b) indicates a potential therapeutic 

focus. 

Study supported by: Muscular Dystrophy Association 

M838. Atrophy and Autophagy in Limb Girdle Muscular 

Dystrophy and Glycogen Storage Disease Type 2 

Corrado I. Angelini, Annachiara Nascimbeni, Marina Fanin 

and Marco Sandri; Padova, Italy 

Few studies have investigated atrophy/autophagy gene 

expression in LGMDs and GSD type 2, to study the fate of 

misfolded proteins in ubiquitin-proteasome system and recovery 

in autophagy-lysosome pathway after Enzyme 

Repacement Therapy (ERT). 

We investigated gene expression from 40 muscle biopsies 

of LGMD 2A, 20 LGMD 2B and 12 GSD type 2, both infantile 

and late onset. We found evidence of activation of 

molecular pathways with upregulation of autophagy-related 



genes: BNIP3 and p62 in Pompe, of atrophy-related genes 

atrogin-1 and MuRF-1 in LGMD 2A (calpainopathy) and 

in LGMD 2B (dysferlinopathy). In one infantile, one juvenile 

and one adult GSD type 2 cases a second biopsy was 

done after ERT and analysed for morphology and gene 

markers: in the second biopsy we observed after ERT 

reduced vacuolated fibers and decreased autophagy related 

markers. 

Our study in GSD type 2 recognises an important contribution 

of autophagic process in the development of muscle 

pathology, after ERT autophagy markers are considerably 

decreased as well as vacuolisation of muscle fibers. Our 

observations in LGMDs are new and support future therapeutic 

interventions to rescue muscle atrophy. 

Study supported by: Telethon, Eurobobank. 

M839. Virtual Demyelination in pmp22 Deficiency 




Nashville, TN 

Safety factor for action potential propagation in pmp22þ/ 

nerves appears impaired (Bai et al, J Neurosci 2010). The 

present study investigates mechanisms responsible for the 

impairment. Fluorescent dyes with different molecular sizes 

were injected into sciatic nerves. After 4-hour incubation, 

sciatic nerves were teased into individual nerve fibers, and 

examined under fluorescence microscopy. Fluorescence was 

of strong intensity in about a half of paranodal tomacula of 

pmp22þ/ nerves (15 mice), but absent or minimal in the 

paranodes of wild-type nerves (11 mice). This finding suggests 

that myelin is abnormaly leaky, and may result in excessive 

outward current. Application of potassium channel 

blocker, 4AP, to reduce outward current improved the amplitude 

of motor response during nerve stimulation. Western 

blot and immunohistochemistry revealed alterations of tight 

junction protein assembly, a potential molecular mechanism 

for the myelin leakage. 

Conclusions: Our results show excessive leakage in 

pmp22þ/ myelin in the absence of demyelination. This 

leakage is functionally similar to demyelination. These findings 

not only reveal novel mechanism for conduction block 

but also establish new therapeutic approach for this disease. 


M840. First in Human Phase 1 Trial of Neural 

Progenitor Cells in ALS: Results in the First 12 Patients 

Jonathan D. Glass, Nicholas Boulis, Meraida Polak, Crystal 

Kelly, Thais Federici, Jane Bordeau, Seward Rutkove, Karl 

Johe, Tom Hazel and Eva Feldman; Atlanta, GA; Boston, 

MA; Rockville, MD and Ann Arbor, MI 

Twelve patients with ALS were injected into the lumbar 

spinal cord with fetal-derived neural stem cells. The design 

is one of ‘‘escalating risk’’, where each group of patients is 

progressively less impaired. The first 6 patients were nonambulatory, 

3 were supported by mechanical ventilation. 

The first 3 patients received 5 unilateral injections at L2- 

L4, and the next 3 received 5 injections bilaterally. Patients 

7–12 were ambulatory and had vital capacities > 60 % 

predicted. Patients 7–9 received 5 unilateral injections, and 

patients 10–12 received bilateral injections. Each injection 

was 10 ll of 10,000 cells/ll; patients received either 

500,000 or 1 million cells through 5 or 10 injections, 

respectively. There have been two deaths, one which was 

unrelated to either ALS or the clinical trial. The surgical 

procedure was well tolerated. There have been no adverse 

105 

events attributable to the cellular injections. We are using 

clinical evaluation, strength testing, and electrical impedence 

myography to monitor progression of disease. Following 

FDA review and approval of safety data from the 

first 12 patients we will move to injections into the cervical 

spinal cord. 

Study supported by: Neuralstem, Inc. 

M841. Clinical Development of an Antisense Therapy 

for the Treatment of Transthyretin Amyloidosis 


Alvarado, Andrew Siwkowski, Merrill Benson, Steve Hughes 

and Brett Monia; Carlsbad, CA and Indianapolis, IN 

Transthyretin (TTR)-associated amyloidosis is a late-onset 

autosomal-dominant genetic disease. Mutations in TTR 

destabilize TTR tetramer thereby inducing the formation of 

amyloid fibrils. This disease mainly affects peripheral nerves 

as in familial amyloidotic polyneuropathy (FAP) or heart as 

in familial amyloid cardiomyopathy (FAC). Circulating 

TTR is predominantly produced by the liver, and the only 

available treatment as of June 2011 is orthotopic liver transplantation. 

Using antisense technology, we identified an 

antisense oligonucleotide, ISIS-TTRRx, for the treatment of 

TTR-associated amyloidosis. When tested in a human TTR 

transgenic mouse model, ISIS-TTRRx showed a dose-dependent 

reduction of human TTR at both the mRNA and protein 

levels. In cynomolgus monkeys, ISIS-TTRRx treatment produced 

a time-dependent reduction in plasma TTR levels. After 

12 weeks of treatment, liver TTR mRNA and plasma 

TTR protein levels were reduced by 80%. We also observed 

a significant decrease in plasma RBP4 levels correlating with 

TTR reduction. ISIS-TTRRx treatment was well tolerated in 

both rodents and monkeys and produced a PK/PD profile 

consistent with prior experience using this chemistry platform. 

ISIS-TTRRx is currently under evaluation in a Phase I 

clinical trial, with the first cohort dosed in May 2011. 

Study supported by: Isis Pharmaceuticals 


Alvarado, Steve Hughes and Brett Monia are all employees 

at Isis Pharmaceuticals 

Neurogenetics 

M1011. EPI-A0001: New Potential Therapy for 

Friedreich Ataxia 

David R. Lynch, Steve M. Willi, Robert B. Wilson, Karlla W. 

Brigatti, Olena Kucheruck, Eric C. Deutsch, William D. 

Shrader, Patrice Rioux, Guy Miller, Amale Hawi and Thomas 

Sciascia; Philadelphia, PA; Patterson, NY and Mountain View, 

CA 

This study tested the ability of EPI-A0001(previously called 

A0001) (a-tocopherol quinone), to improve in vitro measures, 

glucose metabolism and neurological function in Friedreich 

ataxia. We used an in vitro assay of cell rescue followed 

by a double blind, placebo controlled trial of 2 doses 

of EPI-A0001 in 30 adults. The primary clinical trial outcome 

was disposition index, a measure of diabetic tendency, 

from an IVGTT, evaluated 4 weeks after treatment. Secondary 

neurologic measures included the Friedreich ataxia rating 

scale (FARS). 

EPI-A0001 inhibited cell death in FRDA models in vitro. 

For the clinical trial, mean GAA repeat length was 699, and 

mean age was 31. Four weeks after treatment initiation, 

changes in disposition index between subjects treated with 

EPI-A0001 and placebo were not statistically significant. In 

contrast, a dose dependent improvement in FARS score was 



found. Patients on placebo improved 2.0 FARS points while 

patients on low dose EPI-A0001 improved by 4.9 points (p 

¼ 0.04) and patients on high dose improved by 6.1 points 

(p















T1538. Proneurogenic Compound Reduces Synaptic 

Ab-42 Oligomer Levels and Shows Cognitive Benefit 

in Alzheimer’s Mouse Model 

Sam Gandy, John W. Steele, Charles Glabe, Kai Treuner, Todd 

Albert, Carrolee Barlow, Michelle E. Ehrlich and Soong Ho 

Kim; New York, NY; Irvine, CA and San Diego, CA 

Group II metabotropic glutamate receptors (Gp II mGluR) 

trigger production of Ab peptides from isolated synaptic terminals, 

and synaptic Ab42 generation is selectively suppressed 

by Gp II mGluR antagonists. Gp II mGluR antagonists 

also stimulate hippocampal neurogenesis and enhance 

cognition. Pilot studies were performed in old (18 mo) and 

young (6 mo) Dutch APP693Q x PS1D Exon 9 bigenic 

mice. After 3 wk oral BCI-838 treatment of 18 mo-old 

mice, levels of prefibrillar A11-immunoreactive Ab oligomers 

were decreased in hippocampi from treated mice (p 

0.01). In young Dutch APP693Q x PS1D Exon 9 mice, 

fibrillar OC-immunoreactive Ab oligomer levels were 

decreased in the treated young mice (p 0.01). Some 

Dutch APP693Q and wildtype littermates were given BCI- 

838 p.o. for 3 mo. BCI-838 treatment was associated with 

improved memory in cued fear conditioning (p ¼ 0.01 

TgþVeh vs TgþBCI-838). A trend toward reduced anxiety 

was also observed (p ¼ 0.086 for TgþVeh vs TgþBCI- 

838). GpII mGluR antagonists are promising compounds 

for prevention or treatment of AD because of their unique 

synaptic Ab42- and Ab-oligomer-lowering activity coexisting 

with pro-cognitive and pro-neurogenic activities. 

Study supported by: P01 AG10491 

Carrolee Barlow is CSO at BrainCells. Todd Albert and 

Kai Treuner are staff scientists at BrainCells. 

T1539. Alzheimer Risk Variant Clusterin (CLU) and 

Brain Function during Aging 

Madhav Thambisetty, Lori Beason-Held, Michael Kraut, 

Michael Nalls, Andrew Singleton, Luigi Ferrucci, Simon 

Lovestone and Susan Resnick; Baltimore and London, United 

Kingdom 

Recent genome wide association studies have identified common 

risk variants for Alzheimer’s disease (AD), although 

these exert small effects and are, by themselves, unlikely to be 

of clinical utility in risk prediction. However, these genetic 

risk variants may reveal mechanisms involved in the transition 

from normal aging to cognitive impairment. We examined 

the effect of the recently discovered AD risk variant 

rs11136000 single nucleotide polymorphism in the clusterin 

gene (CLU) on longitudinal changes in resting state regional 

cerebral blood flow (rCBF) during normal aging. 15O-water 

107 

positron emission tomography measurements of rCBF were 

available at baseline and up to 7 annual follow-up visits in 88 

cognitively normal older individuals in whom genome-wide 

genotyping data were collected. Cognitively normal carriers 

of the risk allele of the rs11136000 SNP in CLU show significant 

and dose-dependent longitudinal increases in resting 

state rCBF in brain regions intrinsic to memory processes. 

We propose that these increments in rCBF over time represent 

compensatory changes in neuronal activity required to 

maintain normal cognition during aging in at-risk individuals, 

and that their eventual failure in some may mark the 

transition from normal aging to Alzheimer’s disease. 

Study supported by: NIA Intramural Research Program 

T1540. Association of High Density Lipoprotein to 

Alzheimer Disease 

Ramin Ebrahimi, Naser Ahmahi, Fereshteh Hajsadeghi and 

Hormoz Babaei; LA, CA 

Background: Alzheimer’s disease (AD) is a devastating progressive 

neurologic disorder with increasing prevalence. Little 

data exists regarding association of HDL-c to AD. This 

study evaluates the association of different HDL-c levels 

with AD. 

