NR AEPJ
AU Giraud,P.; Alperovitch,A.; Chazot,G.
TI [Creutzfeld-Jakob disease]
OT Maladies de Creutzfeldt-Jakob
QU Revue du Praticien 1999 May 1; 49(9): 948-53
PT journal article; review; review, tutorial
AB Described in the early 1920s in Germany, Creutzfeldt-Jakob disease now comprises 4 entities: 1. familial forms are linked to mutations of the gene coding for prion protein (about 8% of the cases); 2. iatrogenic forms are due to interventions using contaminated material or human extracts (about 5% of the cases); 3. sporadic forms represent the majority of the cases (about 87%) without any established explanation; 4. recently described new variant, related to bovine spongiform encephalopathy, remains quite rare (41 known cases). In all its forms, the disease is constantly mortal after a usually short course. Major signs are rapidly evolving; dementia with myoclonus, ataxia, and electroencephalographic abnormalities (periodic activity). Formal diagnosis is histologic.
ZR 34
MH Animal; Ataxia/etiology; Cattle; Creutzfeldt-Jakob Syndrome/diagnosis/*pathology/transmission; Dementia/*etiology; Diagnosis, Differential; Electroencephalography; Encephalopathy, Bovine Spongiform/*transmission; English Abstract; *Genetic Predisposition to Disease; Human; Iatrogenic Disease; Myoclonus/etiology; Sheep
AD Service de neurologie, hopital neurologique et neuro-chirurgical Pierre Wertheimer 69394 Lyon.
SP französisch
PO Frankreich