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.


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.
Bui
Esther
E
Division of Neurology, Department of Medicine, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada.
Ehrensperger
Eric
E
Sahlas
Demetrios J
DJ
Murray
Brian J
BJ
Bergeron
Catherine
C
Glikstein
Rafael S
RS
Aviv
Richard
R
Schipper
Hyman M
HM
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
D011328:Prions