title: The French surveillance network of Creutzfeldt-Jakob disease. Epidemiological data in France and worldwide.
abstract: France, involved for a long time in the epidemiological surveillance of transmissible spongiform encephalopathy (TSE), created a national network of surveillance in 1991, because of the description of the first cases of Creutzfeldt-Jakob disease (CJD) linked to a treatment by growth hormone of human origin and the observation of cases of cats infected with the agent of the bovine spongiform encephalopathy in the United Kingdom (UK). The French surveillance network is integrated into the European network of surveillance since its creation in 1993. As in other countries, sporadic CJD is the most frequent form of TSE in France with an annual mortality rate of 1.44 per million. Genetic forms are most often associated with a mutation at codon 200. Among the cases of iatrogenic CJD, 13 cases of CJD after duramater grafts were observed and 119 related to treatment with growth hormone. France is the country worst affected in Europe and the world by this latter form, before the USA and UK. Since 1996, 27 cases of variant of CJD (vCJD) has been observed, making France the second country in the world most affected after the UK. No cases of transfusion-associated vCJD have been observed.
authors.affiliation: Cellule nationale de référence des maladies de Creutzfeldt-Jakob, groupe hospitalier Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France; Inserm, UMR-S-975, équipe maladie d'Alzheimer-Maladies à prions, Paris, France; CNRS, UMR 7225, Paris, France; Centre de recherche de l'institut du cerveau et de la moelle épinière (CRICM), UMRS 975, équipe Alzheimer's and Prion Diseases, université Pierre-et-Marie Curie-Paris 06, Paris, France. Electronic address: jean-philippe.brandel@psl.aphp.fr.
authors.forename: J-P
authors.initials: JP
authors.lastname: Brandel
authors.forename: L
authors.initials: L
authors.lastname: Peckeu
authors.forename: S
authors.initials: S
authors.lastname: Haïk
mesh_terms: D000818:Animals; D058070:Asymptomatic Diseases; D002353:Carrier State; D002371:Cat Diseases / Q000453:epidemiology / Q000635:transmission; D002415:Cats; D002417:Cattle; D003062:Codon / Q000235:genetics; D007562:Creutzfeldt-Jakob Syndrome / Q000453:epidemiology* / Q000235:genetics / Q000635:transmission; D004340:Drug Contamination; D004388:Dura Mater / Q000637:transplantation; D016643:Encephalopathy, Bovine Spongiform / Q000453:epidemiology; D005506:Food Contamination; D005602:France / Q000453:epidemiology; D014943:Global Health; D019382:Human Growth Hormone / Q000009:adverse effects / Q000302:isolation & purification; D006801:Humans; D007049:Iatrogenic Disease; D008460:Meat / Q000009:adverse effects; D011159:Population Surveillance; D011183:Postoperative Complications / Q000453:epidemiology; D015995:Prevalence; D017096:Prion Diseases / Q000635:transmission / Q000662:veterinary; D000072002:Prion Proteins; D011328:Prions / Q000235:genetics; D065227:Transfusion Reaction; D006113:United Kingdom / Q000453:epidemiology; D015047:Zoonoses
chemical_list: D003062:Codon; C512656:PRNP protein, human; D000072002:Prion Proteins; D011328:Prions; D019382:Human Growth Hormone
keywords: Creutzfeldt–Jakob disease; Encéphalopathie spongiforme transmissible; Epidemiology; Genetic; Génétique; Iatrogenic; Iatrogène; Maladie de Creutzfeldt–Jakob; Réseau de surveillance; Sporadic; Sporadique; Surveillance network; Transmissible spongiform encephalopathy; vCJD; vMCJ; Épidémiologie