Ruffié A, Delasnerie-Lauprêtre N, Brandel J P, Jaussent I, Dormont D, Laplanche J L, Hauw J J, Richardson S, Alpérovitch A
INSERM Unité 360, Hôpital de la Salpêtrière, Paris.
Rev Epidemiol Sante Publique. 1997 Dec;45(6):448-53.
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.
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.
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.
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.
动物和人类可传播性海绵状脑病的最新进展促使人们对法国及其他4个欧洲国家的克雅氏病（CJD）发病率和危险因素展开研究。
通过一个涵盖250个神经科或神经病理学实验室的全国性网络确定CJD病例。CJD病例被分类为确诊、很可能或可能病例。计算各部门的总体发病率和年龄标准化发病率。计算标准化发病比及其95%置信区间，以比较各部门CJD病例的观察数和预期数。
1992年至1995年期间，登记了216例CJD病例（平均发病率：每百万居民0.87例）。CJD病例的分布存在异质性（p < 0.007）。然而，标准化发病比的分布相当符合泊松分布。在4个部门中，CJD病例的观察数显著高于预期，在1个部门中则低于预期。
法国CJD的发病率与其他欧洲国家观察到的发病率相似。按部门分析CJD病例的分布显示出一些显著差异，这可能是由于随机波动所致。