Methods: This study consists of 2144 Veterans with AD 

and 16175 without AD. VA electronic medical records from 

veterans were used to evaluate the presence or absence of 

AD, HDL levels and risk factors. HDL-c was classified as 

HDL-c 50 mg/dl. Conditional 

logistic regression analysis was employed to assess the 

association of lower levels of HDL-c with AD. 

Results: The mean age was 7966 years. After adjustment 

for risk factors, odds ratio of AD was 2.20 (95% CI 2.09– 

2.31, p ¼ 0.0001) for those with HDL-c of 40–50 mg/dl and 

2.89 (95% CI 2.75–3.05, p ¼ 0.0001) for those with HDL-c 

50 mg/dL. 

Conclusion: There is a strong association between lower 

levels of HDL-c and AD. Furthermore, population attribution 

risk of lower levels of HDL-c for new AD is very high; 

warranting future studies evaluating the role of specific lipid 

therapy to improve HDL-c levels to potentially decreased 

future development of AD. 

Study supported by: Self 

T1541. Amyloid-b 42:40 Metabolism Is Altered in 

Autosomal Dominant Alzheimer’s Disease (ADAD) 

Rachel E. Potter, Vitaliy Ovid, Tom Kasten, Wendy Sigurdson, 

Kwasi Mawuenyega, Bruce Patterson, Don Elbert, Scot Fague, 

Sumi Chakraverty, Alison Goate, Kevin Yarasheski, John C. 

Morris, Tammie Benzinger and Randall J. Bateman; St. Louis, 

MO 

We hypothesized that ADAD is caused by mutations that 

lead to higher production rates for amyloid-beta (Ab) 42 

compared to Ab40. We measured Ab isoform production 

and clearance rates in ADAD participants to determine the 

effects of mutations which invariably lead to AD. Presenilin 

1 mutation carriers (46.2yrs 6 15.8 (N ¼ 7)), and related 

non-carriers (52.0yrs 6 20.5 (N ¼ 7)) completed Ab stable 

isotope labeling 13 C6-Leu kinetic studies of cerebrospinal 

fluid, and PET PIB imaging. Differences in the Ab42 relative 

to Ab40 labeling curves were specifically altered in 

mutation carriers versus non-carriers. Newly generated Ab42 

was detected before Ab40 or 38, and there were differences 

in area under the curve analyses. These findings support 

prior in vitro studies that Ab42 is overproduced relative to 

other Ab isoforms. Ab isoform production and clearance 


may be used to define the therapeutic effectiveness of treatments 

which target abnormal Ab metabolism in AD. 

Study supported by: This work was supported by a grant 

from NIH (no. P01AG026276), an anonymous foundation, 

Eli Lilly research, the Knight Initiative for Alzheimer’s 

Research, and the James and Elizabeth McDonnell Fund for 

Alzheimer’s Research. R.J.B. is a cofounder of a company 

(C2N Diagnostics) that has licensed a pending Washington 

University patent on the technology described in this article. 

T1542. Acetylated Tau, a Novel Pathological Signature 

in Alzheimer’s Disease and Other Tauopathies 

David J. Irwin, Todd Cohen, Murray Grossman, Steven E. 

Arnold, Sharon X. Xie, Virginia M.Y. Lee and John Q. 

Trojanowski; Philadelphia, PA 

The microtubule-binding protein, tau, is the major component 

of neurofibrillary inclusions characteristic of Alzheimer’s 

disease (AD) and related tauopathies. Recently, we reported 

that the acetylation of tau at lysine residue 280 (ac-K280) is 

a novel, pathological post-translational modification. Here, 

we performed detailed immunohistochemistry (IHC) to further 

characterize tau ac-K280 expression in tauopathies. 

Ac-K280 tau IHC was conducted on 30 postmortem central 

nervous system (CNS) regions from AD (n ¼ 10), corticobasal 

degeneration (CBD; n ¼ 5), and progressive 

supranuclear palsy (PSP; n ¼ 5) patients. Ac-K280-immunoreactive 

(Ir) tau pathology was compared to multiple 

well-characterized tau epitopes. 

All cases showed significant CNS ac-K280-Ir tau pathology 

in a distribution pattern similar to hyperphosphorylated-tau. 

In AD cases, the majority of ac-K280-Ir tau pathology 

was in intracellular, thioflavin-S (ThS) positive tau 

inclusions, and also ThS negative tau pathology in CBD 

and PSP. Ac-K280-Ir was present throughout all stages of 

AD pathology, but more prominent in later stages. 

Ac-K280-Ir is a pathological modification of tau that 

may contribute to neurodegeneration by augmenting losses 

of normal tau properties, as well as toxic gains of function. 

Thus, inhibiting tau acetylation could be a disease modifying 

drug discovery target for tauopathies. 

Study supported by: NIH grants: T32-AG000255 Training 

in Age-Related Neurodegenerative Diseases and P30 

AG010124-20 Alzheimer’s Disease Core Center grant. 



T1623. Retrospective Analysis of Major Congenital 

Malformations (MCMs) and Oral Clefts (OC) Associated 

with In-Utero Topiramate Exposure 

Mark W. Green and Arun Bhattachuria; New York, NY and 

Yardley, PA 

Purpose: To analyze the prevalence of malformations 

among infants born to women exposed to topiramate. 

Methods: This study used retrospective data from Wolters-Kluwer 

Pharma Solutions that followed patients’ Pharmacy 

and Medical Claims (1/2003–12/2010) to identify 

infants exposed to topiramate during pregnancy (n ¼ 778). 

Probable exposure during pregnancy was refined using data 

on script fill date/supply, infant birth date, and ICD-9 

codes for birth term. 

Control cohorts included women with diagnosis of migraine 

without epilepsy (n ¼ 26,920) and a subset exposed 

to acute-preventive migraine drugs (APMD) during pregnancy 

(n ¼ 2,964). Topiramate was excluded from both 

groups. All cohorts excluded known or suspected teratogens. 

108 

Results: MCM rates were 4.11%, 4.02% and 4.15% for 

TPM, migraineurs, and APMD-treated migraineurs, respectively. 

Relative risks (95% CI) for TPM vs. migraineurs and 

APMD-treated migraineurs were 1.02 (0.73–1.45) and 0.99 

(0.68–1.45), respectively. OC rates were 0.26%, 0.17%, and 

0.37% for the TPM, migraineurs, and APMD-treated 

migraineurs, respectively. Relative risks (95% CI) for TPM 

vs. migraineurs and APMD-treated migraineurs were 1.50 

(0.37–6.19) and 0.69 (0.15–3.12), respectively. 

Conclusions: TPM exposure did not significantly 

increase the rates of OC or MCM. 

Study supported by: Vivus, Inc. 


T1741. Comparison of MRI Techniques for Monitoring 


Manuela Vaneckova, Jan Krasensky, Tomas Kalincik, Dana 

Horakova, Eva Havrdova and Zdenek Seidl; Prague, Czech 

Republic 

Goal: To compare contemporary MRI measures for prediction 

of future clinical disability in multiple sclerosis patients 

by analysis of our MRI data (brain atrophy, T2 lesion volume, 

T1 lesion volume and corpus callosum atrophy). 

Methods: Long term (7-year) longitudinal MRI data of 

178 patients were analyzed Area of corpus callosum, T2 

lesion volume, T1 lesion volume, brain parenchymal fraction 

and brain atrophy were measured. Clinical disability 

was assessed with Expanded Disability Status Scale (EDSS). 

Patients were divided in two groups: clinically stable and 

those with sustained progression during seven years. 

Results: Statistically significant correlation of future clinical 

disability (as characterized by EDSS score) with brain atrophy 

and corpus callosum atrophy was found. In this retrospective 

study, clinically stable patients were distinguished from 

patients with sustained progression in less than 1 year using 

the corpus callosum atrophy (probability over 98%) and after 

3 years using the brain atrophy (with probability of 96%). 

Conclusions: We have shown that the corpus callosum 

atrophy is a superior marker of disease progression compared 

to the brain atrophy, which was considered to be its 

most significant marker. 

Supported by Biogen Idec Inc. and the grants 

MZOVFN2005, MSM 0021620849. 

Study supported by: Supported by Biogen Idec Inc. and 

the grants MZOVFN2005 and MSM 0021620849. 

T1742. Effects of Rituximab on T-Cells in MS 

Uma Vinayagasundaram, Ellen M. Mowry, Ian R. Matthews, 

Julia Marino and Emmanuelle Waubant; San Francisco, CA 

Objective: To examine rituximab effect on T-cell subsets. 

Background: Rituximab, an anti-CD20 antibody, profoundly 

depletes circulating B-cells. It is unclear how it 

impacts T-cells. Methods: In a retrospective study of 

patients who received >1 rituximab infusions at UCSF MS 

center, within-patient pre- and post-infusion (mean duration 

¼ 7 months) lymphocyte counts for the first rituximab 

course were compared. Results: Of 119 patients who 

received rituximab, 56 (45 MS, 10 NMO, 1 other indication) 

had both pre- and post-infusion labs. The mean (þ 

SD) absolute count of CD19þ B-cells dropped from 273 

(þ 25) pre- vs. 59 (þ17) post-rituximab (p


count below 500, and 4 (6%) had a CD4 count below 200. 

NK cell number (CD56þ, CD16-) tended to increase postinfusion 

(182 þ 13 vs. 199 þ 14; p ¼ 0.2). Impact of retreatment 

is being analyzed. Conclusions: Rituximab induces 

long-term T-cell reduction, whose impact is unclear. 

Study supported by: Research grant from Roche 

T1743. Fast Macromolecular Proton Fraction (MPF) 

Mapping in Multiple Sclerosis (MS) 

Vasily L. Yarnykh, James D. Bowen, Bart P. Keogh, Pavle 

Repovic, Angeli Mayadev, Beena Gangadharan, Daniel S. 

Rizzuto, Hunter R. Underhill, Kenneth R. Maravilla and Lily 

K. Jung-Henson; Seattle, WA 

MPF mapping is a new quantitative imaging method, which 

determines relative concentration of macromolecular protons 

involved into the magnetization transfer effect. Literature suggests 

strong correlations between MPF and myelin content in 

neural tissues. We recently developed a fast 3D MPF mapping 

technique for clinical applications, which utilizes two images 

(with and without off-resonance saturation), and complimentary 

T1, B0, andB1maps, providing whole-brain coverage, 

1.5 1.5 4mm 3 resolution, and


immunopathology. Therefore, iTregs could play contrasting 

roles depending upon the stage of virus-mediated demyelinating 

diseases. 

Study supported by: National Center for Research 

Resources of the National Institutes of Health. 

T1747 Idiopathic Relapsing Conus Myelitis 

Raghav Govindarajan and Efrain Salgado; Weston, FL 

Background: Idiopathic acute transverse myelitis (TM) 

involves the thoracic cord. In rare cases conus might be the 

only site involved. Bladder symptoms can be a presenting 

feature without an obvious sensory level. Imaging studies 

might remain inconclusive thus posing a diagnostic challenge. 

Relapses in TM are rare and even more so restricted 

to the conus. We report a case of a 42 year-old female who 

presented with urinary retention (UR) that progressed to a 

flaccid paraplegia (FP). Imaging studies were negative ini- 

110 

tially with repeat imaging 5 days from symptom onset 

revealing a conus myelitis (CM). 

Case report: A 42-year-old female woke up with UR, 

which progressed to an ascending FP without a sensory 

level. MRI of spinal axis w/wo was normal. Lumbar 

puncture revealed 0 WBC, 1 RBC, normal protein, 

glucose and negative immune markers (IgG Index and 

oligoclonal bands). EMG was normal. MRI of the lumbar 

spine repeated 5 days later revealed CM. Extensive 

infectious, inflammatory and autoimmune work up 

including NMO was negative. Brain MRI w/wo was 

normal as were VEPs. She had a relapse 3 months after 

the initial bout. 

