Hostname: page-component-586b7cd67f-rdxmf Total loading time: 0 Render date: 2024-11-28T15:32:29.390Z Has data issue: false hasContentIssue false
  • English
  • Français

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.
Rights & Permissions [Opens in a new window]

Abstract

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.
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

References

1. Oesch, B, Westaway, D, Walchli, M, McKinley, MP, Kent, SB, Aebersold, R, et al. A cellular gene encodes scrapie PrP 27-30 protein. Cell. 1985; 40:735–46.Google Scholar
2. Prusiner, SB. Prions . Sci Am. 1984; 251:50–9.Google Scholar
3. Parchi, P, Giese, A, Capellari, S, Brown, P, Schulz-Schaeffer, W, Windl, O, et al. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol. 1999; 46:224–33.Google Scholar
4. Zeidler, M. WHO manual for strengthening diagnosis and surveillance of Creutzfeldt-Jakob disease: World Health Organization: 1988. p. 47–51.Google Scholar
5. Kretzschmar, HA, Ironside, JW, DeArmond, SJ, Tateishi, J. Diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Arch Neurol. 1996; 53:913–20.Google Scholar
6. Demaerel, P, Sciot, R, Robberecht, W, Dom, R, Vandermeulen, D, Maes, F, et al. Accuracy of diffusion-weighted MR imaging in the diagnosis of sporadic Creutzfeldt-Jakob disease. J Neurol. 2003; 250:222–5.Google Scholar
7. Meissner, B, Kortner, K, Bartl, M, Jastrow, U, Mollenhauer, B, Schroter, A, et al. Sporadic Creutzfeldt-Jakob disease: magnetic resonance imaging and clinical findings. Neurology. 2004; 63:450–6.Google Scholar
8. Shiga, Y, Miyazawa, K, Sato, S, Fukushima, R, Shibuya, S, Sato, Y, et al. Diffusion-weighted MRI abnormalities as an early diagnostic marker for Creutzfeldt-Jakob disease. Neurology. 2004; 63: 443–9.Google Scholar
9. Haywood, AM. Transmissible spongiform encephalopathies. N Engl J Med. 1997; 337:1821–8.Google Scholar
10. Johnson, RT, Gibbs, CJ Jr. Creutzfeldt-Jakob disease and related transmissible spongiform encephalopathies. N Engl J Med. 1998; 339:1994–2004.Google Scholar
11. Mendez, OE, Shang, J, Jungreis, CA, Kaufer, DI. Diffusion-weighted MRI in Creutzfeldt-Jakob disease: a better diagnostic marker than CSF protein 14-3-3? J Neuroimaging. 2003; 13:147–51.Google Scholar
12. Na, DL, Suh, CK, Choi, SH, Moon, HS, Seo, DW, Kim, SE, et al. Diffusion-weighted magnetic resonance imaging in probable Creutzfeldt-Jakob disease: a clinical-anatomic correlation. Arch Neurol. 1999; 56:951–7.Google Scholar
13. Shinobu, LA, Frosch, MP. Case records of the Massachusetts General Hospital. Case 28-1999. A 68-year-old woman with rapidly progressive dementia and a gait disorder. N Engl J Med. 1999; 341:901–8.Google Scholar
14. Zeidler, M, Green, A. Advances in diagnosing Creutzfeldt-Jakob disease with MRI and CSF 14-3-3 protein analysis. Neurology. 2004; 63:410–1.Google Scholar
15. Jacobi, C, Zerr, I, Arlt, S, Schröter, A, Otto, M, Poser, S. Cerebral spinal fluid pattern in patients with definite Creutzfeldt-Jakob disease. J Neurol. 2000; 247 Suppl 3:III/14.Google Scholar
16. Goldhammer, Y, Bubis, JJ, Sarova-Pinhas, I, Braham, J. Subacute spongiform encephalopathy and its relation to Jakob-Creutzfeldt disease: report on six cases. J Neurol Neurosurg Psychiatry. 1972; 35:1–10.Google Scholar
17. Poser, S, Mollenhauer, B, Kraubeta, A, Zerr, I, Steinhoff, BJ, Schroeter, A, et al. How to improve the clinical diagnosis of Creutzfeldt-Jakob disease. Brain. 1999; 122 (Pt 12):2345–51.Google Scholar
18. Rowland, LP. Merritt’s neurology. Philadelphia: Lippincott Williams & Wilkins; 2000.Google Scholar
19. Obi, T, Takatsu, M, Kitamoto, T, Mizoguchi, K, Nishimura, Y. [A case of Creutzfeldt-Jakob disease (CJD) started with monoparesis of the left arm]. Rinsho Shinkeigaku. 1996; 36:1245–8.Google Scholar
20. Panagariya, A, Jain, RS, Sharma, AK. Stroke like presentation of Creutzfeldt Jakob disease: an unusual variant. J Assoc Physicians India. 1999; 47:548–50.Google Scholar