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Pediatric Neurology - UT Southwestern

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Susan Iannaccone, M.D.<br />

Professor,<br />

Director, <strong>Pediatric</strong> Nephrology<br />

Faculty<br />

<strong>Pediatric</strong> <strong>Neurology</strong><br />

2011 Academic Review<br />

The Division of <strong>Pediatric</strong> <strong>Neurology</strong> is the only academic group in North Central Texas<br />

providing consultations and multi-disciplinary care for children with all kinds of<br />

neurologic disease. Internationally recognized faculty provide care at Children’s Medical<br />

Center’s Dallas, Legacy and Southlake campuses and at Texas Scottish Rite Hospital for<br />

Children (TSRHC).<br />

Each member of the faculty provides special expertise in areas of focus including<br />

epilepsy and epilepsy surgery, neuromuscular disorders, stroke, demyelinating disease,<br />

autism, cerebral palsy and headache. Patients receive a sophisticated and integrated<br />

approach to diagnosis including genetic testing, metabolic analysis and interpretation<br />

of neuroimaging.<br />

Our multidisciplinary clinics include, but are not limited to:<br />

• Urgent epilepsy<br />

• Ketogenic diet<br />

• Myasthenia<br />

• Childhood stroke<br />

• Autism<br />

• Transverse myelitis<br />

• Neuroncology (directed by<br />

Hematology/Oncology)<br />

• Headache (directed by Pain<br />

Management Team)<br />

Our group includes 13 full-time faculty based at Children’s and two based at TSRHC. In addition, we have one part-time<br />

faculty serving in Neuro-oncology and one volunteer faculty serving in a teaching capacity. There are a total of 10 advanced<br />

practice providers: two physician assistants and eight nurse practitioners who work alongside our faculty to provide timely<br />

and comprehensive care.<br />

Susan Arnold, M.D.<br />

Associate Professor<br />

Michael Dowling, M.D., Ph.D.<br />

Assistant Professor<br />

Patricia Evans, M.D.<br />

Associate Professor<br />

Sailaja Golla, M.D.<br />

Assistant Professor<br />

Susan Iannaccone, M.D.<br />

Professor, Division Director<br />

Muna Kahn, M.D.<br />

Assistant Professor<br />

Saadat Kahn, M.D.<br />

Assistant Professor<br />

Fares Kokash, M.D.<br />

Assistant Professor<br />

Rana Said, M.D.<br />

Assistant Professor<br />

Deepa Sirsi, M.D.<br />

Assistant Professor<br />

William Zinser, M.D.<br />

Associate Professor<br />

In addition to our faculty, the division has:<br />

• one psychometrician<br />

• four academic administrative staff<br />

• one program administrator-clinic<br />

• one program administrator-epilepsy<br />

• eleven nursing staff<br />

• one case manager<br />

• one social worker<br />

• one interpreter<br />

• seven research coordinators<br />

• one dietitian<br />

• four clinic techs/MA’s<br />

• two front desk/call center support people<br />

Dr. William Zinser retired in December 2011.<br />

1


Research Activities<br />

Division faculty conduct a variety of neurologic bench/basic research and clinical studies, including clinical trials. Current<br />

studies are focused in five areas:<br />

• metabolic disorders of the nervous system<br />

• neuromuscular disease clinical trials<br />

• anti-epileptic drug trials<br />

• clinical trials in pediatric stroke<br />

• clinical studies in the autism spectrum<br />

Faculty present results of their work at many national and international meetings and published over 30 articles in peerreviewed<br />

journals in 2011. Dr. Iannaccone is a co-investigator for the NIH Network for Excellence in Neuroscience Clinical<br />

