Inflammatory Cerebrospinal Fluid in Sporadic Creutzfeldt-Jakob Disease Academic Article uri icon

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abstract

  • 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.

authors

  • Bui, Esther
  • Ehrensperger, Eric
  • Sahlas, Demetrios
  • Murray, Brian J
  • Bergeron, Catherine
  • Glikstein, Rafael S
  • Aviv, Richard
  • Schipper, Hyman M

publication date

  • November 2008