Wernicke encephalopathy and Creutzfeldt-Jakob disease

• Published in: Journal of neurology Vol. 256; no. 6; pp. 904 - 909
• Main Authors: Bertrand, A., Brandel, J. P., Grignon, Y., Sazdovitch, V., Seilhean, D., Faucheux, B., Privat, N., Brault, J. L., Vital, A., Uro-Coste, E., Pluot, M., Chapon, F., Maurage, C. A., Letournel, F., Vespignani, H., Place, G., Degos, C. F., Peoc’h, K., Haïk, S., Hauw, J. J.
• Format: Journal Article
• Language: English
• Published: Heidelberg D. Steinkopff-Verlag 01.06.2009; Springer; Springer Nature B.V
• Subjects: 14-3-3 Proteins - cerebrospinal fluid; Adult; Aged; Aged, 80 and over; Ataxia; Autopsies; Biological and medical sciences; Blood vessels; Brain - pathology; Creutzfeldt-Jakob disease; Creutzfeldt-Jakob Syndrome - diagnosis; Creutzfeldt-Jakob Syndrome - epidemiology; Creutzfeldt-Jakob Syndrome - pathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Dementia; Diagnosis, Differential; Humans; Iatrogenesis; Medical sciences; Medicine; Medicine & Public Health; Middle Aged; Myoclonus - epidemiology; Myoclonus - pathology; Neurology; Neuropathology; Neuroradiology; Neurosciences; Original Communication; Prevalence; Proteins; Registries; Time Factors; Wernicke Encephalopathy - diagnosis; Wernicke Encephalopathy - epidemiology; Wernicke Encephalopathy - pathology; Young Adult; France; Paris France; Diagnosis of dementia; Creutzfeldt-Jakob; Wernicke encephalopathy; Prion disease; Nervous system diseases; Creutzfeldt-Jakob disease; Cerebral disorder; Infection; Central nervous system disease; Degenerative disease; Prion; Diagnosis; Dementia
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-009-5038-1

We assessed the prevalence of Wernicke encephalopathy (WE) in all 657 cases suspected of Creutzfeldt-Jakob (CJD) referred from 2001 to 2006 to the French Neuropathology Network of CJD. Clinical, biological and imaging data were reviewed when the diagnosis of WE was made at autopsy. No CJD was found in five cases suspected of sporadic CJD. In these five cases, myoclonus had been observed in four, CSF 14-3-3 protein in two. In 14 other cases, WE was combined with CJD, 13 of which were sporadic. These belonged mainly to the molecular variants of sporadic CJD associated with a long duration of disease. This stresses the necessity of remaining alert to the diagnosis of WE when CJD is suspected.