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Neurology 2000;55:1401-1404
© 2000 American Academy of Neurology
Brief Communications

Dementia with Lewy bodies in a neuropathologic series of suspected Creutzfeldt–Jakob disease

S. Haïk, MD, J. P. Brandel, MD, PhD, V. Sazdovitch, MD, N. Delasnerie-Lauprêtre, MD, K. Peoc’h, PharmD, J.-L. Laplanche, PhD, N. Privat, BA, C. Duyckaerts, MD, J. L. Kemeny, MD, N. Kopp, MD, A. Laquerrière, MD, M. Mohr, MD, J. P. Deslys, MD, PhD, D. Dormont, MD and J. J. Hauw, MD

From the Raymond Escourolle Neuropathology Laboratory (Drs. Haïk, Sazdovitch, Duyckaerts, and Hauw and N. Privat), Association Claude Bernard INSERM U 360 (Drs. Sazdovitch and Hauw and N. Privat, Drs. Brandel and Delasnerie-Lauprêtre) and INSERM U 106 (Dr. Duyckaerts), Pitié-Salpêtrière Hospital, Paris; Service d’Anatomie-Pathologique (Dr. Kemeny), Gabriel Montpied Hospital, Clermont-Ferrand; Laboratoire de Diagnostic des Maladies à Prion (Dr. Kopp), Pierre Wertheimer Hospital, Lyon; Laboratoire d’Anatomie et de Cytologie Pathologique (Dr. Laquerrière), Charles Nicolle Hospital, Rouen; Institut de Pathologie (Dr. Mohr), Faculté de Médecine, Strasbourg; Central Laboratory of Biochemistry (Drs. Peoc’h and Laplanche), Lariboisière Hospital, Association Claude Bernard, Paris; and Service de Neurovirologie (Drs. Haïk, Deslys, and Dormont), CEA, DSV/DRM, CRSSA, Fonntenay aux Roses, France.

Address correspondence and reprint requests to Dr. S. Haïk, Laboratoire de Neuropathologie Raymond Escourolle, Hôpital de la Salpétrière, 47 Boulevard de l’hôpital, 75013, Paris, France; e-mail: haik{at}mailhost.chups.jussieu.fr

Discriminating Creutzfeldt-Jakob disease (CJD) from dementia with Lewy bodies (DLB) may be clinically difficult to achieve. The authors describe 10 patients with DLB initially referred to the French Network of Human Spongiform Encephalopathies as having suspected CJD. In a series of 465 autopsied cases, DLB ranked second among degenerative alternative diagnoses to CJD. The authors analyzed the factors that contributed to misleading the diagnosis, and suggest that the detection of 14-3-3 protein in CSF may be useful to distinguish CJD from DLB.




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