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Inflammatory Cerebrospinal Fluid in Sporadic Creutzfeldt-Jakob Disease

Published online by Cambridge University Press:  02 December 2014

Esther Bui ,
Eric Ehrensperger ,
Demetrios J. Sahlas ,
Brian J. Murray ,
Catherine Bergeron ,
Rafael S. Glikstein ,
Richard Aviv  and
Hyman M. Schipper
Esther Bui
Affiliation:
Division of Neurology, Department of Medicine, University of Toronto, Toronto, Ontario
Eric Ehrensperger
Affiliation:
Department of Neurology & Neurosurgery, Sir Mortimer B. Davis Jewish General Hospital, McGill University, Montreal, Quebec, Canada
Demetrios J. Sahlas
Affiliation:
Division of Neurology, Department of Medicine, University of Toronto, Toronto, Ontario
Brian J. Murray
Affiliation:
Division of Neurology, Department of Medicine, University of Toronto, Toronto, Ontario
Catherine Bergeron
Affiliation:
Department of Laboratory Medicine and Pathobiology, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario
Rafael S. Glikstein
Affiliation:
Department of Radiology, Sir Mortimer B. Davis Jewish General Hospital, McGill University, Montreal, Quebec, Canada
Richard Aviv
Affiliation:
Division of Neuroradiology, Department of Medical Imaging, University of Toronto, Toronto, Ontario
Hyman M. Schipper*
Affiliation:
Department of Neurology & Neurosurgery, Sir Mortimer B. Davis Jewish General Hospital, McGill University, Montreal, Quebec, Canada
*
Lady Davis Institute for Medical Research, Sir Mortimer B. Davis Jewish General Hospital, 3755 Cote St. Catherine Road, Montreal, Quebec, H3T 1E2, Canada.
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Abstract

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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.

Résumé:

RÉSUMÉ:Contexte:

La maladie de Creutzfeldt-Jakob (MCJ) est une encéphalopathie spongiforme transmissible qui est fatale et qui se caractérise par une démence rapidement progressive, du myoclonus, de l'ataxie et un mutisme akinétique. Le mécanisme sous-jacent est, croit-on, un changement de conformation d'une protéine prion native qui ne provoque donc pas de réponse immunitaire. Le liquide céphalorachidien (LCR) a un profil non inflammatoire chez ces patients.

Observations:

Nous rapportons les observations faites chez deux patients, dont la MCJ a été prouvée en anatomopathologie, qui présentaient une pleiocytose importante du LCR.

Conclusion:

Bien que rare, la présence d'un profil inflammatoire du LCR ne devrait pas exclure le diagnostic de MCJ sporadique.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 35 , Issue 5 , November 2008 , pp. 625 - 629
Copyright
Copyright © The Canadian Journal of Neurological 2008

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