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From Centre National de Reference de la Maladie de Creutzfeldt-Jakob Iatrogene (Dr. Billette de Villemeur, A. Pradel, and Dr. Agid), Hopital de la Salpetriere, Paris, France; Service de Neuropediatrie (Dr. Billette de Villemeur), Hopital Necker-Enfants Malades, Paris; Laboratoire de Neuropathologie Experimentale et Neurovirologie (Drs. Deslys and Dormont), CEA Fontenay aux Roses; Centre de Pharmacovigilance (Dr. Soubrie), Hopital de la Salpetriere, Paris; INSERM U 360 (Drs. Alperovitch and Hauw), Hopital de la Salpetriere, Paris; Service de Neurologie Pediatrique (Dr. Tardieu), Hopital de Bicetre, Bicetre; Service d'Endocrinologie Pediatrique (Dr. Chaussain), Hopital Saint Vincent de Paul et Association France Hypophyse, Paris; Laboratoire de Neuropathologie R Escourolle (Dr. Hauw), Association Claude Bernard, Hopital de la Salpetriere, Paris; and INSERM U 289 (Drs. Ruberg and Agid), Hopital de la Salpetriere, Paris.
Supported in part by the French Health Ministry-Programme Hospitalier de Recherche Clinique: AOA94005.
Received January 17, 1996. Accepted in final form March 20, 1996.
Address correspondence and reprint requests to Dr. T. Billette de Villemeur, Centre National de Reference de la Maladie de Creutzfeldt-Jakob Iatrogene, INSERM U289, Hopital de la Salpetriere, 47 Boulevard de l'Hopital, 75651 Paris Cedex 13, France.
We diagnosed Creutzfeldt-Jakob disease in 34 patients (16 definite, 18 probable) who had received human growth hormone extract for various periods of time (mean +/- SD, 6.4 +/- 2.9 years), but particularly during the period between January 1984 and July 1985, a potential high-risk factor. Disease duration for deceased patients (n = 30) was 17 +/- 9 months. The clinical picture was homogeneous, starting with cerebellar ataxia and ocular motor disorders in about 90% of the patients. Neurologic deterioration, including dementia and myoclonic jerks, occurred within months. The high number of cases (1.5% of those treated between 1959 and 1988, 3% of those treated during the putative high-risk period) is still unexplained. We discuss the possibility that new cases will be detected, the risk of contaminating the general public, and the sanitary measures undertaken to prevent this.
NEUROLOGY 1996;47: 690-695
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