Objectives-To find and investigate, retrospectively, as many cases as possible of Creutzfeldt-Jakob disease (CJD) in Sweden dying during the period 1 January 1985 to 31 December 1996 and to detect any possible case (s) of new variant CJD.
Methods-The patients were found through computer search of all death certificates in Sweden on which CJD was mentioned, through information from the Swedish neuropathologists, and spontaneous reports from Swedish doctors and hospitals.
Data concerning the patients were then collected from patients'case records and from brain histopathology reports.
In total 72 cases of spongiform encephalopathy were confirmed as definite by neuropathology, one of them with Gerstmann-Stäussler-Scheinker disease.
In 51 further cases there were no brain pathology data but the diagnosis « probable » (37 patients) or « possible » (14 patients) CJD according to WHO criteria could be made on clinical grounds.
There was a variation in number of deaths/year, from a minimum of five (1985) to a maximum of 16 (1990).
Sixty patients died during the period 1985-90 and 62 during 1991-6.
The sex ratio was nearly 1 : 1. Calculated for a population of 8.6 million (mean of 12 years) in Sweden this gives 1.18/million/year.
Age at the time of the presenting symptoms ranged from 34 to 84 years.
Only one patient was under 40 at the onset of symptoms.
He had a spongiform encephalopathy but prion protein staining was negative. (...)
Mots-clés Pascal : Encéphalopathie spongiforme Creutzfeldt Jakob, Infection, Suède, Europe, Age, Symptomatologie, Forme clinique, Incidence, Epidémiologie, Homme, Prion, Système nerveux pathologie, Système nerveux central pathologie, Encéphale pathologie, Maladie dégénérative
Mots-clés Pascal anglais : Creutzfeldt Jakob disease, Infection, Sweden, Europe, Age, Symptomatology, Clinical form, Incidence, Epidemiology, Human, Prion, Nervous system diseases, Central nervous system disease, Cerebral disorder, Degenerative disease
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 99-0038887
Code Inist : 002B17G. Création : 31/05/1999.