title: Inflammatory cerebrospinal fluid in sporadic Creutzfeldt-Jakob disease.
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.
  
  
  METHODS
  We report two patients with pathologically-proven sporadic CJD presenting with a significant CSF pleocytosis.
  
  
  CONCLUSIONS
  Although uncommon, the presence of an inflammatory CSF profile should not exclude the diagnosis of sporadic CJD.
authors.affiliation: Division of Neurology, Department of Medicine, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada.
authors.forename: Esther
authors.initials: E
authors.lastname: Bui
authors.forename: Eric
authors.initials: E
authors.lastname: Ehrensperger
authors.forename: Demetrios J
authors.initials: DJ
authors.lastname: Sahlas
authors.forename: Brian J
authors.initials: BJ
authors.lastname: Murray
authors.forename: Catherine
authors.initials: C
authors.lastname: Bergeron
authors.forename: Rafael S
authors.initials: RS
authors.lastname: Glikstein
authors.forename: Richard
authors.initials: R
authors.lastname: Aviv
authors.forename: Hyman M
authors.initials: HM
authors.lastname: Schipper
mesh_terms: D000368:Aged; D000405:Akinetic Mutism / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D001284:Atrophy / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D001921:Brain / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D002555:Cerebrospinal Fluid / Q000166:cytology*; D007562:Creutzfeldt-Jakob Syndrome / Q000134:cerebrospinal fluid* / Q000276:immunology* / Q000503:physiopathology; D003704:Dementia / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D018450:Disease Progression; D004660:Encephalitis / Q000134:cerebrospinal fluid* / Q000276:immunology* / Q000503:physiopathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007958:Leukocyte Count; D007964:Leukocytosis / Q000276:immunology* / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D009069:Movement Disorders / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D011328:Prions / Q000378:metabolism
chemical_list: D011328:Prions