Epidemiology of motoneuron diseases in Swabia

在上一个资助期间，我们成功地在德国实施了第一个肌萎缩侧索硬化症（ALS）临床流行病学登记。为了进行国际比较和未来的合作，这些工具与欧洲ALS MND组（EURALS）的工具进行了协调，超出了既定的方法，用于在中国（北京：覆盖1000万人）和蒙古（乌兰巴托：覆盖150万人）建立登记册。斯瓦比亚ALS的年龄标准化发病率为每10万人2.5（95%CI 2.3至2.7），与其他欧洲登记册的估计值一致。用捕获-再捕获方法对完整性进行了测试，估计其完整性为81%。从2010年10月1日起，ALS患者被纳入病例对照研究，并与对照受试者进行频率匹配，以探讨可能的危险因素。从大多数患者和对照受试者中收集生物材料。第一阶段的目标是实现与建立有效的结构和质量保证的过程中实施登记为基础的研究，并证实了第一publications.Recent出版物显示额颞叶痴呆（FTD）和ALS之间的相关性。为了在未来的评估中考虑该表型，FTD病例将额外记录在登记中，并将采用改良的方法。我们将对ALS登记中心斯瓦比亚与欧洲EURALS集团的其他登记中心以及中国和蒙古的合作伙伴进行比较分析。一旦达到把握度计算所要求的病例数（预计在2013年底），将在涉及生物材料的情况下对病例对照研究进行统计学评价。此外，我们将继续进行ALS疾病风险空间模式的研究。

During the previous funding period we were able to successfully implement the first clinical epidemiological register for amyotrophic lateral sclerosis (ALS) in Germany. In order to allow international comparisons and future collaborations, the instruments were coordinated with those of the European ALS MND group (EURALS), beyond the established methodology was used to a register in China (Beijing: 10 million people covered) and Mongolia (Ulanbaatar:1.5 million persons covered) establish.The age-standardized incidence of ALS in Swabia is 2.5 (95% CI 2.3 to 2.7) per 100,000 people, and is consistent with estimates from other European registries. The completeness has been tested with the capture-recapture method and is estimated to be 81%. From 1.10.2010, ALS patients were recruited in a case-control study and frequency matched to control subjects in order to explore on possible risk factors. From most patients and control subjects bio material has been collected. The objectives of the first phase were realized with the establishment of effective structures and quality assured processes for implementing register-based studies and confirmed by first publications.Recent publications show a correlation between the frontotemporal dementia (FTD) and ALS. To account for this phenotype in future evaluations, FTD cases will additionally be recorded in the register and a modified method will be applied.We will conduct a comparative analysis of the ALS Registry Swabia with other registries of European EURALS Group and the cooperation partners in China and Mongolia. Once, expected end of 2013, as required by the power calculations case numbers are achieved statistical evaluation of the case-control study will be conducted with the involvement of biological material. In addition, we will continue to pursue the study of spatial patterns of the ALS disease risk.