Trials (NeuroNEXT) which started in October 2011. <strong>UT</strong> <strong>Southwestern</strong> <strong>Neurology</strong> is one of 25 sites to participate in this<br />

study. NeuroNEXT is designed to increase the efficiency of clinical trials, facilitate patient recruitment and retention,<br />

increase the quality of neuroscience clinical trials, and enable public-private partnerships. The network aims to share<br />

expertise and infrastructure across diseases, leverage research resources at clinical sites and take advantage of clinical<br />

research opportunities as they arise. This clinical research network will provide a robust, standardized, and accessible<br />

infrastructure to facilitate rapid development and implementation of protocols in neurological disorders affecting adult<br />

and/or pediatric populations. The network is designed to assure the broadest access to new therapies for patients by carrying<br />

out trials coming from partnerships between NINDS and industry, foundations or academia.<br />

Clinical Activities<br />

The expertise of the faculty is focused on several sub-specialties: comprehensive epilepsy management including<br />

seizure surgery; neuromuscular medicine clinics including myasthenia gravis, muscular dystrophy, Charcot Marie<br />

Tooth and transitional care for adolescent muscle; cerebrovascular disease; neonatal intensive care follow-up; Zero-2-3<br />

neurodevelopment clinic; rare disorders clinic; and neurodevelopmental disabilities clinics including autism, neurocognitive<br />

delay, attention-deficit hyperactivity disorder/learning disabilities (ADHD/LD) and traumatic brain injury.<br />

Neurodevelopmental Disabilities Program<br />

The Neurodevelopmental Disabilities Program (NDD) concludes its fourth year under the direction<br />

of Dr. Patricia Evans at <strong>UT</strong> <strong>Southwestern</strong>. The program continues to experience significant growth<br />

specifically with regard to clinical opportunities, research, education and community outreach. NDD<br />

clinics are multi-disciplinary in structure and are specifically created to serve children with autism,<br />

developmental delay, traumatic brain injuries and ADHD/LD issues. The program has expanded to<br />

provide full-time NDD services to both the Childrne’s Dallas and Legacy campuses.<br />

The NDD team serves the North Texas community at large. The team attends special needs children<br />

on site at the Dallas-based Autistic Treatment Center on a quarterly basis, and works closely with Dr.<br />

Carolyn Garver and her outstanding team of specialists. Drs. Evans and Morris speak throughout<br />

the metropolis in various educational settings, including the Dallas ISD, the Dallas Theological<br />

Seminary, and other specialty programs regarding children with special needs.<br />

Dr. Patricia Evans<br />

Associate Professor<br />

Epilepsy Program<br />

The Epilepsy Program, under the direction of Dr. Susan Arnold, includes a Designated Level 4<br />

Epilepsy Center, the first and only <strong>Pediatric</strong> Epilepsy program in the Country to be certified by the<br />

Joint Commission.<br />

The program includes the Children’s Epilepsy Monitoring Unit (EMU) that contains eight beds<br />

and providing 24/7 coverage for patients in the pediatric intensive care unit (PICU), the neonatal<br />

intensive care unit (NICU) and the emergency department. The EMU team includes an attending,<br />

support staff, and several third-year medical students on their required neurology rotation plus<br />

rotating residents from adult neurology, psychiatry or other specialties.<br />

Dr. Susan Arnold<br />

Associate Professor<br />

2


Neuromuscular Clinic<br />

Under the supervision of Dr. Susan Iannaccone the neuromuscular clinic<br />

is the only full-time pediatric program in North Texas to be sponsored<br />

by the Muscular Dystrophy Association (MDA). The aim of these<br />

multidisciplinary clinics are to provide comprehensive health care for the<br />

pediatric neuromuscular population. The multidisciplinary approach<br />

fosters an environment of all-inclusive health care. Clinic is held four<br />

days per week.<br />

Stroke Clinic<br />

Annual Ketogenic Diet Halloween Party<br />

The <strong>Pediatric</strong> Stroke Clinic, established in 2003, is directed by Dr. Michael Dowling and holds two<br />

half-day clinics per week. The patient population consists of children and young adults with arterial<br />

ischemic stroke, cerebral venous sinus thrombosis, Sturge-Weber Syndrome, moya-moya or other<br />

cerebrovascular disorders. Patients are referred from all parts of Texas, Oklahoma, New Mexico,<br />