Conclusion: Idiopathic CM appears to be a rare clinical 

entity, which can have protean manifestations requiring a 

high index of clinical suspicion and a thorough systematic 

workup. 

Study supported by: None 




September 25, 2011 Career 

Development Poster Session 

Abstracts 




CD515. Non-Convulsive Status Epilepticus Is Associated 

with Mortality and Worse Short-Term Outcome in 

Critically Ill Children 

Nicholas S. Abend, Alexis A. Topjian and Dennis J. Dlugos; 


Objective: Determine whether non-convulsive seizures 

(NCS) and non-convulsive status epilepticus (NCSE) were 

associated with higher mortality and worse short-term 

outcome. 

Methods: Critically ill children with acute encephalopathy 

underwent EEG monitoring. EEGs scored as no seizures, 

NCS, or NCSE. Co-variates included age, etiology of 

acute encephalopathy, and EEG background. Outcomes 

were mortality and change in Glasgow Outcome Scale 

(GOS) from admission to ICU discharge. Chi-squared analysis 

and multi-variable logistic regression were used to evaluate 

for associations. 

Results: 200 children underwent cEEG. NCS occurred 

in 84 (42%) of which 43 (22%) had NCSE. 83 (41%) had 

a decrease in GOS of which 36 (18%) died. In multi-variate 

analysis NCSE was associated with mortality (OR 3.05, 

p¼0.04) and change in GOS (OR 15.5, P


Aasly, J. S204, S209 

Aasly, Jan T1503 

Abbott, Robert D. S203 

Abolfazlee, R. S114, T1732 

Achim, Critian L. T1905 

Adams, Patrick E. T1521 

Agadi, Jagadish B. M822 

Agbemenyah, Hope Yao M1004 

Aggarwal, Swati M821 

Ahlskog, J.E. M606 

Ahmed, Aiesha M833 

Akbar, Umer M1205, T1620, 

T1809 

Akber, Umer M719 

Akfirat, Gokhan T1536 

Akiyama, Hisanao S120 

Aksan, Nazan M615 

Al Samara, Mershed M722 

Alaedini, Armin M602 

Alam, Nurul S123 

Alcalay, Roy N. S225 

Aldea, Patricia T1901 

Alexander, Katie M821 

Alexopoulos, Andreas V. M711 

Alexopoulos, Harry T1729 

Alfahad, Tariq S127 

Alfano, Lindsay N. M802 

Alfuth, Kathleen S. M832 

Algom, Avi M1007 

Alkawadri, Mhd Rafeed M711 

Allen, Mariet T1503 

Allred, Peggy M821 

Alqadi, Khalid S. S127 

Altafullah, Irfan S102 

Althaus, Alison L. M1401 

Amini Harandi, Ali S114, T1712, 

T1718, T1722, T1723, 

T1731, T1732, T1734 

Ances, Beau T1901 

Andersen, O. S204, S209 

Anderson, C. Alan M616 

Andres, Patricia M821 

Andress-Rothrock, Diane C. 

S113, T1602 

Andrews, Mark M1106 

Annex, Brian H. S115 

Ansari, Hossein S236 

Ansari, Morad T1503 

Appel, Shmuel M702 

Arain, Fazal M. M707 

Arcilla-Londono, Ximena M828 

Arciniegas, David B. M616 

Ard, M. Colin T1531 

ABSTRACT AUTHOR INDEX 

Arnoldi, Alessia M1002 

Artibee, Kay M810 

Asadollahi, Marjan T1732 

Ascherio, Alberto S216 

Askanas, Valerie M807, M808, 

M816, M835 

Assadi, Mitra S223 

Astafiev, Serguei V. M601 

Atassi, Farrah S215 

Atkinson, Elizabeth J. M1008 

Au, Rhoda T1504 

Auchus, Alexander P. M1301 

Awkar, Nelly M717 

Azimi, Amirreza T1722 

Babikian, Viken L. S122 

Babovic, Mihajlo T1727 

Backus, Carey M804 

Bagai, Kanika S303 

Baiser, Alexa S. T1504 

Baker, M. S204, S209 

Bakken, Erik C. M618, M619 

Bakst, Isaac M821 

Balasubramanian, Archana B. 

T1524 

Balice-Gordon, Rita 

T1701, T1716 

Baloh, Bob M815 

Baloyannis, Stavros J. T1528 

Bammler, Theodor K. T1511 

Banerjee, Chirantan S101 

Bankiewicz, Krystopf S. T1529 

Barbato, Luigi M. T1735 

Barcikowska, Maria T1503 

Bardin, Jonathan C. M1104 

Barkhof, Frederik T1708 

Barnwell, Stanley L. T1606 

Bartz, Traci T1513 

Bass, Dale M1107 

Bassi, Mariateresa M1002 

Bassile, Clare M1403 

Basta, Milan S103 

Bateman, Lisa M. M703 

Bateman, Randall J. T1501 

Bayat, Elham T1739 

Bayram, Mehmed B. T1527 

Beal, Flint S216 

Beck, James S216 

Beckmann, Karola T1702 

Beh, Shin C. S210 

Belichenko, Pavel V. M609, 

M712 

Bellugi, Ursula M608 

Benke, Tim A. M706 

112 

Ben-Menachem, Elinor M713 

Bennett, C.F. M821 

Bennett, David A. T1502 

Bennett, Jeffrey L. T1736 

Benzinger, Tammie T1901 

Berlau, Daniel J. T1524 

Bernard, Bryan S211 

Bernard, Paul B. M706 

Beyer, Richard P. T1511 

Bhabad, Sudeep S119 

Bhargava, Pavan S140 

Bhat, Sushanth M718, M834, 

S134, S137 

Bhattacharya, Pratik S129 

Bieber, Allan M1101 

Biglan, Kevin S228 

Biglan, Kevin M. S402 

Bigwood, Doug T1736 

Bilal, Yasmin T1737 

Billings, Nia M. M613 

Biondo, Andrew M828 

Bisceglio, Gina T1503 

Bishop, Kathie M821 

Black, Robert E. S123 

Blair, Aaron S208 

Blakeman, Alan T1621 

Blasco, Maria R. T1713 

Blechschmidt, Cristiane M835 

Blennow, Kaj T1514 

Bliton, Mark S232 

Bliwise, Donald L. S301 

Blythe, Anne M819 

Bockenek, William L. M819 

Bodda, Chiranjeevi M1004 

Boden-Albala, Bernadette M1403 

Bodensteiner, John B. M1303 

Boes, Benjamin M1008 

Boeve, Bradley F. T1530 

Boffa, Michael S302 

Bonanni, Paolo M1002 

Bonato, Sara M1002 

Bondar, Galyna T1704 

Boon, Andrea M826 

Bordelon, Yvette S213 

Borkowski, Thomas M. T1615 

Borland, Scott T1617 

Bota, Daniela T1804 

Botuyan, Maria V. M1008 

Boutin, Paula T1719 

Boyd, Scott D. T1736 

Bravver, Elena M814, M819 

Brennan, K.C. T1601 

Bresolin, Nereo M1002 



Bressman, Susan B. S233 

Brewer, James B. T1514 

Bringas, J. T1529 

Britton, Jeffrey W. M1202, 

M1203, S403 

Broderick, D.F. S204 

Broderick, Joseph S112 

Brooks, Benjamin M821 

Brooks, Benjamin R. M814, 

M819 

Brooks-Kayal, Amy R. M705 

Brott, Thomas G. S102, S116 

Brown, Amanda S407 

Brown, O.W. S102 

Bruce, Nhu T. S131 

Bryant, Monthaporn S. S215 

Bucelli, Robert S406 

Buckley, Camilla T1711 

Buckley, Charlotte T1903, T1904 

Bumbaugh, Jon T1614 

Burakgazi, Evren T1620 

Burakgazi-Dalkili, Evren M1205 

Burge, Daniel J. T1724 

Burger, Kathleen S127 

Burke, James M1107 

Buse, Dawn C. T1608, T1610, 

T1611, T1612, T1613, 

T1615 

Bushara, Khalaf O. M602 

Busta, Angela S. M804 

Cacabelos, Ramon T1506 

Cacciottolo, Mafalda M807 

Cai, Haipeng M1301 

Caillier, Stacy T1713 

Callaghan, Brian M1005 

Camargo, Carlos A. S139 

Cambon, Alex C. S213 

Campbell, Christina M1204 

Campbell, Harry T1503 

Campos-Rivera, Juanita T1719 

Can, Karolina M1004 

Cannigaiper Uthamaroyan, 

Velmurugendran S121 

Cao, Chuanhai S202 

Capehart, Bruce P. M1107 

Caplan, Louis S136 

Carlson, H. T1522 

Carlson, Noel G. T1801 

Carone, Marco S123 

Carran, Melissa T1809 

Carrasquillo, Minerva M. T1503 

Carter, Alex R. M601 

Casadesus, Gemma M620 

Casanova, Vanessa M1002 

Case, Amanda M1001 

Castano, Anna M706 

Catellier, Diane J. T1505 

Cava, Luis S113 

Cen, Steven S221 

Cervantes, Anna M. S122 

Chai, High Seng T1503 

Chana, Gursharan T1905 

Chandrakumar, 

Manokaraananthan T1621 

Chandrasekaran, Krish M801 

Chang, Bernard S. M704 

Charles, David S224, S232 

Charles, P. David S226 

Chaturvedi, Seemant S129 

Chemali, Zeina S214 

Chen, Chueh-Tan T1537 

Chen, Helen M801 

Chen, Honglei S208, S234 

Chen, Jian M1301 

Chen, Roujie M1403 

Chen, Shun-Sheng T1537 

Chen, Xinzhi S103 

Chen, Yi T1715 

Cheng, Chu M1402 

Cheng, Hsinlin T. T1604 

Cheng, Jiin-Tsuey T1537 

Cheng, Ping M607 

Cheng, Yuching S107 

Cheong, Hae Kwan T1519 

Chernoff, Jonathan M803 

Cheung, Ying K. M1403 

Chibnik, Lori B. T1502 

Chipendo, Portia I. T1502 

Chisulo, Brian T1908 

Chitnis, Shilpa S210 

Chiu, David S116 

Cho, Chi T1740 

Choi, Jae-Hwan S126 

Choi, Kwang-Dong S126 

Choi, Soon Gang M824 

Chokroverty, Sudhansu M718 

Choudary, Zahid S130 

Choudary, Zahid I. S135 

Choudhary, Shazia Z. S130, 

S135 

Chretien, Nathalie T1721 

Christensen, Jon T1901 

Christie, Kimberly J. M1402 

Christine, C.W. S204, S209 

Christodoulou, Joanna A. 

