Geomedical studies on amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia complex (PDC) in the Kii Peninsula of Japan

尽管人们认为ALS的显著聚集性已经消失，但Kii半岛南部仍然是ALS/PDC的高风险地区。从1962年到1994年，在日本纪井半岛的三重县、奈良县和歌山县对ALS患者的死亡证明进行了审查。在这22年中，发现了799例ALS病例；男性472例，女性372例（男女比例为1.44:1）。这三个县经年龄调整的年死亡率在本期间结束时逐渐平均为每10万人0.9人。在此期间，平均死亡年龄从58.4岁增加到64.5岁。在三重县，死亡率从0.79略微上升到0.9；三重县的死亡率从0.5到0.89。相反，和歌山县的死亡率从1.24下降到0.93。但是，Kii半岛南部的死亡率仍然较高。在上述死亡率研究的基础上，进一步揭示了在和歌山县和三重县南部，标准化死亡率（SMR）随着地区河流中Ca和Mg含量的降低而增加，Kii半岛南部的死亡率最高，Ca和Mg含量最低。因此，死亡率与河流中钙、镁含量呈显著负相关。1980年以后，这种趋势消失了。在Kii半岛底部的大岛，南部Muro区的水井和溪流的饮用水显示出异常高的钙和镁含量。四十多年来，我们在这个岛上没有观察到任何ALS患者。在2000年1月1日最新的流行病学调查中，小川地区的粗患病率为71.5，包括1例ALS和2例PDC-ALS，表现为痴呆、帕金森症和肌萎缩，伴有病理和锥体征象。两例PDC-ALS病例均来自小川地区一个ALS家庭。在以往的调查中，我们未在该地区发现任何PDC-ALS病例。在大岛，没有出现PDC/ALS病例。与关岛南部村庄一样（Lavine et al., 1991），确定Kii半岛Kozagawa焦点地区的疾病是否持续，实际上取决于后续研究的强度，特别是需要进行挨家挨户的调查。少

Although the remarkable clustering of ALS had been thought to have disappeared, the southern Kii Peninsula remains a high-risk area for ALS/PDC. A review of death certificates of patients with ALS was conducted in the Mie, Nara and Wakayama prefectures in the Kii Peninsula of Japan, from 1962 to 1994. For these 22 years, 799 ALS cases were found ; 472 male and 372 female cases (male to female ratio : 1.44 to 1).Age-adjusted annual mortality rates of the three prefectures were gradually averaging to 0.9 per 100,000 population at the end of the period. In this period, the average age at death increased from 58.4 to 64.5 years old. In Mie prefecture, the mortality rates slightly increased from 0.79 to 0.9 ; the mortality rate in Mie prefecture from 0.5 to 0.89. On the contrary, the mortality rates in Wakayama prefecture decreased from 1.24 to 0.93. However, the mortality rates in the southern part of the Kii Peninsula still maintained higher rates.Based on the above mortality studies, it … More revealed that toward the south in Wakayama and Mie prefectures, the standardized mortality rates (SMR) were increasing with a decrease of Ca and Mg contents in area's rivers : the highest mortality rates and lowest Ca and Mg contents in the southern part of the Kii Peninsula. Thus, the mortality rates strongly and negatively correlated with the Ca and Mg contents in the rivers. After 1980, this tendency, however, disappeared. In Oshima Island at the bottom of the Kii Peninsula, the drinking water from wells and streams showed exceptionally high contents of Ca and Mg in the southern Muro district. We have observed no ALS patients in this island over forty years.In the latest prevalence survey on January 1, 2000, a crude prevalence was 71.5 in Kozagawa, including one ALS and two PDC-ALS cases, showing dementia, parkinsonism and amyotrophy with pathological and pyramidal signs. The two PDC-ALS cases were each found in an ALS family in Kozagawa area. In the previous surveys, we had not found any PDC-ALS case in this area. In Oshima Island, there have been no PDC/ALS cases.Just as in the southern village of Guam (Lavine et al., 1991), the determination of continuation of the disease in the Kozagawa focus of the Kii peninsula is really dependent on the intensity of follow-up studies, and particularly the need for house -to-house surveys. Less