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NEUROSAE 2018 ANNUAL MEETING EDITION (VOLUME 10, ISSUE 3)
QUESTION 54 OF 100
A 50-year-old male attorney presents with a 4-month history of increasingly inappropriate behavior, including leaving the house in his pajamas, failure to groom himself normally, inability to balance the checkbook or pay bills, and most recently inability to cook or clean the house. Due to inappropriate behavior at work, he was placed on leave of absence. He also reports joint pain, fevers, chills, nausea, and diarrhea. Examination reveals the patient has poor attention and memory, a right upper quadrant field cut, slurred speech, right hemiparesis, and significant ataxia. Which of the following is the most likely diagnosis?
A. Creutzfeldt-Jakob disease
B. Wernicke’s encephalopathy
C. Herpes simplex encephalitis
D. Vascular dementia
E. Whipple disease **
** = Your answer
The patient’s clinical presentation is consistent with a rapidly progressive dementia with multiple neurologic and systemic symptoms. This neurologic presentation is possible with any of the disorders listed; however, the combination of arthralgias, fevers, and gastrointestinal symptoms, along with the neurologic decline, is most suspicious for Whipple disease due to infection with Tropheryma whippelii. Definitive diagnosis can be made with PCR testing in blood or CSF or via intestinal biopsy. Rapid identification of this disorder is important as it can be easily treated if recognized quickly.
Geschwind MD. Rapidly progressive dementia. Continuum Lifelong Learning Neurol. 2016;22(2):510-537.
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