Amyotrophic lateral sclerosis and parkinsonism-dementia on Guam: a 25-year prospective case-control study.
Amyotrophic lateral sclerosis and parkinsonism-dementia on Guam: a 25-year prospective case-control study.
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关岛的肌萎缩侧索硬化症和帕金森痴呆症：一项为期 25 年的前瞻性病例对照研究。
DOI：
10.1093/oxfordjournals.aje.a114437
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发表时间：
1986
影响因子：
5
通讯作者：
D. Gajdusek
中科院分区：
文献类型：
--
作者：
C. Plato;R. Garruto;K. Fox;D. Gajdusek
关岛查莫罗人高发肌萎缩侧索硬化症和帕金森痴呆症的家族和遗传学研究始于1958年，建立了一个前瞻性病例对照登记系统。这项登记的主要目的是确定肌萎缩侧索硬化症或帕金森氏痴呆患者的一级亲属和配偶是否与年龄、性别和村庄匹配的未受影响的对照组的亲属相比，发生疾病的风险增加。在1963年关闭时，登记包括126名患者(77名肌萎缩侧索硬化症患者，42名帕金森氏痴呆患者，7名同时患有肌萎缩侧索硬化症和帕金森痴呆症的患者)和同等数量的对照组；994名患者的在世一级亲属(父母、兄弟姐妹和子女)和1218名对照组；88名患者的在世配偶和101名对照组。目前对25年随访研究(1958-1983)的分析表明，在患者的父母、兄弟姐妹和配偶中，发生肌萎缩侧索硬化症或帕金森氏痴呆的风险显著增加，但在对照的亲属中没有。患者和对照组的后代患疾病的风险都没有显著增加。患者配偶的风险增加，而他们的子女的风险没有增加，再加上最近的组织化学研究结果，支持了外源性因素是肌萎缩侧索硬化症和帕金森痴呆症的病因的强大贡献者的观点。目前的结果还表明，这两种疾病的发病率都在下降。
Familial and genetic studies of high-incidence amyotrophic lateral sclerosis and parkinsonism-dementia among the Chamorro people of Guam were initiated in 1958 with the establishment of a prospective case-control registry. The major objective of this registry was to determine if first-degree relatives and spouses of patients with amyotrophic lateral sclerosis or parkinsonism-dementia had an increased risk of developing disease compared with relatives of nonaffected controls individually matched for age, sex, and village. At the time of its closing in 1963, the registry included 126 patients (77 with amyotrophic lateral sclerosis, 42 with parkinsonism-dementia, and seven with both amyotrophic lateral sclerosis and parkinsonism-dementia) and an equal number of controls; 994 living first-degree relatives (parents, siblings, and offspring) of patients and 1,218 of controls; and 88 living spouses of patients and 101 of controls. The present analysis of the 25-year follow-up study (1958-1983) demonstrated a significantly increased risk of developing amyotrophic lateral sclerosis or parkinsonism-dementia among parents, siblings, and spouses of patients, but not among relatives of controls. Offspring of both patients and controls showed no significantly increased risk of developing disease. The increased risk among spouses of patients and the lack of increase among their offspring, together with recent histochemical findings, support the contention that exogenous factors are strong contributors to the etiology of amyotrophic lateral sclerosis and parkinsonism-dementia. The present results also demonstrate a declining incidence of both diseases.