q#18 - drtaher

[ArchivalUser]

A 54-year-old white male presents with gradual onset of mild dementia, ataxic gait, and startle
myoclonus. An MRI scan is normal, and an examination of his cerebrospinal fluid reveals no
abnormalities, but the patient's EEG is remarkable for recurrent bursts of high-voltage slow
waves. Over the next 6 months, the patient's dementia rapidly worsens, accompanied by general
hypertonicity and profound dysarthria. The patient dies shortly thereafter. Which of the
following is the mostly likely neuropathological finding on autopsy?
A. Cerebellar hyperplasia
B. Diffuse spongiform change
C. Multiple lacunar infarcts
D. Negri bodies
E. Neurofibrillary tangles

[ArchivalUser]

BBBBBBBBbbb

[ArchivalUser]

ddddd

[ArchivalUser]

DD ??

[ArchivalUser]

yeah, B

[ArchivalUser]

crozfeld jacob

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[ArchivalUser]

But rabies is faster ... dammit

[ArchivalUser]

b....

[ArchivalUser]

bb

[ArchivalUser]

The correct answer is B. The rapidly progressive dementia in this case is characteristic of
Creutzfeldt-Jakob disease (CJD). The dementia is usually accompanied by motor dysfunction and
abnormal EEG activity, as described in the question stem. The pathological hallmark of this
disease is spongiform change in the gray matter. Death usually occurs within 6-12 months of
disease onset.
At autopsy, the cerebellum in CJD appears atrophic, not hyperplastic (choice A).
Multiple lacunar infarcts (choice C) are seen with vascular dementia, and patients typically
present with focal neurological signs. Additionally, vascular dementia typically presents with
a more gradual decline in cognitive function, measured in years rather than months.
Negri bodies (choice D) are pathognomic for rabies, which does not cause dementia.
Neurofibrillary tangles (choice E) are often seen in patients with Alzheimer's disease.
Alzheimer's disease primarily affects the higher order association cortex; motor dysfunction is
not generally observed. Additionally, in Alzheimer's disease, there is a more gradual decline
in cognitive function, measured in years rather than months.