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Abstract Details

Case Report of Creutzfeldt-Jakob Disease Presenting as Agraphia
Aging, Dementia, and Behavioral Neurology
Behavioral and Cognitive Neurology Posters (7:00 AM-5:00 PM)
044

To describe a rare case of Creutzfeldt-Jakob Disease presenting with agraphia.

The incidence of Creutzfeldt-Jakob Disease (CJD) is 1 per million per year.   There are very few case reports of CJD associated with agraphia.  Here we present a patient with agraphia, who was eventually diagnosed with sporadic CJD.
A 75-year-old male presented to the hospital with three days of an inability to write.  He had normal verbal language (speaking and comprehension), reading, and visual fields. MRI Brain demonstrated gyriform-restricted diffusion of the bilateral parietal and left occipital lobes consistent with acute to subacute ischemia.  Further inspection revealed a “cortical ribbon” sign involving gray matter.  Basal ganglia were normal.  The patient later developed myoclonus of both arms.  EEG showed intermittent theta wave slowing; none were sharply contoured.  Over a few weeks, cognition deteriorated rapidly, and the patient became nonverbal, with depressed consciousness and increased tone of the extremities.  A lumbar puncture was performed which was positive for both 14-3-3 protein and RT-Qu1C, with T-tau protein> 4,000.  Brain biopsy was offered but the patient’s family declined.  His condition progressively worsened over the next few months, and the patient was eventually transferred to a hospice facility. 
NA

The typical presentation of Creutzfeldt-Jakob Disease is a rapidly progressive dementia with myoclonus.  While the cognitive deficits vary considerably, aphasia, forgetfulness, poor concentration, or visual disturbance are often seen.  There are only three other cases of CJD-associated agraphia reported in the literature.  For this case, we suspect the agraphia to have resulted from involvement of the angular gyrus of the left parietal lobe.
Authors/Disclosures
Miracle Wangsuwana, DO, FAAN (VA Southern Nevada)
PRESENTER 		Dr. Wangsuwana has nothing to disclose.
No disclosure on file
No disclosure on file
No disclosure on file