M704 

Christopher, Hanlon T. T1602 

Chuang, Yu-Ming S106 

Chun, Jerold T1710 

Chung, Amy S. T1802 

Churchwell, Mona I. T1511 

Ciric, Bogojub T1725 

113 

Clark, Chris M. T1508 

Clark, K. Reed M802 

Clawson, Susan A. T1801 

Clifford, David T1901 

Clifton, Guy L. M1103 

Cohen, Jeffery A. T1710 

Cohen, Jeffrey T1708 

Cohen, Jeffrey A. T1709 

Coker, Laura H. T1505 

Cole, John W. S107 

Collins, William T1709 

Comi, Giacomo M1002 

Comi, Giancarlo T1708 

Conte, Mary M. M1104 

Contractor, Anis T1701 

Cook, Jason A. S224 

Corbetta, Maurizio M601 

Coresh, Josef T1505 

Corey-Bloom, Jody S205, S220, 

T1507 

Corrada, Maria M. T1508, 

T1524 

Corrigan, John M828 

Cote, Lucien S225 

Couto, Joseph T1614 

Cowell, Lindsay G. T1736 

Crimella, Claudia M1002 

Crook, Julia T1503 

Crowder, Kermit S109 

Cruz Del Angel, Yasmin M705 

Cruz-Flores, Salvador T1513 

Cudkowicz, Merit M821 

Cuevas, Ivan T1506 

Cunningham, 

Christopher R. S213 

Cunningham, Julie M. M1008 

Cutter, Gary T1702 

D’Agostino, Carla M807, M808 

Dakka, Youssef A. M828 

Dalakas, Marinos C. T1729 

Dale, Anders M. T1525 

Dalmau, Josep T1701, T1716 

D’Angelo, Grazia M1002 

Das, Moromi S104 

Dauch, Jacqueline R. T1604 

David, William M821 

Davis, Bonnie S109 

Davis, Thomas L. S232 

D’Cruz, O’Neill M714 

De Jager, Philip T1720 

De Jager, Philip L. T1502 

DeArmond, S. S204, S209 

DeCarli, Charles T1504 

Decker, David A. S130, S135 

Deeley, Cheryl S228 

DeFreitas, Tiffani T1615 



DeFries, Ashleigh T1534 

Dekermenjian, Rony S134, S137 

del Pilar Cortes 

Nino, Maria T1906 

Del Tufo, Stephanie N. M704 

Delaney, Colin E. M1005 

Delgado, Monica T1705 

Demaerschalk, 

Bart M. S102, S116 

Denic, Aleksandar T1703 

DeOrchis, Vincent S. T1518 

Derani, Lena T1739 

Desai, Urvi G. M819 

Dham, Bhavpreet S223, T1620, 

T1809 

Dhamija, Radhika S403 

Diaz-Medina, Gloria E. S403 

Dickson, D.W. S204, S209 

Dickson, Dennis W. M1007, 

S207, T1503, 

T1515, T1530 

DiClemente, Guillermo T1534 

Diep, Lien S109 

Dilley, Deanne M714 

DiVito, Brittany M. M618, M619 

Dodge, Hiroko T1532 

Dodick, David T1617 

Doerge, Daniel R. T1511 

Dominantly Inherited 

Alzheimer Network T1501 

Dorsey, E. Ray S402 

Dosado, Lea M830 

Doty, Pamela M713, M714 

Dowling, James J. M804 

Du, Guangwei S206, S212, S234 

Du, Senxi T1804 

Du, Sienmi T1704 

Duane, Drake D. M618, M619 

Dubinsky, Richard S213 

Dueker, Nicole S107 

Duncan, G. T1522 

Duquette, Pierre T1713 

Durand, Dominique M620 

Dustin, Irene M702 

Dyck, P. James M1008 

Dyck, Peter J. M1008 

Eastman, Alison J. M1001 

Eastman, Eric T1736 

Eberly, Shirley S216 

Ebers, George T1702 

Edland, Steven D. M1010, 

S205, T1531 

Edlow, Jonathan A. S139 

Edson, Jean S109 

Edythe, Wiggs A. M820 

Eizenamn, Moshe M607 

Elble, Rodger J. S140 

Eleopra, Roberto S237 

El-Hagrassy, Mirret T1536 

Elkind, Mitchell S.V. S101 

Ellik, Shehanaz M828 

Elliott, Robin S216 

Ellis, Ronald J. T1905 

Elsayed, Mohammad M613 

Engel, King W. M808, M816, 

M835 

Engel, W. King M807 

Ertekin-Taner, Nilufer T1503 

Erten-Lyons, Deniz T1532 

Eslinger, Paul J. S206, S212 

Etemadifar, Masoud T1732 

Evans, Denis A. T1502 

Evensen, Laura A. M1403 

Everall, Ian P. T1905 

Faezi, Sholeh T1731 

Fagan, Anne T1901 

Fallah, A. T1712, T1723 

Fang, Yin T1527 

Fanning, Kristina T1608 

Fanning, Kristina M. 