Arkansas, Louisiana and the United Kingdom.<br />

Dr. Michael Dowling<br />

Assistant Professor<br />

The Multidisciplinary Stroke Clinic sees patients twice monthly for half days. Dr. Dowling<br />

established this clinic in October 2007 and meets twice monthly with attendings from <strong>Pediatric</strong><br />

Hematology, Physical Medicine and Rehabilitation and Neuropsychology, as well as research nurses<br />

for coordinated patient visits (approximately four patients per clinic). This serves as a unique clinical<br />

and teaching program, with involvement of medical students, <strong>Pediatric</strong> Psychiatry fellows, <strong>Pediatric</strong><br />

<strong>Neurology</strong> fellows, Adult <strong>Neurology</strong> residents, and Physical Medicine and Rehabilitation fellows.<br />

The Acute Stroke Team is on call 24 / 7 / 365 for any stroke or suspected stroke in a Children’s Dallas or Legacy campus<br />

patient. Dr. Dowling provides emergent telephone consultation with in-person evaluation and treatment in the emergency<br />

setting and follows the patients clinically alongside the neurology inpatient service.<br />

<strong>Neurology</strong> Patient Visits<br />

Epilepsy 4,574<br />

General <strong>Neurology</strong> 683<br />

Headache 935<br />

Neuromuscular 1,010<br />

Neurodevelopment 832<br />

Rare Disorders 106<br />

Research 162<br />

Residents 519<br />

Stroke/Sickle Cell 260<br />

Demyelinating 268<br />

Faculty 6,871<br />

APPs 2,532<br />

Total 9,403<br />

3


Education and Training<br />

Our three-year training program for pediatric neurology is the only one in north Texas and is highly competitive. Our<br />

residency for neurodevelopmental disabilities is one of only seven in the country. We graduate three pediatric neurologists<br />

each year and offer subspecialty fellowships in epilepsy, neurophysiology, demyelinating disease, and neuromuscular<br />

medicine for our graduates as well as candidates from other programs.<br />

Dr. Rana Said, Program Director for the pediatric neurology residency and clerkship, leads the teaching program that<br />

educates residents and medical students for inpatient and outpatient rotations, with the assistance of Program Coordinator,<br />

Kellie Shaw. The program includes:<br />

• two adult neurology residents for three four-week blocks each,<br />

• seven <strong>UT</strong> <strong>Southwestern</strong> Internal Medicine residents for two months each,<br />

• one to three adult and pediatric neurophysiology/epilepsy fellows, and<br />

• one to three neuromuscular medicine fellows for one year each.<br />

More than 200 medical student neurology clerkship participants rotate for two-week intervals on either inpatient or<br />

outpatient rotations each year.<br />

The faculty participates in up to eight neurology teaching conferences per week as well as a series of pediatric teaching<br />

conferences on neurologic topics.<br />

The Carrell-Krusen Neuromuscular Symposium is held annually in February. Dr. Susan Iannaccone is the course director for<br />

this symposium designed for muscular dystrophy clinic directors, case managers, nurses and members of interdisciplinary<br />

teams. The focus is on protocols for treatment and management of both common and unusual neuromuscular disorders,<br />

identifying characteristic findings on muscle biopsies and EMG studies, and describing recent advances in basic and clinical<br />

research in neuromuscular disorders.<br />

Presentations<br />

Michael M. Dowling, M.D., Ph.D., M.S.C.S.<br />

• Ichord RN, Bastian R, Abraham L, Askalan R, Benedict S, Bernard T, Beslow L, deVeber G, Dowling M, Friedman M,<br />

Fullerton H, Jordan L, Kirton A, Amlie-Lefond C, Licht D, Lo W, McClure C, Pavlakis S, Smith SE, Tan M, Jawad AF.<br />

“Inter-observer Reliability of the <strong>Pediatric</strong> NIH Stroke Scale (PedNIHSS) in a Multicenter Study,” International Stroke<br />

