Kahana E, Alter M, Feldman S
J Neurol. 1976 Jun 14;212(3):205-13. doi: 10.1007/BF00314523.
A country-wide study of the frequency of amyotrophic lateral sclerosis (ALS) was undertaken in Israel for the period 1960-1970. Israel was chosen for this study because of its excellent medical facilities and detailed demographic information. Moreover, the population includes representative groups from all parts of the world for comparison of frequency. A wide variety of motor system disease was screened in all hospitals, clinics, and chronic care facilities in the country, death certificates were reviewed and physicians with a neurological practice were contacted to derive a tentative list of cases. Only those who fit strict clinical diagnostic criteria or had autopsy confirmation were included in estimates of prevalence and incidence. On January 1, 1965, the mid-point of the study, 62 patients with ALS were living in Israel. The age-adjusted prevalence of ALS on that date was 3 per 100,000 population. The average annual age-adjusted incidence for the period 1960-1970 was 0.78 per 100000 population )0.86 in males, 0.46 in females; ratio 1.9:1). There was no appreciable change in trend of incidence over the study interval. Age-specific incidence rates were similar in native-born inhabitants of Israel, immigrants from Europe and immigrants from Afro-Asian countries. The range in age-adjusted incidence among subgroups of immigrants to Israel from various countries was 0.25 to 1.20 per 100000 population but small numbers precluded testing the statistical significance of these rather narrow differences. Mean age at onset was 55.4 years for males and 52.4 years for females. The mean age at death was 60.2 for males and 58.0 for females. The average annual mortality from ALS was 0.58 per 100000 population. There were no familial aggregates of ALS in Israel and autopsy data showed no neurofibrillary changes, granulovacuolar or inclusion bodies. There are only a few other population studies of ALS in different regions of the world. The average annual incidence in these other studies ranged from 0.4 to 1.4 per 100000 population. Thus, the incidence in Israel falls within this narrow range. The present study lends further support to the impression that ALS has a remarkably uniform geographic distribution with Guam and the Kii peninsula of Japan being the only known areas with significantly high rates. If an environmental factor contributes to the pathogenesis of ALS, the factor must also have a uniform geographic distribution.
1960年至1970年期间，以色列开展了一项关于肌萎缩侧索硬化症（ALS）发病率的全国性研究。选择以色列进行这项研究是因为其拥有优质的医疗设施和详尽的人口统计信息。此外，该国人口包含来自世界各地的代表性群体，便于比较发病率。研究人员在该国所有医院、诊所和慢性病护理机构中筛查了多种运动系统疾病，查阅了死亡证明，并联系了从事神经科诊疗工作的医生，以得出一份初步的病例清单。只有符合严格临床诊断标准或经尸检确诊的病例才被纳入患病率和发病率的估算。1965年1月1日，即研究的中点时间，以色列有62例ALS患者。当日经年龄调整后的ALS患病率为每10万人中有3例。1960年至1970年期间经年龄调整后的年均发病率为每10万人中有0.78例（男性为0.86例，女性为0.46例；男女比例为1.9:1）。在研究期间，发病率趋势没有明显变化。以色列本地居民、欧洲移民和亚非国家移民的年龄别发病率相似。来自不同国家的以色列移民亚组中，经年龄调整后的发病率范围为每10万人中有0.25至1.20例，但由于样本数量较少，无法检验这些细微差异的统计学显著性。男性发病的平均年龄为55.4岁，女性为52.4岁。男性的平均死亡年龄为60.2岁，女性为58.0岁。ALS的年均死亡率为每10万人中有0.58例。在以色列，ALS没有家族聚集性，尸检数据显示没有神经原纤维变化、颗粒空泡或包涵体。世界上其他不同地区仅有少数关于ALS的人群研究。这些其他研究中的年均发病率范围为每10万人中有0.4至1.4例。因此，以色列的发病率处于这个较窄的范围内。本研究进一步支持了以下观点：ALS具有显著一致的地理分布，关岛和日本的纪伊半岛是仅知的发病率显著较高的地区。如果环境因素促成了ALS的发病机制，那么该因素也必定具有一致的地理分布。