T1610, T1611 

Fantin, Marianna M1002 

Farber, Charles R. S115 

Farheen, Amtul M833, M834, 

S134, S137 

Farrer, Lindsay T1512 

Farrer, Matthew J. S207 

Fasih, Zoha S137 

Fatima, Tasneem S229 

Feany, Mel B. T1502 

Feldman, Eva L. M804, M1005, 

T1604 

Fennema-Notestine, 

Christine T1525 

Fernandez, Lucia T1506 

Ferrari, Michel T1617 

Fields, Jeremy D. T1606 

Figueroa-Romero, Claudia M1005 

Filley, Christopher M. M616 

Fincher, Linda S215 

Finder, Stuart G. S232 

Finlayson, Marcia T1740 

Fire, Andrew Z. T1736 

Fisher, Barbara C. M610, M614 

Fisher, Mark S108 

Fishman, Robert A. S124, T1806 

Fitzpatrick, Annette T1513 

Flagg, Emily S216 

Flanagan, Eoin P. M811, T1805 

Flanigan, Kevin M. M802 

Flemming, Kelly D. S128 

Forsayeth, John T1529 

114 

Forst, Amy T1616 

Foulds, Pamela T1726 

Fountain, Nathan B. M713, 

M714 

Fowkes, Mary E. M824 

Francis, Gordon T1709 

Frederick, Meredith C. M616 

Freeman, William D. M1204 

Friedland, Robert P. M1301 

Frohman, Elliot M. T1736 

Frost, Nicholas M723 

Fry, Rebecca C. T1511 

Fu, Beverly D. T1804 

Fugate, Jennifer E. S128, T1714 

Fuh, Jong-Ling T1605 

Fujioka, Shinsuke M1007, S207 

Fulda, Stephany M610, M614 

Furuya, Hirokazu S201 

Gabbard, Jennifer T1737 

Gabrieli, John D.E. M704 

Gadallah, Nancy S134 

Galasko, Douglas T1525 

Galasko, Douglas R. T1531 

Galbraith, Sareen T1903 

Galbraith, Sareen E. T1904 

Gallagher, Martin J. M707 

Gallin, Eliza S213 

Gamez, Jeffrey T1727 

Gamez, Jeffrey D. T1715 

Ganeshan, Veena R. T1803 

Garbern, J.Y. S204, S209 

Garcia, Paul S. S301 

Garden, Gwenn A. M1001 

Gardiner, Irenita S228 

Garges, Danielle M. M610, 

M614 

George, Benjamin P. S402 

Georgescu, Constantin T1503 

Gerhardt, Greg A. M709 

Ghaffarpour, Majid T1732 

Ghaffar-Pour, Majid S114 

Gharagozli, Kourosh T1731 

Ghareghozli, Kourosh T1718 

Ghargozlee, Kourosh T1732 

Ghosh, Partha S. M1302 

Ghoshal, Shivani S136 

Ghragozlee, Kourosh S114 

Gill, Chandler E. S224, S232 

Gluhm, Shea S205, S220, 

T1507 

Goadsby, Peter J. T1603, T1617 

Gobinathan, Devathasan M830 

Godfrey, Rena A. M820 

Goetz, Christopher G. S211, S231 

Gohar, Dina T1527 

Gokhale, Sankalp S136 



Goldfarb, Neil T1614 

Goldfine, Andrew M. M1104 

Goldman, Jennifer G. S211 

Goldstein, Jerome T1618 

Goldstein, Jody S205, S220, 

T1507 

Goltz, Herbert C. T1621 

Gomez, Megan S221 

Gomez, Yessenia M605 

Gonnella, Patricia T1725 

Gonzalez, Marco I. M705 

Goodin, Douglas S. T1702 

Goodman, B. S204, S209 

Gordon, Barry M613 

Gottdiener, John T1513 

Gottesman, Rebecca F. S125, 

S132, T1505, 

T1513 

Gourraud, Pierre-Antoine T1713 

Govindarajan, Raghav M822 

Grabenstatter, Heidi L. M705 

Grabstein, Kenneth T1724 

Graff-Radford, Jonathan M606, 

T1714 

Graff-Radford, Neil T1515 

Graff-Radford, Neill R. T1503 

Grafton, Scott T. M604 

Graham, Stephen M. T1516, 

T1517, T1523, 

T1526, T1533 

Green, Peter H. M602 

Greenberg, Benjamin M. T1736 

Greenfield, L. John S404 

Greenia, Dana E. T1508 

Greenlee, John E. T1801 

Greenspan, Ralph J. M1006 

Grewal, Raji M833 

Griffiths, Michael T1904 

Griffiths, Mike T1903 

Griggs, Robert C. M831 

Grinspan, Augusto T1707, 

T1708 

Groden, Catherine A. M820 

Grossardt, Brandon R. T1703 

Gu, Yian S225 

Gualdi, Sabrina S237 

Guo, Fuzheng T1705 

Guo, Jiasong M803, M813 

Guo, Xuguang S208 

Gupta, Divya M718 

Gupta, Varun M816 

Gutmann, Laurie S236 

Gutmann, Ludwig S236 

Guzik, Amy K. S115 

Habermann, Thomas M. T1805 

Hachinski, Vladimir T1510 

Hafler, David T1720 

Hagerman, Paul J. T1521 

Hagerman, Randi J. T1521 

Hagler, Jr, Donald J. T1525 

Haines, Jonathan T1512 

Haleem, Darakhshan Jabeen S229 

Hall, Charles T1615 

Hall, Deborah A. S213 

Hall, Ira M. S115 

Hallett, Mark M602 

Halvorsen, Mark B. M710 

Hamby, Mary T1704 

Hammans, Simon M1008 

Hanna, Michael M831 

Happ, Erik T1806 

Hara, Hajime M829 

Harbour, Leia S109 

Hardy, Duriel M1401 

Häring, Dieter A. T1707 

Harman, Jennifer S216 

Haro SIlva, Rubén M617 

Harper, Charles M. M811 

Harris, Tamara B. T1504 

Hart, Ian J. T1903, T1904 

Hartung, Hans-Peters T1708 

Hasegawa, Yasuhiro S120 

Hassan, Anhar M826 

Hastie, Nicholas D. T1503 

Hatemian, A. S114 

Haut, Sheryl R. T1615 

Hayward, Caroline T1503 

Heck, Donald V. S102 

Heiss, Wolf-Dieter T1510 

Heister, David S. T1514 

Hemmen, Thomas M. S105 

Henchcliffe, Claire S213 

Hendrix, Suzanne T1516, 

T1517, T1523, 

T1526 

Heppner, Frank L. M835 

Hernandez, Leticia S230 

Herr, Barbara M831 

Heshmat, Ramin T1722 

Hill, Kenneth E. T1801 

Hillis, Argye E. M605, M612, 

S125, S132 

Hinson, Vanessa S219 

Hinton, Sabrina S129 

Hirano, Makito T1520 

HNRC Group T1905 

Hocker, Sara M1202, M1203 

Hogan, Edward M708 

Hohjoh, Hirohiko S201 

Hohlfeld, Reinhard T1707 

Holland, Dominic T1525 

Hollenbeck, Albert S208 

115 

Holtzman, David T1901 

Hong, Yu M1005 

Hopkins, Mark T1903, T1904 

Hopkins, Mary T1614 

Hornung, Richard S112 

Hou, Jing T1527 

Hou, Jyhgong Gabriel S215 

Howard, George S102, S116 

Howard, Katherine L. M616 

Hsieh, Yu-Hsiang S139 

Huang, Juebin M1301 

Huang, Xuemei S206, S208, 

S212, S234 

Huentelman, Matthew A. T1502 

Huh, Young Eun S138 

Hui, Jennifer S. S221 

Hur, Junguk M1005 

Husain, Samea S218 

Husan, Shema S229 

Hussain, Tiki S222, S227, S235 

Hye, Robert J. S102 

Ikeda, Ken M812 

Ikuno, Yasushi T1520 

Irani, Sarosh T1711 

Ishihara, Ryu T1520 

Isobe, Noriko M1009, T1706 

Isojarvi, Jouko M713 

Itoh, Noriko T1704 

Iwahashi, Hiromi T1520 

Iwasaki, Yasuo M812 

Iwashita, Julie T1616 

Iyadurai, Stanley M720, T1607 

Iyadurai, Stanley J. M815 

Jack, Clifford T1503 

Jack, Clifford R. T1530 

Jack, Jr., Clifford R. M606, 

T1505 

Jackson, Dan M1204 

Jackson, Leila S213 

Jacobson, Mark T1507 

Jacobson, Mercedes S401 

Jaffer, Zahara M. M803 

Jankovic, Joseph S213 

Järvinen-Pasley, Anna M608 

Javan, Alireza K. T1521 

Javedani, Parisa P. T1606 

Jayadev, Suman M1001 

Jayam-Trouth, Annapurni S109 

Jeffrey, Kaye T1532 

Jen, Jin T1503 

Jenkins, Andrew S301 

Jensen, Michael D. M606 

Jeon, Seun T1519 

Jianfeng, Cai S202 

Jiang, Shan H. T1908 

Jimi, Takahiro M829 



John, Sanjna M. T1906 

Johnson, Aaron J. T1715 

Jones, Timothy F. M823 

Josephs, Keith A. M606, M811, 

T1515, T1530 

Joshi, Abhinay D. T1508 

Jovic, Sofija T1534 

Ju, Y. Sungtaek T1601 

Juarez, Humberto S230 

Juncos, Jorge S213 

Kaida, Ken-ichi T1733 

Kalita, Jayantee M1003, S104 

Kalyanam, Janaki S109 

Kamakura, Keiko T1733 

Kanazawa, Ichiro S201 

Kang, Huicong S110 

Kano, Osamu M812 

Kaplan, Johanne T1719, T1721 

Kappos, Ludwig T1707, T1708, 

T1709 

Karageorgiou, Clementine E. 

T1729 

Karagogeos, Domna T1729 

Karanam, Deepthi M717 

Karimi, Mehdi S114 

Karin, Ernstrom S105 

Karpf, Adam R. M1008 

Karunapuzha, Cherian A. S210 

Kasarda, Donald D. M602 

Kase, Carlos S. S122 

Kashouty, Rabih M722, S133, 

T1808 

Kaspar, Brian M802 

Kassar, Darine M720, T1607 

Kass-Hout, Omar S117 

Kass-Hout, Tareq S117 

Katzir, Tami M704 

Kaubrys, Gintaras M713 

Kavanagh, Terrance J. T1511 

Kawabe, Kiyokazu M812 

Kawano, Yuji T1706 

Kawas, Claudia H. T1508, T1524 

Ke, Qing M831 

Keegan, B. Mark T1805 

Keenan, Brendan T. T1502 

Kellerman, Don T1616 

Kells, Adrian P. T1529 

Kelly, John T1620 

Kelly, Kevin M. M701 

Khan, Omar M702 

Khanna, Ashwani T1717 

Kharlamov, Elena A. M701 

Khatri, Bhupendra O. T1708 

Khitrov, Greg M824 

Kiernan, Matthew T1711 

Kifayathulla, Liakath Ali M1004 

Kim, Chi Hun T1519 

Kim, Jae Seung T1519 

Kim, Ji Soo S138 

Kim, Suk-Hui T1519 

Kim, Sung Tae T1519 

Kimonis, Virginia E. M805 

Kimura, Fumihiko T1733 

Kira, Jun-ichi M1009, T1706 

Kisby, Glen E. T1511 

Kissel, John M806 

Kittner, Steven J. S107 

Klein, Christopher J. M1008 

Kleschevnikov, Alexander M. 

