Inflammatory Cerebrospinal Fluid in Sporadic Creutzfeldt-Jakob Disease

BACKGROUND: Sporadic Creutzfeldt-Jakob disease (CJD) is a fatal, transmissible spongiform encephalopathy characterized by rapidly progressive dementia, myoclonus, ataxia and akinetic mutism. The underlying mechanism is believed to be a conformational change of a native prion protein which characteristically fails to provoke an immune response. Commensurate with the latter, cerebrospinal fluid (CSF) classically exhibits a non-inflammatory profile. CASES: We report two patients with pathologically-proven sporadic CJD presenting with a significant CSF pleocytosis. CONCLUSION: Although uncommon, the presence of an inflammatory CSF profile should not exclude the diagnosis of sporadic CJD.