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A 60-year-old man is referred for neurologic consultation because of rapidly progressive memory loss and jerking movements of the upper and lower extremities. Neurologic examination confirms the presence of severe cognitive deficits and memory dysfunction. An electroencephalogram shows generalized periodic sharp waves. Neuroimaging studies show moderately advanced cerebral atrophy. A cortical biopsy is obtained, which shows diffuse vacuolar changes of the gray matter with reactive astrocytosis but no significant inflammatory infiltration. CSF analysis is unremarkable. Which of the following is the most likely diagnosis?
A. Alzheimer dementia
B. Creutzfeldt-Jakob disease
C. Glioma
D. Pseudotumor cerebri
E. Viral encephalitis
 

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Rapidly progressing dementia in middle-aged patients is almost pathognomonic of Creutzfeldt-Jakob disease. The rest of the findings only come to support clinical suspicion.
 
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