Using data from Belgian neuropathological archives, completed with the results of a comprehensive study of available medical records, we found 100 patients who fulfilled diagnostic criteria for probable or definite Creutzfeldt-Jakob d1551isease (CJD).
Mean age at death was 63 years.
The median disease duration was 9 months.
Progressive mental deterioration was present in all cases, whereas signs of cerebellar dysfunction and myoclonus were found in approximately 80% of the patients.
In 50% of the population, the EEG revealed characteristic abnormalities.
Ninety-six patients suffered from the sporadic type of CJD, while 4 suffered from a hereditary form.
In our series, we could find no evidence for the new variant, neither for an iatrogenic cause.
Mots-clés Pascal : Encéphalopathie spongiforme Creutzfeldt Jakob, Infection, Electroencéphalographie, Prion, Registre, Epidémiologie, Incidence, Homme, Belgique, Europe, Rétrospective, Système nerveux pathologie, Système nerveux central pathologie, Encéphale pathologie, Maladie dégénérative, Electrodiagnostic
Mots-clés Pascal anglais : Creutzfeldt Jakob disease, Infection, Electroencephalography, Prion, Register, Epidemiology, Incidence, Human, Belgium, Europe, Retrospective, Nervous system diseases, Central nervous system disease, Cerebral disorder, Degenerative disease, Electrodiagnosis
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 99-0467690
Code Inist : 002B17G. Création : 22/03/2000.