American Academy of Neurology Guideline on sCJD Diagnosis

Sporadic Creutzfeldt-Jakob disease (sCJD) affects just one in a million people and most often presents as rapidly progressive dementia with variable associated symptoms affecting motor, sensory, cerebellar, and visual systems. The protean manifestations of the degenerative brain disease and the lack of standardized ancillary testing render definitive clinical diagnosis difficult, yet concern about infectivity of samples from patients with sCJD makes diagnosis particularly important. Diagnostic criteria have historically included electroencephalography (EEG) and cerebrospinal fluid (CSF) features coexistent with the dementia. However, recent data have raised doubts about the sensitivity and specificity of CSF 14-3-3 protein. In addition, som…