EPIDEMIOLOGICAL AND GENETIC STUDIES OF ALS/PD COMPLEX OF GUAM

肌萎缩侧索硬化症（ALS）和帕金森痴呆症（PD）是 发生在三个高发病灶的神经退行性疾病 西太平洋：关岛的查莫罗人和北方 马里亚纳群岛、纪伊半岛上的日本人和奥尤群岛 和西新几内亚的贾凯人。 临床上ALS和PD是两种 不同的疾病 然而，初步分析表明， 所有三种诊断（ALS、PD和ALS+PD）合并为一种受影响的诊断， 遗传分析，（从而假设任何遗传效应对易感性， 这两种疾病是由于相同的遗传机制）是合理的。 定义了年龄、性别和出生群体特定的责任， 在logistic和正态模型下对此进行了分析 疾病发生时的责任。 在任何一种模式下，纯 环境，孟德尔显性和孟德尔隐性假说， 被拒绝，但两个等位基因加性主基因座假说不能被接受。 拒绝

Amyotrophic Lateral Sclerosis (ALS) and Parkinsonism dementia (PD)are neurological degenerative disorders that occur in three high incidence foci in the western Pacific: among the Chamorros of Guam and the northern Mariana islands, among the Japanese on the Kii peninsula and among the Auyu and Jakai peoples of West New Guinea. Clinically ALS and PD are two distinct disorders. However, preliminary analyses indicated that combining all three diagnoses (ALS, PD, and ALS+PD) into one affected diagnosis for genetic analyses, (thereby assuming any genetic effect on susceptibility to the two disorders was due to the same genetic mechanism) was reasonable. An Age, sex and birth cohort-specific liability was defined and segregation analysis was performed under both logistic and normal models for this liability at the time of disease onset. Under either model, purely environmental, Mendelian dominant and Mendelian recessive hypotheses could be rejected, but a two allele additive major locus hypothesis could not be rejected.