Conference, Los Angeles, CA, February 2011<br />

• “PFO and Stroke in SCD,” Hematology Clinical Research Seminar, Cincinnati Children’s Hospital Medical Center,<br />

University of Cincinnati College of Medicine, OH, June 2011<br />

• “Sickle Cell in Focus,” Stroke and Cerebrovascular Complications of Sickle Cell Disease, Kings College, London, UK,<br />

June 2011<br />

• “Stroke in Children,” Texas <strong>Pediatric</strong> Society Annual Meeting Plenary Session, Sugarland, TX, September 2011<br />

4


• Dowling MM, Hynan L, Lo W, Licht D, McClure C, Yager J, Dlamini N, Kirkham F, deVeber G, Pavlakis S.<br />

“International <strong>Pediatric</strong> Stroke Study: Stroke Associated with Cardiac Disorders,” World Congress of <strong>Neurology</strong>,<br />

Marrakesh, Morocco, November 2011<br />

• Quinn CT, Hoof PD, Dowling MM. “Cerebral Tissue Desaturation in Children with Sickle Cell Disease,” American<br />

Hematology Society Annual Meeting, San Diego, CA, December 2011<br />

Patricia Evans, M.D.<br />

• “Reel Docs,” Film Series, Dr. Temple Grandin, Documentary on Autism, June 2011<br />

• “Autistic Spectrum Disorder,” University of Texas Permian Basin, July 2011<br />

Susan T. Iannaccone, M.D.<br />

• “An Overview - Recent Advances in SMA and Other <strong>Pediatric</strong> Neuromuscular Diseases,” Texas Scottish Rite Hospital<br />

for Children, Dallas, February 2011<br />

• “Standard of Care for Duchenne Muscular Dystrophy - Recent Advances in SMA and Other <strong>Pediatric</strong> Neuromuscular<br />

Diseases,” Northwestern University Medical Center, Chicago, IL, May 2011<br />

Juan M. Pascual, M.D., Ph.D.<br />

• 3 rd Symposium on Glucose Transporter Deficiency, New Orleans, LA, July 2011<br />

Rana R. Said, M.D.<br />

• “Neurological Disorders: Seizures, Headaches, Brain Tumors, Neurodegenerative and Neuromuscular Disorders,”<br />

American Academy of Child & Adolescent Psychiatry (AACAP), Honolulu, Hawaii, March 2011<br />

• “Epilepsy Management: In the Office and ER,” Children’s Medical Center at Legacy, November 2011<br />

• “Early Surgical Treatment of Infants with Catastrophic Epilepsy Symptomatic to Cortical Dysplasias,” American<br />

Academy of <strong>Neurology</strong> Annual Meeting, Honolulu, Hawaii, April 2011<br />

Awards and Honors<br />

Susan Arnold, M.D.<br />

• Best <strong>Pediatric</strong> Specialists in Dallas, D Magazine<br />

• Top US Doctors, U.S. News & World Report<br />

Michael Dowling, M.D., Ph.D., M.S.C.S.<br />

• Best <strong>Pediatric</strong> Specialists in Dallas, D Magazine<br />

Susan Iannaccone, M.D.<br />

• Best <strong>Pediatric</strong> Specialists in Dallas, D Magazine<br />

• Texas Super Doctors, Texas Monthly<br />

• Top US Doctors, U.S. News & World Report<br />

Rana Said, M.D.<br />

• Best <strong>Pediatric</strong> Specialists in Dallas, D Magazine<br />

• Child <strong>Neurology</strong> Residents’ Program Director Appreciation Award<br />

Publications<br />

Articles<br />

1. Castro D, Iannaccone ST, Gilbreath H, et al. Juvenile myasthenia gravis: a twenty year experience (33rd Annual<br />

Carrell-Krusen Neuromuscular Symposium, Texas Scottish Rite Hospital for Children, Dallas, Texas, February 17-18,<br />

