A 54 year old white male presents with gradual onset of mild dementia, ataxic gait, and stale 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 hypeonicity and profound dysahria. The patient dies sholy thereafter. Which of the following is the mostly likely neuropathological finding on autopsy?
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. Multiple lacunar infarcts 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 are pathognomonic for rabies, which does not cause dementia. Ref: Ropper A.H., Samuels M.A. (2009). Chapter 33. Viral Infections of the Nervous System, Chronic Meningitis, and Prion Diseases. In A.H. Ropper, M.A. Samuels (Eds), Adams and Victor's Principles of Neurology, 9e.
Cerebellar hyperplasia
Diffuse spongiform change
Multiple lacunar infarcts
Negri bodies