Creutzfeldt-Jakob disease is a low incidence progressive neurodegenerative disorder and, meanwhile, the most frequent human prion disease. We report here the case of a 65 years old female with a 2-month history of rapidly progressive dementia. The clinical examination identified patent cerebellar and extrapyramidal signs. Despite the absence of myoclonic jerks and pathological findings in T2 and FLAIR MRI, the presence of protein 14-3-3 in a significant amount in cerebrospinal fluid (CSF) was identified. The patient succumbed to the illness within 2 month of hospitalization.
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