M712 

Klingstedt, Therése M835 

Kluger, Benzi S213 

Knappertz, Volker T1702 

Knopman, David S. T1505, 

T1530 

Ko, Nerissa S112 

Koch, Heather A. M618, M619 

Koffmann, Boyd S404 

Kolbert, Christopher P. T1503 

Kompoliti, Katie S231 

Kong, Xiao-Tang T1804 

Konrad, Peter E. S232 

Korczyn, Amos D. T1535 

Kori, Shashidhar T1603, 

T1616, T1617 

Koubeissi, Mohamad Z. M620, 

M1301 

Koyfman, Feliks S122 

Krauss, Gregory M714 

Kremenchutzky, Marcelo T1702, 

T1707 

Kronk, Lisa M817 

Kronmal, Richard T1513 

Kumar, Bishwanath M1003, S104 

Kumar, Sunil M1003 

Kunschner, Lara T1806 

Kunyu, Li S202 

Kupsky, W. S209 

Kurantsin-Mills, Joseph S109 

Kurlan, Roger S216 

Kurtzke, John F. T1730 

Kusunoki, Susumu M818, 

T1520, T1733 

Kuwabara, Motoi T1733 

Kuwahara, Motoi M818 

Kwagyan, John S109 

Kwan, Justin Y. M820 

Lai, Eugene C. S215 

Laidlaw, David H. M1301 

LaManna, Joseph M620 

Lambrecht, Lawrence J. M710 

LaMorte, Michael T1721 

116 

Lancaster, Eric T1701 

Lander, Cecilie M1008 

Lang, Anthony E. S216 

Lange, Dale J. M824 

Langford, Velma L. M814, M819 

Larery, Angela T1522 

Lasarev, Michael R. T1511 

Lash, J. S204, S209 

Latov, Norman M602 

Lau, Helena S122 

Lazar, Ronald M. M1403 

Leblang, Spencer A. M618, M619 

Ledoux, Kerry M613 

Lee, Christopher D. M823 

Lee, Jae-Hong T1519 

Lee, John D. M615 

Lee, Jong-Min T1519 

Lee, Joseph T1512 

Lee, Michael M703 

Lee, Seung-hun S118 

Lee, Szexian T1802 

Leep Hunderford, Andrea M826 

Lees, Monica M615 

Lees, Peter S213 

Lehky, Tanya J. M820 

Leimgruber, Pierre P. S116 

Leone, Paola S223 

LeRoy, Robert F. M714 

Lessig, Stephanie S205, T1507 

Leverenz, James S213 

Levesque, Jessica T1807 

Levitt, Jacob M831 

Lewis, Mechelle M. S206, S212, 

S234 

Li, Chin-Shang M703 

Li, Jun M803, M810, M813 

Li, Sean S. S115 

Li, Wang S202 

Lieberman, Abraham S222, S227, 

S235 

Lieberman, Abraham N. S218 

Ligocki, Ann J. T1736 

Lin, Wen-Lang T1515 

Lin, Xiaoyang S202 

Lincoln, Sarah T1503 

Lindblom, Scott C. M814, M819 

Linskey, Mark E. T1804 

Lipton, Richard T1617 

Lipton, Richard B. S233, T1608, 

T1610, T1611, T1612, 

T1613, T1615 

Lipton, Stuart A. T1509 

Lirng, Jiing-Feng T1605 

Litchy, William J. M1008 

Litvan, Irene S213 

Liu, Kenneth C. T1606 



Liu, Rui S208 

Liu, Shuo S108 

Liu, Xinyeu S107 

Liu, Yu M1401 

Llinas, Rafael H. S125, S132 

Lohrey, Anne K. T1715 

Lok, Edwin T1802 

Lombardi, Valter T1506 

Longbottom, Mary E. S116 

Longstreth, Jr., W.T. T1513 

Lowes, Linda P. M802 

Lu, Bo M701 

Luders, Hans M620 

Luke, Sothear M1204 

Luna, Jennie M717, M721, 

T1619 

Lunn, J. Simon M804 

Lunn, John S. M1005 

Luo, Benyan M831 

Luthra, Indermohan M816 

Ma, Joyce T1705 

Ma, Li T1503 

Maccotta, Luigi M708 

Macura, Slobodan T1703 

Macura, Slobodan I. T1727 

Mada, Flicia S129 

Madjid, Keyvani T1609 

Maeda, Yoshiko T1705 

Maganti, Sombabu M718 

Magda, Sebastian T1514 

Maghzi, Amir Hadi T1732 

Mahadevan, Anita M822 

Mahajan, Shalini M816 

Mahaparn, Irisa M609 

Maharjan, Sooraj T1503 

Mailman, Richard B. S234 

Malhotra, Konark S111 

Malhotra, Manoj T1735 

Malik, Tafheem S229 

Malikova, Irina T1713 

Malow, Beth A. S303 

Malphrus, Kimberly G. T1503 

Manack, Aubrey N. T1608, 

T1612 

Mandell, Daniel M1105 

Mandrekar, Jay M1202, M1203 

Manivanh, Richard T1506 

Mannan, Ashraf U. M1004 

Mano, Toshiyuki T1520 

Mantese, Vito A. S116 

Manus, Neil D. S224 

Marchidann, Adrian S133 

Marder, Karen S216 

Marder, Karen S. S225 

Marek, Kenneth S216 

Marras, Connie S213 

Marsh, Elisabeth B. S125, S132 

Marshall, Randolph S. M1403 

Marshall, Theresa S404 

Martens, William B. M806 

Martinez, Ashley M702 

Martinez, Jose A. M1402 

Martinez-Hernandez, Eugenia 

T1701, T1716 

Martinuzzi, Andrea M611, 

M1002, S237 

Masaki, Kamal H. S203 

Masaki, Katsuhisa M1009, T1706 

Masliah, Eliezer M609 

Mastorodemos, Vasileios M603 

Mateen, Farrah J. S123 

Matsushita, Takuya 

M1009, T1706 

Mattson, Mark P. S103 

Matveeva, Elena E. M709 

Matyushov, Alexei M1201 

Mayeux, Richard T1512 

McArthur, Justin C. S407 

Mcavoy, Mark P. M601 

McCague, Kevin T1735 

McCauley, Stephen R. M1103 

McCracken, Lindsey T1716 

McDermott, Michael P. M806 

McDonald, Jamie T1713 

McEvoy, Linda K. T1514, 

T1525 

McGarry, Megan M621, M717, 

T1738 

McShea, Cindy M713 

Mehia-Santana, Helen S225 

Mehlenbacher, Adam M1107 

Mehta, Bijal S117 

Meira, Lisiane B. T1511 

Mejia, Nicte S214 

Melman, Tamar T1802 

Mendell, Jerry R. M802 

Mendoza, Jesus F. T1907 

Meng, Cheryl S216 

Meng, Xiangyi T1707, T1708 

Meola, Giovanni M1002 

Mer, Georges M1008 

Meschia, James F. M1204, S102, 

S116 

Metcalf, Nick M601 

Meyer, Brett C. S131 

Meysamie, A. T1712 

Michael, Benedict D. T1903, 

T1904 

Middha, Sumit M1008, T1503 

Miglis, Mitchell G. S302 

Miller, Alistair T1903, T1904 

Miller, Eric R. M701 

117 

Miller, Michael L. T1516, T1517, 

T1523, T1526 

Miller, Timothy M. M821 

Millis, Scott S129 

Ming, Ming M831 

Mintun, Mark A. T1508 

Misra, Usha K. M1003, S104 

Mitchell, Amber N. T1807 

Mitchell, Braxton D. S107 

Mitchell, James F. S107 

Mittal, Balraj M1003, S104 

Mobley, William M712 

Moeller, Thomas M1001 

Mohammad, Yousef S119 

Mohammad, Yousef M. S111 

Mokin, Maxim S117 

Molina, Enrique S230 

Molinari, Anna L. S224, S226, 

S232 

Molteni, Silvia S237 

Mondani, Massimo S237 

Monson, Nancy L. T1736 

Moodley, Manikum M1302 

Moomaw, Charles S112 

Moon, In Soo S126 

Moon, Yeseon P. S101 

Moore, Ryan P. M609 

Moretti, Paolo M1103 

Morgan, Kevin T1503 

Morris, John T1901 

Morrison, Richard S. M1001 

Morrow, Leslie S301 

Moseley, Brian D. S403 

Moshirzadeh, Sasan T1718 

Mosley, Thomas H. T1505 

Mowry, Ellen M. T1713 

Mozaffar, Tahseen M805 

Mtchedlishvili, Zakaria M701 

Muldowney, James S303 

Multani, Manpreet M833 

Murata, Kiyoko M812 

Murata, Miho S201 

Musumeci, Olimpia M1002 

Myers, Iliza M810 

Na, Duk L. T1519 

Nafissi, Shahriar T1722 

Nagahara, Alan T1529 

Nagata, Riya M812 

Nair, Asha A. T1503 

Nakamura, Yusaku T1520 

Nanakul, Rawi T1521 

Nance, Matthew T1522 

Nath, Avindra S407 

Naylor, David E. M716 

Neafsey, Edward S405 

Nehrebecky, Michele E. M820 



Neil, William P. S105 

Neiman, Eli S. M718 

Newman-Toker, David E. S118, 

S139 

Ng, Rowena M608 

Ng-Mak, Daisy S. T1613, T1614 

Nguyen, Thanh N. S122 

Nguyen, Thuy T1503 

Nichols, Mindy S. M814, M819 

Nicholson, Garth A. M1008 

Nilsson, Peter K.R. M835 

Nogalska, Anna M807, M808, 

M835 

Nourian, A. S114, T1732 

Oakes, David S216, S228 

Oaklander, Anne Louise M825 

O’Connell, Jeffrey R. S107 

O’Connor, Paul T1707 

Ogawa, Go T1733 

Oger, Joel T1702 

Oh, Sang Su M804 

Oh, Seung Jun T1519 

Ohishi, Mitsuru T1520 

Olichney, John M. T1521 

O’Neill, Brian P. T1805 

O’Neill, Mifflin M819 

Onken, Mitch M801 

Opler, Mark T1534 

Oplinger, Heather M819 

Ottoboni, Linda T1720 

Ouyang, Bichun S119 

Owens, Michael J. S301 

Ozelius, Laurie J. S233 

Paccico, Thomas J. M819 

Pacut, Crystal M1005 

Paganini-Hill, Annlia S108 

Paik, Myunghee C. S101 

Pakdaman, Hosein T1734 

Pakdaman, Hossein S114, T1712, 

T1722, T1723, T1731, T1732 

Pakdaman, Hossien T1718 

Pakdaman, Reza T1712, T1723 

Palmer, Valerie S. T1511 

Palusak, Ryan T1503 

Pan, Dengji S110 

Pan, Di S222, S227, S235 

Pan, Yue S. T1908 

Pandya, Dipak T1737 

Pandya, Dipak P. M621, M717, 

M721, T1619, T1738 

Pandya, Shree M806 

Pankratz, V. Shane T1503 

Panou, Theodora M603 

Papadaki, Efrosyni M603 

Paparella, Gabriella M611, 

M1002 

Parent, Jack M. M1401 

Parisi, J.E. S204, S209 

Parisi, Joseph E. M1008, T1515, 

T1530 

Park, Yikyung S208 

Parker, Kathy S301 

Parkinson Study Group S228 

Pascual, Franchette M703 

Patel, Vivek S140 

Pati, Sandipan M715, M1201 

Paul, Joya S119 

Pavlakis, Pantelis P. T1729 

Paz Soldan, M Mateo T1715 

Paz Soldan, Mateo S403 

Pazdera, Ladislav M724 

Pearlman, Starr H. T1613 

Pease, Larry M1101 

Pease, Larry R. T1703 

Peavy, Guerry T1507 

Pedraza, Otto T1503 

Pejovic, Vojislav T1516, T1517, 

T1523, T1526 

Pelletier, Jean T1708, T1713 

Pelster, Michael W. S224 

Peltier, Amanda C. M810 

Peltz, Carrie B. T1508, T1524 

Perez, Veronica M1403 

Perhach, James T1533 

Pericak-Vance, Margaret T1512 

Peruch, Francesca M1002 

Pervez, Shahid S229 

Pestreich, Linda T1735 

Pestronk, Alan M815, M821 

Petacchi, Elisa S237 

Petersen, Bryan T1606 

Petersen, Ronald C. M811, 

T1503, T1515, T1530 

Petrovitch, Helen S203 

Pettingill, Philippa T1711 

Pettingill, Rosie T1711 

Phibbs, Fenna T. S226 

Picard, Christophe T1713 

Piccoli, Sara M611 

Pickett, Erin J. M613 

Pikula, Aleksandra S122, T1504 

Pillai, Jagan M1010, S205, S220 

Pillai, Jagan A. T1525 

Piña-Crespo, Juan C. T1509 

Pirko, Istvan T1703, T1715, 

T1727 

Plaitakis, Andreas M603 

Pleasure, David T1705 

Plow, Ela B. T1527 

Plow, Matthew A. T1740 

Polasek, Ozren T1503 

Pomerants, Polina S401 

118 

Pontecorvo, Michael J. T1508 

Pope, Daniel L.W. M601 

Prabhakaran, Shyam S119 

Pracilio, Valerie P. T1614 

Prakash, Neal T1601 

Presti, Michael F. S403 

Prokop, Stefan M835 

Rabinstein, Alejandro A. M1202, 

M1203, S128, T1714 

Race, David S. M612 

Rademakers, R. S204, S209 

Rademakers, Rosa S207 

Radlinska, Basia T1510 

Radue, Ernst-Wilhelm T1707 

Rahimian, E. T1723 

Rai, Shesh N. S213 

Rajamani, Kumar S129 

Rajput, Alex H. S217 

Rajput, Ali H. S217 

Rajput, Michele L. S217 

Raman, Rema S105 

Rametta, Mark T1702 

Ramos-Crawford, Ana-Luisa 

T1511 

Ramsey-Williams, Vicki S404 

Rangaraju, Srikant T1902 

Ranjan, Tulika T1808 

Rao, Sambasiva T1719 

Raol, Yogendra H. M705 

Rashid, Saifur M620 

Rasmuss, Brett M712 

Ravina, Bernard S216 

Raymond, Deborah S233 

Reda, Haatem S403 

Reder, Anthony T. T1702 

Reed, Michael T1613 

Reed, Michael L. T1608, T1610, 

T1611 

Reeves-Tyer, Patricia M702 

Reich, Stephen S213 

Reiman, Eric M. T1502 

Reitz, Christiane T1512 

Reminick, Jason I. S402 

Ren, Xuefeng T1511 

Rengachary, Jennifer M601 

Rentería Palomo, Ana A. M617 

Riley, David S213 

Ritchie, James C. S301 

Rivera, Susan M. T1521 

Rizzo, Matthew M615 

Roberts, Bruce T1719, 

T1721 

Robinson, Karen A. S118 

Rodriguez, Alcibiades S302 

Rodriguez, Ildefonso T1907 

Rodriguez, Jeronimo T1907 



Rodriguez, Moses M1101, 

T1703, T1727 

Rodríguez Leyva, Ildefonso M617 