2011). J Clin Neuromuscular Disease 2011;12:17:173-95.<br />

2. Choi C, Ganji SK, DeBerardinis RJ, Dimitrov IE, Pascual JM, et al. Measurement of glycine in the human brain in<br />

vivo by 1H-MRS at 3 T: application in brain tumors. Magn Reson Med;66:609-18.<br />

3. Derisavifard S, Castro C, Guleserian K, Iannaccone ST, Andersen M, Greene M. Juvenile myasthenia gravis: a twentyyear<br />

experience. <strong>Neurology</strong> 2011;76:A94 (Suppl 4).<br />

4. Gilbreath H, Iannaccone ST, Bonnemann C. Variable phenotypes in Ullrich congenital muscular dystrophy (33rd<br />

Annual Carrell-Krusen Neuromuscular Symposium, Texas Scottish Rite Hospital for Children, Dallas, Texas,<br />

February 17-18, 2011). J Clin Neuromuscular Disease 2011;12:7:173-95.<br />

5. Ichord RN, Bastian R, Abraham L, Askalan R, Benedict S, Bernard TJ, Beslow L, Deveber G, Dowling M, et al. Interrater<br />

reliability of the <strong>Pediatric</strong> National Institutes of Health Stroke Scale (PedNIHSS) in a multicenter study. Stroke;42:613-7.<br />

6. Kaufmann P, Engelstad K, Wei Y, Kulikova R, Oskoui M, Sproule DM, Battista V, Koenigsberger DY, Pascual JM, et<br />

al. Natural history of MELAS associated with mitochondrial DNA m.3243A>G genotype. <strong>Neurology</strong>;77:1965-71.<br />

7. Marin-Valencia I, Good LB, Ma Q, Jeffrey FM, Malloy CR, Pascual JM. High-resolution detection of (1)(3)C<br />

multiplets from the conscious mouse brain by ex vivo NMR spectroscopy. J Neurosci Methods;203:50-5.<br />

8. Montgomery M, Joyce C, Iannaccone ST, et al. Utilizing a national continuing medical education (CME) series to<br />

highlight emerging therapeutic opportunities in spinal muscular atrophy (SMA). <strong>Neurology</strong> 2011;76:A94 (Suppl 4).<br />

5


9. Pascual JM. Animal models of the human mind: Is there anything like being autistic Neurosci Lett;505:59-60.<br />

10. Pascual JM. Synaptic transporters are deceived if they think themselves free. Neurosci Lett;487:1-2.<br />

11. Perez-Duenas B, Ormazabal A, Toma C, Torrico B, Cormand B, Serrano M, Sierra C, De Grandis E, Marfa MP,<br />

Garcia-Cazorla A, Campistol J, Pascual JM, Artuch R. Cerebral folate deficiency syndromes in childhood: clinical,<br />

analytical, and etiologic aspects. Arch Neurol;68:615-21.<br />

12. Rogers ZR, Wang WC, Luo Z, Iyer RV, Shalaby-Rana E, Dertinger SD, Shulkin BL, Miller JH, Files B, Lane PA,<br />

Thompson BW, Miller ST, Ware RE, BABY HUG Investigators (including Dowling M). Biomarkers of splenic function<br />

in infants with sickle cell anemia: baseline data from the BABY HUG Trial. Blood;117:2614-7.<br />

13. Scharner J, Brown CA, Bower M, Iannaccone ST, et al. Novel LMNA mutations in patients with Emery-Dreifuss<br />

muscular dystrophy and functional characterization of four LMNA mutations. Hum Mutat;32:152-67.<br />

14. Sirsi D. Is intranasal midazolam better than rectal diazepam for home management of acute seizures Arch<br />

Neurol;68:120-1.<br />

15. Tang W, Gilbreath H, Burns D, Iannaccone ST. Heat or exercise-induced rhabdomyolysis in ryanodine receptor<br />

channelopathy (33rd Annual Carrell-Krusen Neuromuscular Symposium, Texas Scottish Rite Hospital for Children,<br />

