[Incidence of Creutzfeldt-Jakob disease in France, 1992-1995].
BACKGROUND
Recent developments in animal and humans transmissible spongiform encephalopathies have motivated a study on incidence and risk factors of Creutzfeldt-Jakob disease (CJD) in France and 4 other European countries.


METHODS
CJD cases were ascertained through a national network including 250 neurological departments or neuropathological laboratories. CJD cases were classified as definite, probable or possible. Overall incidence rate and age-standardized incidence rates by department were computed. Standardized incidence ratios and their 95% confidence intervals were computed for comparing observed and expected number of CJD cases in each department.


RESULTS
Between 1992 and 1995, 216 CJD cases were registered (mean incidence rate: 0.87 per million inhabitants). The distribution of CJD cases was heterogeneous (p < 0.007). Nevertheless, the distribution of standardized incidence ratios fitted quite well a Poisson distribution. The observed number of CJD cases was significantly higher than expected in 4 departments and lower in 1 department.


CONCLUSIONS
Incidence of CJD in France is similar to that observed in other European countries. Analysis of distribution of CJD cases by department showed a few significant differences which can be due to random fluctuations.
Ruffié
A
A
INSERM Unité 360, Hôpital de la Salpêtrière, Paris.
Delasnerie-Lauprêtre
N
N
Brandel
J P
JP
Jaussent
I
I
Dormont
D
D
Laplanche
J L
JL
Hauw
J J
JJ
Richardson
S
S
Alpérovitch
A
A
D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D007562:Creutzfeldt-Jakob Syndrome / Q000145:classification / Q000453:epidemiology* / Q000635:transmission; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D016012:Poisson Distribution; D011159:Population Surveillance; D012042:Registries; D012307:Risk Factors
Incidence de la maladie de Creutzfeldt-Jakob en France, 1992-1995.