Roeber, S. S204, S209 

Rogaeva, Ekaterina T1512 

Roger, Elaine T1713 

Romero, Jose R. S122 

Rosales, Xiomara Q. M802 

Rose, Kathryn M. T1505 

Rosenbaum, Anna V. M723 

Rosenberg, Michael S134 

Rosenfeld, William M713 

Ross, Owen A. S207 

Rossetto, Mariagiovanna M1002 

Rostami, Abdolmohamad T1725 

Rothman, Brian T1534 

Rothner, A.D. M1302 

Rothrock, John F.S113, T1602, 

T1609 

Rothstein, Jeffrey M821 

Roubenoff, Ronenn T1504 

Rounds, William H. T1736 

Rowley, Christopher N. T1503 

Royal, III, Walter T1717 

Rudan, Igor T1503 

Ruhoy, Ilene M1304 

Rumbaugh, Jeffrey A. T1902 

Rundek, Tatjana S101 

Russek, Shelley J. M705 

Russell, James W. M801 

Russo, Priscilla M819 

Rye, David B. S301 

Saber Tehrani, Ali S. S118, S139 

Sabouri, Amir H. T1905 

Sacco, Ralph L. S101 

Sadzewicz, Lisa K. S107 

Safavi, Farinaz T1725 

Sahenk, Zarife M802 

Sahraian, Mohammad T1732 

Sahraian, Mohammad Ali 

T1712, T1723 

Sakowski, Stacey A. M804 

Salami, Shiva T1722 

Salim, Sumaiya S137 

Salins, Naomi S218, S222, 

S227, S235 

Salmon, David P. T1525 

Salowich-Palm, Leeza S129 

Salvador, Santamaria S230 

Samadpour, Reza T1731 

Samson, Leona D. T1511 

San Luciano, Marta S233 

Sanchez-Ramos, Juan S202 

Sancho, Jose T1719 

Sando, Sigrid B. T1503 

Sanjak, Mohammed S. M819 

Santiago, Anthony S218, S222, 

S227, S235 

Sanz-Blasco, Sara T1509 

Sarkar, Korak M1102 

Sarwar, Aliya S215 

Sato, Susumu M702 

Sauerbeck, Laura S112 

Saunders-Pullman, Rachel S233 

Scarmeas, Nicholas S225 

Schiff, Nicholas C. M1104 

Schirrmacher, Ralf T1510 

Schneck, Michael J. S405 

Schneider, Andrea T1521 

Schneider, Julie A. T1502 

Schofield, Lesley T1735 

Schor, Nina F. T1803 

Schretlen, David J. M613 

Schwarzchild, Michael S216 

Schweitzer, K.J. S204, S209 

Scoglio, Nicholas J. S402 

Scott, Bonnie M. T1522 

Scott, James N. M1103 

Scott, Thomas T1726 

Sealfon, Stuart C. M824 

Sen, Saurav M721 

Sen, Sourav T1619 

Sen, Suman S206, S212 

Sennett, Cary T1614 

Seo, Sang Won T1519 

Seritan, Andreea T1521 

Serrano, Daniel T1612, T1613 

Seshadri, Sudha T1504 

Severy, Peter T1719 

Seyal, Masud M703 

Shaffer, Michel L. S212 

Shaffer, Michele L. S206, S234 

Shah, Amol T1905 

Shah, Anish M721, T1619 

Shah, Umang M719 

Shahbazi, Mona M824 

Shahbegi, Said S114 

Shahbeigi, Saeed T1734 

Shahkarami, Mohammad Amir 

T1722 

Shahlaie, Kia M1102 

Shamim, Ejaz M602 

Shankar, S.K. M822 

Shankara, Srinivas T1719 

Sharpe, James A. M607, T1621 

Sharrett, A. Richey T1505 

Sheikh, Kazim M809 

Shepherd, Cassandra S213 

Shibata, Dean K. T1505 

Shijie, Song S202 

Shimizu, Kanako S120 

Shin, Robert K. M723 

119 

Shiramizu, Bruce S407 

Shojaee, Maziar T1731 

Shojaei, Maziar T1718 

Shoulson, Ira S216 

Shprecher, David S213 

Shtilbans, Alexander M824 

Shulman, Gordon L. M601 

Shulman, Joshua M. T1502 

Shuster, E.A. S209 

Shy, Michael E. M803 

Siddiqui, Fazeel S140 

Siders, William T1719, T1721 

Siemionow, Vlodek T1527 

Silber, Michael H. M811 

Silberstein, Stephen T1603, 

T1614 

Silbert, Lisa T1532 

Sillau, Stefan M616 

Silva, Jessica M613 

Simon, Mariella M1008 

Simos, Panagiotis G. M603 

Simpson, Ericka M821 

Simpson, Ericka P. M832, 

T1728 

Simuni, Tanya S228 

Singh, Anuradha S302 

Singleton, Robinson J. T1604 

Skrap, Miran S237 

Slevin, John T. M709 

Sloan, Michael S130, S135 

Smith, David I. M1008 

Smith, Glenn E. M1008 

Smith, Gordon A. T1604 

Smith, Jonathan H. S403 

Smith, Richard M821 

Smith-Hammond, Carol M1107 

Snider, Barbara J. S406 

Snyder, Abraham Z. M601 

So, Norman K. M711 

Sofroniew, Michael T1704 

Solomon, Tom T1903, T1904 

Somarajan, Bindu I. M1003, 

S104 

Song, Yanna S226, S303 

Sorenson, Eric M826 

Sorenson, Eric J. M817 

Soucy, Jean-Paul T1510 

Spence, Rory T1704 

Spencer, Peter S. T1511 

St. George-Hyslop, Peter T1512 

St. Louis, Erik K. M811 

Standaert, David S213 

Stankiewicz, James M. S408 

Stanley, Kaili M. S233 

Statland, Jeffrey M. M806 

STEADY PD Investigators S228 



Stebbins, Glenn T. S211, S231 

Stern, Barney J. S107 

Stewart, Anna T1903, T1904 

Stine, Oscar C. S107 

Stites, Tracy T1708 

Stouter, Josephine T1522 

Strongosky, Audrey M1007 

Struck, Peter M834 

Strutt, Adriana M. T1522 

Sullivan, Robert S. T1511 

Sundal, C. S204, S209 

Sundar, Kaushik S121 

Sung, Kyongje M613 

Sura, Ankit M801 

Suter, Ueli M803 

Suzuki, Seiko M818 

Swanson, Kenneth D. T1802 

Tabasi, M. S114 

Tabatabae, Al S114 

Takahashi, Masaki S201 

Talantova, Maria V. T1509 

Tallon, Luke J. S107 

Tan, Caroline M602 

Tanaka, Ichiro T1520 

Tang, Dejun T1709 

Tang, Zhouping S110 

Tanner, Caroline M. S203, S216 

Tawil, Rabi M806 

Tayal, Ashis S124 

Taylor, Glyn T1616 

Teich, Douglas L. T1622 

Tenderini, Erika M1002 

Tennen, Howard T1615 

Tepper, Stewart T1603, T1617 

Teshome, Mengesha T1901 

Thant, Minn S215 

Thawani, Sujata S302 

Theodore, William H. M702 

Theriot, Jeremy J. T1601 

Thiel, Alexander T1510 

Thomas, Jewell T1901 

Thomas, Tracy T1616 

Tilluckdharry, Natasha 

M621, T1738 

Timaran, Carlos H. S116 

Ting, Jess M824 

Title, Wallace S224 

Titulaer, Maarten J. T1701, 

T1716 

Tobias, Kathy T1735 

Tocco, Michael T1516, T1517, 

T1523, T1526 

Todd, Wesley M. M710 

Toga, Arthur W. T1601 

Tokuyama, Yoshiaki S120 

Toro, Camilo M820 

Toscano, Antonio M1002 

Tosches, William M1105 

Tramontana, Michael G. S232 

Tran, Dong T1502 

Traynor, Bryan J. M820 

Trenerry, Max R. M606 

Trotti, Lynn Marie S301 

Tselis, A. S209 

Tufail, Yusuf M715, M1201 

Turkel, Catherine C. T1608, 

T1612 

Turner, Travis H. S219 

Tuszynski, Mark H. T1529 

Tyler, William J. M715, M1201 

Ueda, Masami M818 

Uitti, Ryan S213 

Umaiorubahan, 

Meenakshisundaram S121 

Umeda, Elizabeth T1704 

Uyehara-Lock, Jane H. S203 

Vallurupalli, Srikanth S140 

Van Gerpen, J.A. S204, S209 

Van Keulen, Virginia M1101 

Van Liew, Charles S205, S220, 

T1507 

Van Loveren, Harry R. 

S130, S135 

Van Ness, Paul C. M711 

Vanaman, Thomas C. M709 

Vannorsdall, Tracy D. M613 

Varon, Sepideh F. T1612 

Vasan, Ramachandran S. T1504 

Vaughan, Douglas E. S303 

Vazquez, Gilberto T1907 

Vecera, Shaun M615 

Velazquez-Rodriguez, 

Yadira M1205 

Veloski, Jon S223 

Venkatasubramaniam, 

Shankar S121 

Verghese, Joe T1518 

Verma, Aparajitha K. M832 

Vestri, Alec M611 

Victor, Jonathan D. M1104 

Vieira, Julio R. S101 

Vigo, Carmen T1506 

Villoslada, Pablo T1713 

Vincent, Angela T1711 

Viollet-Callendret, Laurence 

M802 

Voeks, Jenifer H. S102, S116 

von Rosenstiel, Philipp T1707 

Voskuhl, Rhonda T1704 

Vysata, Oldrich M724 

Wada, Keiji S201 

Wagner, Daymond S206, S212 

120 

Wagner, Jenee M1006 

Wagner, Kathryn R. M806 

Wakayama, Yoshihiro M829 

Walgren, Kristy M819 

Walker, Linsey M. M704 

Wallace, Douglas C. M1008 

Wallin, Mitchell T. T1730 

Wang, Aijun T1724 

Wang, Chunyang M719 

Wang, Jay-Ming S130, S135 

Wang, Lily S226, S303 

Wang, Min T1603, T1617 

Wang, Morgan S130 

Wang, Shuu-Jiun T1605 

Wang, Wei S110 

Wang, Yen-Feng T1605 

Ward, Amber L. M814, M819 

Ward, Christopher J. M1008 

Warrington, Arthur M1101 

Warrington, Arthur E. T1703 

Watanabe, Osamu T1711 

Watanabe, Shoko S201 

Waters, Patrick T1711 

Watson, James M826 

Watson, Sam S129 

Waubant, Emmanuelle T1713 

Webber, Christine A. M1402 

Webster, Ross S203 

Weibelt, Silvia M. T1602 

Weihl, Chris M815 

Weiner, Howard T1720 

Weiss, John H. M805 

Werner, Perla T1535 

Westwood, Andrew J. T1504 

White, Lon R. S203 

Whiteheart, Sidney W. M709 

Whitfield-Gabrieli, Susan M704 

Whitwell, Jennifer M606 

Whitwell, Jennifer L. T1530 

Wicklund, Meredith S403 

Wider, C. S204, S209 

Wiggs, Edythe M702 

Wijdicks, Eelco F.M. M1202, 

M1203, S128 

Wilde, Elisabeth A. M1103 

William, Gahl M820 

Williams, Nicole M. M814, 

M819 

Wilson, Karen R. S130, S135 

Winblade Nairn, Natalie T1724 

Winner, Paul T1603 

Wojna, Valerie S407 

Wolf, Philip A. T1504 

Woltjer, Randy T1532 

Wong, Agnes M. T1621 

Wong, Amy S109 



Wong, Eric T. T1622, T1802 

Wong, Sarah T1701 

Wood, Blair T1801 

Woodruff, B.K. S204, S209 

Worrall, Bradford B. S115 

Wozniak, Marcella A. S107 

Wright, Alan T1503 

Wright, Brent M1101 

Wright, Kathryn A. M819 

Wszolek, Z.K. S204, S209 

Wszolek, Zbigniew T1503 

Wszolek, Zbigniew K. M1007, 

S207 

Wu, Yanhong M1008 

Wyant, Alexandria T1527 

Wymbs, Nicholas M604 

Xiang, Diane T1736 

Xu, Feng S110 

Xu, Jian T1701 

Xu, Kui M620 

Xu, Xiaohua M1101 

Xue, YuanYuan T1728 

Yamanishi, Hiromitch M1008 

Yamashita, Toshihide T1520 

Yan, Qing M803, M813 

Yanagihara, Keiko T1520 

Yang, Fan S108 

Yang, Lauice S221 

Yanik, Brandon M. T1604 

Yavorsky, Christian T1534 

Yin, Hong Z. M805 

Yonekawa, Tomomi M1009, 

T1706 

Yono, Noor M722, S133, T1808 

York, Michele K. T1522 

Yoshihiro, Anna M715, M1201 

Yoshii, Yasuhiro M812 

Yoshimura, Satoshi M1009, T1706 

You, Xiaojun T1726 

Younger, David S. M827 

121 

Younkin, Curtis S. T1503 

Younkin, Steven G. T1503 

Yu, Alexander T1622 

Yu, Chuanhui S108 

Yue, Guang H. T1527 

Yung, Raymond M1005 

Zarbl, Helmut T1511 

Zerafati, Gazelle S223 

Zghouzi, Mohamed S119 

Zhang, Gang M809 

Zhang, Xuebao M803 

Zhang-Auberson, Lixin T1707, 

T1709 

Zhou, Yun S118 

Zhu, Suiqiang S110 

Zimmerman, Earl T1807 

Zinn, Kristi M601 

Zivadinov, Robert S117 

Zochodne, Douglas W. M1402 

Zou, Fanggeng T1503 



Abdemalik, Peter M729 

Ackermann, Elizabeth M841 

Ahmahi, Naser T1540 

Albers, Gregory W. M1206 

Albert, Todd T1538 

Alvarado, Luis M841 

Angelini, Corrado I. M838 

Arnold, Steven E. T1542 

Askanas, Valerie M837 

Avila-Costa, Maria Rosa M730 

Babaei, Hormoz T1540 

Bai, Dongsheng M730 

Barlow, Carrolee T1538 

Bateman, Randall J. T1541 

Beason-Held, Lori T1539 

Benson, Merrill M841 

Benzinger, Tammie T1541 

Berdichevsky, Yevgeny M727 

Bettermann, Kerstin S142 

Bhattachuria, Arun T1623 

Booten, Sheri M841 

Bordeau, Jane M840 

Boulis, Nicholas M840 

Bowen, James D. T1743 

Brigatti, Karlla W. M1011 

Brookler, Kenneth H. T1745 

Cacciottolo, Mafalda M837 

Carlen, Peter L. M729 

Cevette, Michael J. T1745 

Chakraverty, Sumi T1541 

Chang, Dae-il S141 

Chaudhry, Zeshan A. M1206 

Cocco, Daniela T1745 

Cohen, Todd T1542 

Connors, James S144 

D’Agostino, Carla M837 

Dambinova, Svetlana S142 

Davis, Stephen M. M1206 

Delgado-Escueta, Antonio V. 

