Epidemiology of amyotrophic lateral sclerosis in Southern Germany
Epidemiology of amyotrophic lateral sclerosis in Southern Germany
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DOI：
10.1007/s00415-017-8413-3
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发表时间：
2017-04-01
影响因子：
6
通讯作者：
Nagel, Gabriele
中科院分区：
文献类型：
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作者：
Rosenbohm, Angela;Peter, Raphael S.;Nagel, Gabriele
本研究的目的是确定目前的临床表型分布，并估计未来趋势的ALS发病率在西方社会。我们报告了一项临床流行病学登记研究，在德国西南部的ALS患者中，捕获-再捕获率> 80%，并进行了基于人群的病例对照研究。共登记了1163起ALS事件。前瞻性收集了699例患者的临床和神经心理学资料。前瞻性病例（N = 699）的平均发病年龄为66.6（SD = 11.6）岁。发病部位以球结膜（34.1%）为主，其次是腰骶（30.7%）、颈（27.0%）或胸（3.1%）。经常检测到认知缺陷（范围从27.5%到42.1%，取决于筛查工具）和行为变化（29%）。79岁以后发病率明显下降，> 75岁的ALS患者更常出现延髓起病和认知功能障碍。ALS患者从首次出现轻瘫开始的平均生存时间为31个月。发现2012/2013年ALS的年龄标准化发病率（ASR）为2.4（95% CI 2.2-2.7）/100，000人-年（导致ASR为3.1/100，000，覆盖率为100%）。根据德国人口的预测年龄分布，估计到2050年，ALS的发病率男性为4.5/100，000，女性为3.3/100，000。到2050年，德国的ALS患病率将上升至约9.2-9.8/100，000人年。延髓发病和/或认知缺陷患者比例的增加可用作ALS的基本流行病学数据，用于未来的医疗保健决策。
The objective of this study is to determine the current distribution of clinical phenotypes and to estimate future trends of ALS incidence in Western societies. We report on a clinical-epidemiological registry with a capture-recapture rate of > 80% and population-based case-control study in ALS patients in South Western Germany. 1163 incidents of ALS were registered. Clinical and neuropsychological data were prospectively collected from 699 cases. The mean age at onset was 66.6 (SD = 11.6) years in prospective cases (N = 699). The site of onset was more frequently bulbar (34.1%) than lumbosacral (30.7%), cervical (27.0%), or thoracic (3.1%). Cognitive deficits (ranging from 27.5 to 42.1%, depending on the screening instrument) and behavioral changes (29%) were frequently detected. The incidence rate dropped markedly after 79 years of age, and bulbar onset as well as cognitive impairment were more frequent in ALS cases > 75 years. The mean survival time of ALS cases from first paresis was 31 months. The age-standardized incidence rate (ASR) of ALS in 2012/2013 was found to be 2.4 (95% CI 2.2-2.7) per 100,000 person-years (resulting in an ASR of 3.1/100,000 with 100% coverage). Based on the predicted age distribution of the German population, the incidence of ALS was estimated to be 4.5/100,000 for men and 3.3/100,000 for women in the year 2050. ALS prevalence will rise to about 9.2-9.8/100,000 person-years in Germany in 2050. An increased proportion of patients with bulbar onset and/or cognitive deficits can be used as basic epidemiologic data on ALS for future health care decisions.