Creutzfeldt-Jakob disease in Austria:: An autopsy-controlled study
Creutzfeldt-Jakob disease in Austria:: An autopsy-controlled study
复制标题
DOI：
10.1159/000126915
复制
发表时间：
2008-01-01
影响因子：
5.7
通讯作者：
Budka, Herbert
中科院分区：
文献类型：
--
作者：
Gelpi, Ellen;Heinzl, Harald;Budka, Herbert
背景：明确诊断朊病毒疾病或传染性海绵状脑病（tse）需要神经病理学，通常在尸检。人类热带病的流行病学依赖于缺乏神经病理学证实的明确和“可能”病例，通常是因为大多数国家尸检率低。方法：奥地利于1996年建立了人类朊病毒疾病的主动监测方案。从那时起，对900多起转诊进行了分析。在每一个疑似TSE病例中，必须对脑组织进行死后调查。因此，奥地利的tse流行病学数据可作为尸检率较低的国家的尸检对照参考。结果：奥地利自1969年以来的TSE病例总数为206例。在1996年至2006年6月30日的积极监测期间，年平均死亡率为1.39‰，维也纳的死亡率最高（2.37‰），高于其他省份。85%的确诊tse归为散发性克雅氏病（sCJD）。我们观察到平均死亡年龄呈显著的线性增长，每日历年增加0.6岁。临床诊断监测标准对可能的克雅氏病的敏感性和特异性分别为82.7%和80.0%，对可能的克雅氏病的阳性预测值分别为80.5%和38.9%。可疑病例的其他神经病理学诊断包括阿尔茨海默病伴或不伴路易体病理、血管性脑病、代谢性脑病和病毒性或边缘脑炎。结论：死亡率的稳步上升，特别是在老年群体中，很可能反映了由于积极监测而提高了医学界的认识，从而改善了病例确定。与其他欧洲国家相比，值得欣慰的是，热脑病的总死亡率与奥地利尸检控制数据没有差异，从而证实了临床监测标准的价值。版权所有2008 S. Karger AG，巴塞尔
Background: Definite diagnosis of prion diseases or transmissible spongiform encephalopathies (TSEs) requires neuropathology, usually at autopsy. Epidemiology of human TSEs has relied on definite as well as 'probable' cases in which neuropathological confirmation is lacking, usually because of low autopsy rates in most countries. Methods: In Austria, an active surveillance program for human prion diseases was established in 1996. Since then, more than 900 referrals were analyzed. Postmortem investigation of brain tissue is mandatory in every suspect case of TSE. Thus, epidemiological data on TSEs from Austria may serve as autopsy-controlled reference for countries with lower autopsy rates. Results: The total number of TSE cases in Austria since 1969 is 206. The average yearly mortality for the active surveillance period from 1996 to 30 June 2006 is 1.39 per million, with the highest rates for Vienna (2.37) compared with other provinces. Eighty-five percent of definite TSEs were classified as sporadic Creutzfeldt-Jakob disease (sCJD). We observed a significant linear increase in the mean age at death of 0.6 years per calendar year. Clinical diagnostic surveillance criteria had a sensitivity and specificity of 82.7 and 80.0% for probable CJD, respectively, and a positive predictive value of 80.5% for probable and 38.9% for `possible' CJD. Alternative neuropathological diagnoses in suspect cases included Alzheimer's disease with or without Lewy body pathology, vascular encephalopathy, metabolic encephalopathies and viral or limbic encephalitis. Conclusion: The steady increase in mortality rates, especially in old age groups, most likely reflects improved case ascertainment due to active surveillance causing higher awareness of the medical community. In comparison with other European countries, it is reassuring to note that the overall death rate of TSEs does not differ from the Austrian autopsy-controlled data, thus confirming the value of clinical surveillance criteria. Copyright (C) 2008 S. Karger AG, Basel.