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Kaplan Qbank USMLE



Author6 Posts
  #1

54 yr. old white male presents with gradual onset of mild dementia, ataxic gait, and startle myoclonus. MRI scan is normal, examination of CSF reveals no abnormalities, but EEG is remarkable for recurrent bursts of high-voltage slow waves. Over the next 6 months, patient's dementia rapidly worsens, accompanied by general hypertonicity and profound dysarthria. The patient dies shortly thereafter. Which of the following is the most likely neuropathological finding at autopsy?

A. Cerebellar hyperplasia

B. Diffuse spongiform change

C. Multiple lacunar infarcts

D. Negri bodies

E. Neurofibrillary tangles


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  #2

B


  #3

Creutzfeld Jacob Disease...diffuse spongiform change

  #4

B. Diffuse spongiform change


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  #5

B


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  #6

CORRECT ANSWER: B

The rapidly progressive dementia in this case is characterisitic of Creutzfeldt-Jakob disease (CJD). The dementia is usually accompanied by motor dysfunction and abnormal EEG activity. The pathological hallmark of this disease is spongiform change in gray matter. Death usually occurs within 6-12 months of disease onset.

At autopsy, the cerebellum in CJD appears atrophic, not hyperplastic (A).

Multiple lacunar infarcts (C) are seen in vascular dementia, and patients typically present with focal neurologic signs. Additionally, vascular dementia typically presents with a more gradual decline in cognitive function, measured in years rather than months.

Negri bodies are pathognomic for rabies.

Neurofibrillary tangles are seen in Alzheimer's disease. There is a more gradual decline in cognitive function, measured in years rather than months.


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