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Creutzfeldt-Jakob disease (CJD) is a rare, untreatable cause of rapidly progressive dementia. WHO diagnostic criteria, last updated in 1998, include periodic sharp waves on electroencephalography and elevated 14-3-3 protein in cerebrospinal fluid (CSF). Both of these criteria have been scrutinized, given limited sensitivity and specificity across case series. For the present study, researchers examined CSF samples from 420 patients for whom CJD was considered in the differential diagnosis and who subsequently underwent autopsy during a 48-month period.
CJD was neuropathologically confirmed in 58.3% of cases. CSF 14-3-3 protein, assessed by Western blot, was reported as negative, positive, or ambiguous. CSF tau was measured by enzyme-linked i…