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

尽管ALS的显著聚集性被认为已经消失，但南部基伊半岛仍然是ALS/PDC的高危地区。本文回顾了1962-1994年间日本纪伊半岛三江县、奈良县和和歌山县ALS患者的死亡证明。在这22年中，发现了799例肌萎缩侧索硬化症病例，其中男性472例，女性372例(男女比例为1.44：1)。这三个县的年龄调整后的年死亡率逐渐上升到每10万人口0.9人。在此期间，平均死亡年龄从58.4岁增加到64.5岁。密州死亡率从0.79略升至0.9；密州死亡率从0.5升至0.89。相反，和歌山县的死亡率从1.24降至0.93。然而，纪伊半岛南部的死亡率仍然保持在较高的水平。根据上述死亡率研究，It…进一步揭示，在和歌山县和三叶县南部，标化死亡率(SMR)随着该地区河流中钙和镁含量的下降而增加：Kii半岛南部死亡率最高，钙和镁含量最低。因此，死亡率与河流中的钙、镁含量呈显著负相关。然而，1980年后，这一趋势消失了。在纪伊半岛底部的大岛岛，来自井水和溪流的饮用水在南部穆罗区显示出异常高的钙和镁含量。我们在岛上40多年没有观察到ALS患者。在2000年1月1日的最新患病率调查中，小泽川的粗患率为71.5，包括1例ALS和2例PDC-ALS，表现为痴呆症、帕金森症和肌萎缩，并有病理和锥体体征。这两例PDC-ALS病例分别在小泽川地区的一个ALS家系中发现。在以往的调查中，我们没有在这一地区发现任何PDC-ALS病例。在大岛岛，没有PDC/ALS病例，正如关岛南部村庄(Lavine等人，1991年)一样，确定在Kii半岛的Kozagawa疫区疾病是否继续存在实际上取决于后续研究的强度，特别是挨家挨户调查的必要性。较少

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