M730 

Deutsch, Eric C. M1011 

Dhir, Ashish M731 

Donnan, Geoffrey A. M1206 

Duron, Reyna M. M730 

Eberly, Shirley S238 

Ebers, George C. M1012, M1013 

Ebrahimi, Ramin T1540 

Ehrlich, Michelle E. T1538 

Elbert, Don T1541 

Elisman, Mark M839 

Elm, Jordan J. M1206 

Engel, W.King M837 

Fague, Scot T1541 

WIP AUTHOR INDEX 

Fanin, Marina M838 

Federici, Thais M840 

Feldman, Eva M840 

Ferrucci, Luigi T1539 

Florez, Carlos M. M729 

Gandy, Sam T1538 

Gangadharan, Beena T1743 

Garg, Rajeev S143 

Glabe, Charles T1538 

Glass, Jonathan D. M840 

Goate, Alison T1541 

Gonzalez, R. G. M1206 

Govindarajan, Raghav T1747 

Grachev, Igor D. S240 

Green, Mark W. T1623 

Grisham, Mathew B. T1746 

Grossman, Murray T1542 

Guevara, Jorge M730 

Guevara, Patricia M730 

Guo, Jiasong M839 

Guo, Shuling M841 

Hajsadeghi, Fereshteh T1540 

Havrdova, Eva T1741 

Hawi, Amale M1011 

Hazel, Tom M840 

Heo, Sung Hyuk S141 

Hogarth, Penelope S240 

Horakova, Dana T1741 

Huber, Vincent J. T1744 

Hughes, Steve M841 

Irwin, David J. T1542 

Iwata, Atsushi M836 

Jacobson, Sven M1206 

Johe, Karl M840 

John, Sayona S143 

Jung-Henson, Lily K. T1743 

Kalincik, Tomas T1741 

Karlsson, Fridrik T1746 

Kasten, Tom T1541 

Kelly, Crystal M840 

Keogh, Bart P. T1743 

Kieburtz, Karl S239 

Kim, Hye Ok S141 

Kim, Soong Ho T1538 

Kimberly, W. T. M1206 

Krasensky, Jan T1741 

Kraut, Michael T1539 

Kubota, Akatsuki M836 

Kucheruck, Olena M1011 

Kwee, Ingrid L. T1744 

Laguerre, Tatanisha S240 

Lam, Ann M622, M728 

Lee, Virginia M.Y. T1542 

122 

Lee, Viven S143, S144 

Leinonen, Mika S239 

Li, Jun M839 

Lian, Gewei M726 

Lovestone, Simon T1539 

Lynch, David R. M1011 

Machado-Salas, Jesus M730 

Mail, Michelle M727 

Malhotra, Konark S143, S144 

Maravilla, Kenneth R. T1743 

Marek, Kenneth S238 

Marino, Julia T1742 

Martinez, Nicholas E. T1746 

Matthews, Ian R. T1742 

Mawuenyega, Kwasi T1541 

Mayadev, Angeli T1743 

McIntyre, Cameron M839 

Miller, Guy M1011 

Minagar, Alireza T1746 

Monia, Brett M841 

Morris, John C. T1541 

Mowry, Ellen M. T1742 

Nakada, Tsutomu T1744 

Nakayama, Yukihiro T1744 

Nalls, Michael T1539 

Nascimbeni, Annachiara M838 

Nissinen, Helena S239 

Nogalska, Anna M837 

Nutt, John G S240 

Oakes, David S238 

Oakley, Sarah T1745 

Ohayon, Elan L. M622, M728 

Olanow, C. Warren S239 

Omura, Seiichi T1746 

Ovid, Vitaliy T1541 

Park, Byung S. S240 

Patterson, Bruce T1541 

Poewe, Werner S239 

Polak, Meraida M840 

Potter, Rachel E. T1541 

Prabhakaran, Shyam S143, S144 

Pradhan, Gaurav T1745 

Rajappa, Sivakumar M. M725 

Ramagopalan, Sreeram V. 

M1012, M1013 

Rascol, Olivier S239 

Ravina, Bernard M. S238 

Repovic, Pavle T1743 

Resnick, Susan T1539 

Rioux, Patrice M1011 

Rizzuto, Daniel S. T1743 

Rogawski, Michael A. M731 

Ross, Mark A. T1745 



Rutkove, Seward M840 

Salgado, Efrain T1747 

Sandri, Marco M838 

Saponjian, Yero M727 

Sato, Fumitaka T1746 

Schapira, Anthony S239 

Sciascia, Thomas M1011 

Seidl, Zdenek T1741 

Sejnowski, Terrence J. M728 

Sheen, Volney M726 

Sheth, Kevin N. M1206 

Shimizu, Jun M836 

Shoulson, Ira S238 

Shrader, William D. M1011 

Sigurdson, Wendy T1541 

Singleton, Andrew T1539 

Siwkowski, Andrew M841 

Smith, Benn E. T1745 

Sosinsky, Gina M839 

Spitz, John T. M732 

Staley, Kevin J. M727 

Steele, John W. T1538 

Stepanek, Jan T1745 

Stern, Barney J. M1206 

Stocchi, Fabrizio S239 

Suter, Ueli M839 

Suzuki, Yuji T1744 

Tanaka, Miyabi M730 

Temes, Richard S143 

Tendolkar, Amol S240 

Thambisetty, Madhav T1539 

Treuner, Kai T1538 

Trojanowski, John Q. T1542 

Troyer, Matthew D. S240 

Tsuji, Shoji M836 

Tsunoda, Ikuo T1746 

123 

Underhill, Hunter R. T1743 

Vaneckova, Manuela T1741 

Venkatesan, Srinivasan A. M725 

Vinayagasundaram, Uma T1742 

Wagner, Lindsay T1745 

Wang, Lily M839 

Waubant, Emmanuelle T1742 

Willi, Steve M. M1011 

Wilson, Robert B. M1011 

Xie, Sharon X. T1542 

Xuan, Fengjuan S240 

Yan, Qing M839 

Yarasheski, Kevin T1541 

Yarnykh, Vasily L. T1743 

Yoo, Albert J. M1206 

Zapala, David A. T1745 

Zhang, Jane M729 

Zhang, Liang M729 


A 

Acute Cerebral Ischemia M721, S102, S105, S109, S116, 

S118, S120, S121, S128, S136, T1619 

Acute Ischemic Stroke S102, S103, S105, S109, S114, 

S116, S117, S120, S122, S124, S125, S127, 

S129, S130, S131, S132, S135 

Acute Neuropathies M818, M822, M823, M825, M827, 

M830, T1733 

Affective Functioning M608, M617, M819, S203 

Alzheimer’s Disease (AD) M616, M808, M1001, T1501, 

T1502, T1503, T1504, T1506, T1508, T1509, 

T1512, T1514, T1516, T1517, T1518, T1521, 

T1522, T1523, T1524, T1525, T1526, T1528, 

T1529, T1531, T1532, T1533, T1534, T1535, T1901 

Amyotrophic Lateral Sclerosis (ALS) M804, M805, 

M807, M813, M817, M821, M824, 

M829, M1005 

Antibodies M602, M809, M818, M822, M825, M1101, 

S202, T1506, T1701, T1703, T1706, T1711, 

T1716, T1719, T1721, T1729, T1733, 

T1736, T1739, T1801, T1908 

Apoptotic Cell Death T1537, T1801, T1803 

Astrocytes M1004, T1506, T1509, T1704, 

T1705, T1710 

Atherosclerosis M1003 

Attention-Deficit/Hyperactivity Disorder M610, M614, 

M617, M618, M619 

Autoimmune Demyelination M1105, M1303, T1704, 

T1713, T1729, T1731, T1732, T1734, T1738 

B 

Brain Imaging M601, M606, M704, M719, M1103, 

M1104, M1301, S117, S119, S125, S128, 

S132, S140, S204, S212, T1510, T1513, T1514, 

T1518, T1525, T1527, T1532, T1601, T1619, 

T1714, T1715, T1804, T1906, T1908 

Brain Ischemia M605, M1201, M1204, S103, S106, 

S114, S115, S126, T1519 

Brain Mapping M601, M604, M605, M609, M708, 

M724, S109, T1527, T1601 

C 

J_ID: ZAY Customer A_ID: SUPP11-12 Cadmus Art: 22585 Date: 26-August-11 Stage: Page: 124

I 

Interferon Treatments T1702, T1722, T1724 

Interferons M1001, T1722, T1726 

Intracerebral Haemorrhage (ICH) M1003, S104, S105, 

S110, S111, S113, S117, S124, S125, 

S128, S132, S136 

Ischemia S124, S126, S133 

L 

L-Dopa Therapy S215 

Levodopa S232, S236 

Lewy Bodies (LB) S207 

Limbic Encephalitis T1701 

M 

Magnetic Resonance Imaging (MRI) M612, M719, 

M721, M828, M1301, S106, S204, S212, S234, 

T1504, T1505, T1514, T1519, T1521, T1525, 

T1530, T1532, T1605, T1726, T1727, 

T1737, T1738 

Magnetic Resonance Spectroscopy (H-MRS) M812 

Migraine S136, T1601, T1602, T1603, T1608, T1609, 

T1610, T1611, T1612, T1613, T1614, 

T1615, T1616, T1617, T1618, T1620 

Morvan Syndrome T1711 

Motoneuron Disease M804, M805, M812, M813, 

M814, M817, M819, M820, M832, 

M833, M1002, T1511 

Multiple Sclerosis (MS) M603, M1009, M1105, T1621, 

T1702, T1703, T1704, T1707, T1708, 

T1709, T1710, T1712, T1713, T1715, T1717, 

T1718, T1719, T1720, T1721, T1722, T1723, 

T1724, T1725, T1726, T1727, T1729, T1730, 

T1731, T1732, T1734, T1735, T1736, 

T1737, T1740 

Muscular Dystrophies M806, M807 

Myelin M803 

Myelin Oligodendrocyte Glycoprotein (MOG) T1737 

Myoclonus M621, M717 

Myopathic Syndrome M815, M816 

Myopathy M802, M806, M807, M808, M811, 

M815, M816, M829, M835 

N 

J_ID: ZAY Customer A_ID: SUPP11-12 Cadmus Art: 22585 Date: 26-August-11 Stage: Page: 125

A 

Acute Cerebral Ischemia S142 

Acute Ischemic Stroke M1206, S142 

Affective Functioning M622, T1745 

Alzheimer’s Disease (AD) M730, T1538, T1539, 

T1540, T1541, T1542 

Alzheimer’s Disease Mutants T1541 

Astrocytes T1744 

Atherosclerosis S141 

Autoimmune Demyelination T1742 

B 

Brain Imaging M622, M1206, S143, S144, S238, 

T1539, T1741, T1743, T1744 

Brain Ischemia M1206, S142 

Brain Mapping M622, T1745 

Brain Metabolism M727, M729, T1539, T1541 

C 

Cell Death M728, M1011 

Cerebrospinal Fluid (CSF) T1541 

Cognitive Outcomes M728, S238 

D 

Dementia T1538 

Demyelination M839, T1741, T1742, T1743 

E 

Epilepsy M726, M727, M731, M732 

Epilepsy Model M728, M729, M730, M731 

Epileptic Seizures M729 

Epileptogenesis M727, M728, M729 

F 

Frontotemporal Dementia (FTD) T1542 

G 

Gene Expression M838, S141 

Gene Regulation M726, M1013 

Genetic Mutations M726, M839, M841 

H 


Headache S143, S144, T1623 

WIP SUBJECT INDEX 

126 

L 

Lafora Disease M730 

L-Dopa Therapy S240 

M 

Magnetic Resonance Imaging (MRI) M1206, T1741, 

T1743 

Migraine T1623 

Multiple Sclerosis (MS) M1012, T1741, T1742, T1743 

Muscular Dystrophies M838 

Myelin M839, T1745 

Myoclonus M730 

Myopathy M836, M837, M838 

N 

Neurodegenerative Diseases M840, M841, T1538, 

T1542 

Neurogenesis M726, M839, T1538, T1744 

Neuromyelitis Optica (NMO) T1742 

Neuroprotection M727, M1011 

O 

Other M622, M725, M731, M836, M837, M840, 

M841, M1011, M1012, M1013, S141, S239, 

S240, T1540, T1623, T1745 

P 

Parkinson’s Disease (PD) S238, S239, S240 

PET T1539, T1744 

Pharmacokinetics M841, S240 

Pompe Disease M838 

R 

Reactive Oxygen Species (ROS) M1011 

S 

Single Photon Emission Computed Tomography S238 

Stroke S141, S142, S143, S144 

Subarachnoid Hemorrhage S143, S144 

T 

Tau M837, T1542 


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136 - American Neurological Association

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