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Wernicke encephalopathy and Creutzfeldt-Jakob disease

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Abstract

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.

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Acknowledgments

We thank Prof. U. de Girolami for helpful suggestions. The contribution of the members of the French CJD Neuropathology Network and of all the physicians who referred their patients to the Network is gratefully acknowledged. InVS, DGS and DHOS supported this study.

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Authors and Affiliations

  1. APHP, Laboratoire de Neuropathologie R Escourolle, Hôpital de la Salpêtrière, Centre National de Référence ATNC (InVS), Faculté de Médecine Pierre et Marie Curie, Paris VI, 75651, Paris Cedex 13, France

    A. Bertrand, V. Sazdovitch, D. Seilhean, S. Haïk & J. J. Hauw

  2. Cellule de Référence des maladies de Creutzfeldt-Jakob, Hôpital de la Salpêtrière, 75651, Paris Cedex 13, France

    J. P. Brandel & S. Haïk

  3. INSERM, Avenir Team, Human Prion Diseases, Paris, France

    J. P. Brandel, V. Sazdovitch, B. Faucheux, N. Privat & S. Haïk

  4. Laboratoire d’Anatomie Pathologique, Hôpital Bel-Air, CHR Metz-Thionville, 57100, Thionville, France

    Y. Grignon

  5. Hôpital Louis Pasteur, BP 407, 28018, Chartres Cedex, France

    J. L. Brault

  6. Département de Neuropathologie, Hôpital Pellegrin, 33076, Bordeaux Cedex, France

    A. Vital

  7. Service d’Anatomie et de Cytologie Pathologiques, Hôpital de Rangueil, 31403, Toulouse Cedex 4, France

    E. Uro-Coste

  8. Laboratoire Central d’Anatomie et de Cytologie Pathologiques, Hôpital Robert Debré, 51092, Reims Cedex, France

    M. Pluot

  9. Service de Neurologie, CHU de Caen, 14033, Caen Cedex, France

    F. Chapon

  10. Pôle Biologie-Pathologie, CHU de Lille, 59037, Lille Cedex, France

    C. A. Maurage

  11. Département de Pathologie Cellulaire et Tissulaire, CHU d’Angers, 49933, Angers, France

    F. Letournel

  12. Service de Neurologie, Hôpital Central, 54053, Nancy Cedex, France

    H. Vespignani

  13. Service de Réanimation, Centre d’accueil et de soins hospitaliers de Nanterre, BP 1403, 92014, Nanterre Cedex, France

    G. Place

  14. Pôle tête, cou et système nerveux, Hôpital Saint Joseph, 75674, Paris Cedex 14, France

    C. F. Degos

  15. APHP, Hôpital Lariboisière, Service de biochimie et biologie moléculaire and EA 3621, Faculté de Pharmacie Paris 5, Paris, France

    K. Peoc’h

  16. Laboratoire de Neuropathologie R Escourolle, GH Pitié-Salpêtrière, 47 Bd. de l’Hôpital, 75651, Paris Cedex 13, France

    J. J. Hauw

Authors
  1. A. Bertrand
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  2. J. P. Brandel
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  3. Y. Grignon
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  4. V. Sazdovitch
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  5. D. Seilhean
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  6. B. Faucheux
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  7. N. Privat
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  8. J. L. Brault
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  9. A. Vital
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  10. E. Uro-Coste
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  11. M. Pluot
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  12. F. Chapon
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  13. C. A. Maurage
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  14. F. Letournel
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  15. H. Vespignani
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  16. G. Place
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  17. C. F. Degos
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  18. K. Peoc’h
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  19. S. Haïk
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  20. J. J. Hauw
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Corresponding author

Correspondence to J. J. Hauw.

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Bertrand, A., Brandel, J.P., Grignon, Y. et al. Wernicke encephalopathy and Creutzfeldt-Jakob disease. J Neurol 256, 904–909 (2009). https://doi.org/10.1007/s00415-009-5038-1

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  • DOI: https://doi.org/10.1007/s00415-009-5038-1

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