Gelpi Ellen, Heinzl Harald, Hoftberger Romana, Unterberger Ursula, Strobel Thomas, Voigtlander Till, Drobna Edita, Jarius Christa, Lang Susanna, Waldhor Thomas, Bernheimer Hanno, Budka Herbert
Institute of Neurology and Austrian Reference Centre for Human Prion Diseases, Medical University of Vienna, Vienna, Austria.
Neuroepidemiology. 2008;30(4):215-21. doi: 10.1159/000126915. Epub 2008 Apr 18.
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.
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.
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.
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.
朊病毒病或传染性海绵状脑病（TSEs）的明确诊断需要神经病理学检查，通常在尸检时进行。人类TSEs的流行病学调查依赖于明确诊断的病例以及缺乏神经病理学确诊的“可能”病例，这通常是因为大多数国家的尸检率较低。
1996年在奥地利建立了一项针对人类朊病毒病的主动监测计划。自那时以来，分析了900多例转诊病例。对于每一例疑似TSE病例，脑组织的尸检调查都是必需的。因此，来自奥地利的TSEs流行病学数据可为尸检率较低的国家提供以尸检为对照的参考。
自1969年以来，奥地利的TSE病例总数为206例。1996年至2006年6月30日主动监测期间的年平均死亡率为每百万人口1.39例，其中维也纳的发病率最高（2.37），高于其他省份。85%的明确TSE病例被归类为散发性克雅氏病（sCJD）。我们观察到平均死亡年龄以每年0.6岁的速度显著线性增加。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为80.5%，对“可能”的克雅氏病为38.9%。疑似病例的其他神经病理学诊断包括伴有或不伴有路易体病理改变的阿尔茨海默病、血管性脑病、代谢性脑病以及病毒性或边缘叶脑炎。
死亡率的稳步上升，尤其是在老年人群中，很可能反映了由于主动监测导致病例确诊率提高，从而使医学界的认识增强。与其他欧洲国家相比，值得欣慰的是，TSEs的总体死亡率与奥地利以尸检为对照的数据没有差异，从而证实了临床监测标准的价值。