Dallas, Texas, February 17-18, 2011). J Clin Neuromuscular Disease 2011;12:13:173-95.<br />

16. Wadman RI, Bosboom WM, van den Berg LH, Wokke JH, Iannaccone ST, Vrancken AF. Drug treatment for spinal<br />

muscular atrophy types II and III. Cochrane Database Syst Rev:CD006282.<br />

17. Wadman RI, Bosboom WM, van den Berg LH, Wokke JH, Iannaccone ST, Vrancken AF. Drug treatment for spinal<br />

muscular atrophy type I. Cochrane Database Syst Rev:CD006281.<br />

18. Waugh J, Plumb P, Rollins N, Dowling MM. Prolonged direct catheter thrombolysis of cerebral venous sinus<br />

thrombosis in children: a case series. J Child Neurol;27:337-45.<br />

19. Zimmerman A, Clemens PR, Tesi-Rocha C, Connolly A, Iannaccone ST, et al. Liquid formulation of pentoxifylline is<br />

a poorly tolerated treatment for duchenne dystrophy. Muscle Nerve;44:170-3.<br />

Book Sections<br />

1. Evans P. End-of-life-care for the neurologically impaired child. In: William,<br />

McGuire, Rizzo, eds. Practical Ethics in Clinical <strong>Neurology</strong>. Philadelphia, PA:<br />

American Academy of <strong>Neurology</strong>, LIppincott, Wilkins and Williams; 2011.<br />

2. Evans P. Informed assent and refusal for children and adolescents. In: William,<br />

McGuire, Rizzo, eds. Practical Ethics in Clinical <strong>Neurology</strong>. Philadelphia, PA:<br />

American Academy of <strong>Neurology</strong>, LIppincott, Wilkins and Williams; 2011.<br />

3. Evans P. Why, O God A Biblical theology of suffering and disability. In: Water L,<br />

ed. Practicing Inclusivity for Children with Special Needs. Wheaton, IL: Good News<br />

and Crossways Publishers; 2011.<br />

4. Pascual JM. Disorders of muscle excitability. In: Brady ST, Siegel GJ, Albers RW,<br />

Price DL, eds. Basic Neurochemistry. 8th ed: Elsevier; 2011.<br />

5. Pascual JM, DiMauro S. Disorders of the Krebs cycle and of pyruvate metabolism and<br />

transport. In: Rudolph CD, Rudolph AM, Lister G, First LR, Gershon AA, eds. Rudolph’s<br />

<strong>Pediatric</strong>s, 22nd edition. New York, NY: McGraw Hill; 2011.<br />

Grants<br />

Sponsored Clinical Trials<br />

Susan Arnold, M.D.<br />

Safety and Effectiveness of Open-Label Clobazam in Subjects with Lennox-Gastuat Syndrome<br />

Ovation Pharmaceuticals, OV-1004<br />

Random, Double-Blind, Placebo-Controled, Parallel-Group Study with Open-Label Ext. Phase to Evaluate Effect of<br />

Perampanel (E2007) on Cognition, Growth, Safety, Tolerability and Pharmocokinetics when Administered as Adjunctive<br />

Therapy in Adolescents (12 to


Double Blind Placebo Controlled Dose-Escalation Study of E2007 (perampanel) Given as Adjunctive Therapy in Subjects<br />

with Refractory Partial Seizures<br />

Eisai Medical Research, Inc., E2007-G000-305<br />

Susan Iannaccone, M.D.<br />

Open-Label Safety Study for Previously Treated Ataluren (PTC124) Patients with Nonsense Mutation Dystrophinopathy<br />

PTC Therapeutics, Inc., PTC124-GD-016-DMD<br />

Phase 2b Study of PTC124 in Subjects with Nonsense-Mutation-Mediated Duchenne and Becker Muscular Dystrophy<br />

PTC Therapeutics, Inc., PTC124-GD-007(e)-DMD<br />

7

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