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A BSTRACTS
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A22
Aphasia vs. Dementia: A Case of Primary Progressive Aphasia.
S. S. Naqvi, C. D. Furman. Family & Geriatric Medicine, University of
Louisville, Louisville, KY.
Introduction: Primary Progressive Aphasia (PPA) presents with
dysnomia and impaired speech. Language dysfunction remains the
most salient feature and deteriorates most rapidly during the illness.
Impairments in other cognitive functions, including dementia, may
also emerge.
Case Report: A 70 y/o white male with h/o bipolar disorder, hallucinations,
hyperlipidemia, and PPA presented with his wife and son
to determine if he had dementia. Patient also had a h/o stuttering as a
child and alcoholism. Patient had memory problems the past 2 years
with a rapid decline over past 2-3 weeks. Patient did not recognize his
own home or wife and often wore wife’s clothes. He had difficulty
swallowing and eating. He developed poor coordination skills and
found it hard to place food into his mouth. In 2003, he retired as an attorney.
His symptoms began in 1999 with speech problems. He had
difficulty with expression of language, incomplete sentences, spelling
errors and word loss without compensation. In 2003, he was diagnosed
with PPA and was seen by multiple local and national neurologists
and neuropsychologists who unanimously agreed on a diagnosis
of PPA. Brain PET scans, done in 2006 and 2010, showed hypometabolism
in the left temporoparietal region, compatible with a neurodegenerative
disorder, most likely Alzheimer’s type. The patient was on
donepezil and memantine with no improvement and received speech
therapy with some improvement. At the current presentation, dementia
was diagnosed based on his memory loss and functional decline.
He was admitted to hospice because of dysphagia and his overall
decline.
Discussion:This case exemplifies affluent type PPA,a rare neurodegenerative
disorder in which symptoms start with language difficulty. Patients
with PPA can become mute and may eventually lose the ability to
understand written or spoken language. PPA is considered a form of dementia
because it causes a gradual cognitive decline to the point where
activities of daily living become compromised. Approximately 30% of
patients have shown the microscopic pathology of Alzheimer’s disease,
presumably with an atypical distribution of lesions. 70% of PPA is known
to be a type of frontotemporal lobar degeneration. Currently, there is no
effective pharmacologic treatment. However, evaluation by a speech
therapist is useful. When PPA is diagnosed, the patient and family should
be informed that PPA is a type of progressive dementia.
A23
Aspergilloma: association with chronic bronchiectasis and IgG2
deficiency.
T. T. Suh, 1,2 T. Efeovbokhan, 1,2 M. R. Hosler. 3 1. Family &
Community Medicine, UT Health Science Center in San Antonio, San
Antonio, TX; 2. ACE Unit, Christus Santa Rosa Hospital-City Centre,
San Antonio, TX; 3. Allergy, Asthma & Immunology Associates of
South Texas, San Antonio, TX.
BACKGROUND: Aspergilloma is fungal lung infection that
often causes cavitary lung lesions and the characteristic “fungus ball”
or mycetoma on CT scan. Up to 10% of patients with bronchiectasis
eventually become infected with aspergillus. It is thought that high
levels of complement found in patients with bronchiectasis prevent
the killing of aspergillus, allowing this fungal infection to occur. Up to
half of patients with idiopathic bronchiectasis have deficiency of either
IgA or IgG2 antibodies. Aspergilloma can also be a very rare
complication of etanercept for rheumatologic conditions like
rheumatoid arthritis, affecting about 0.01% of patients reporting side
effects with this drug.
CASE: A 75 year old Caucasian woman with multiple drug allergies,
BMI of 19.5, chronic bronchiectasis, rheumatoid arthritis
(being treated with etanercept and prednisone 5mg daily), an IgG2
subclass deficiency (being treated with monthly IVIg) and a functional
humoral immunodeficiency (with poor response to Pneumovax)
presented to her allergist with hemoptysis for one week. A
contrast-enhanced CT scan of the chest revealed a cavitary right
upper lobe lung lesion. The patient was hospitalized, and bronchoscopy
was performed. Tuberculosis and cancer were ruled out
from bronchial washings and biopsies, but results were suggestive for
aspergilloma. Because the patient was not thought to be a good surgical
candidate, she was placed on IV voriconazole for 4 weeks. After
completing this course of antifungal therapy, her hemoptysis resolved
and overall respiratory status improved.
DISCUSSION: Aspergilloma is a generally rare fungal infection
that can be seen in patients who are immunosuppressed. It is important
for geriatricians to be aware that aspergilloma is associated with
chronic bronchiectasis. In turn, chronic bronchiectasis is associated
with IgG2 deficiency that can be treated with IVIg. In particular,
geriatricians should also note that non-CF-related bronchiectasis is
more common and more severe in underweight Caucasian women
over the age of 60 years.
A24
CADASIL Disease: a case report of a rare, genetic early onset
dementia.
T. Leucker, 1 B. Setters. 2 1. Internal Medicine, University of Louisville,
Louisville, KY; 2. Family & Geriatric Medicine, University of
Louisville, Louisville, KY.
INTRODUCTION: Cerebral autosomal dominant arteriopathy
with subcortical infarcts and leukoencephalopathy (CADASIL), a
genetically determined arteriopathy involving the NOTCH 3 gene on
chromosome 19, is a rare cause of vascular dementia affecting only a
few hundred families worldwide. Clinical progression involves the sequential
development of migraine with aura at age 30, followed by ischemic
strokes, mood disorders then dementia at 50 yo and gait difficulty
at 60.
CASE REPORT: A 60 y/o AAF with a 40 year history of recurrent
headaches with nausea/vomiting, light sensitivity, worsening
memory loss and depression presented to the hospital 2 years ago
with a blurry vision, a lazy eye and left sided weakness. Exam on admission
did not reveal any neurological deficits, but given her extensive
family history of stroke and personal history of persistent symptoms
over the past few months, a full evaluation was completed. A
head CT demonstrated subtle areas of abnormal attenuation. Follow
up MRI identified diffuse hyper-intensities, heaviest in the temporal
lobes in a subcortical location involving white matter, which was very
concerning for a possible diagnosis of CADASIL disease given the
patient’s history. The remaining clinical workup, including lumbar
puncture was negative. The patient was offered genetic testing for
CADASIL disease, but due to lack of insurance no further testing was
done. Since this initial evaluation the patient had two more admission
for stroke like symptoms. She has shown progressive decline in cognitive
function including memory loss, aphasia, apraxia and a disturbance
in executive functioning resulting in a loss of her job and dependence
on her family for care with her daily activities.
CONCLUSION: CADASIL disease is a rare genetic cause of
dementia that can affect people of all ages and as such is not part of
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