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 神经退行性疾病，发生在三个高发病率 西太平洋疫源地：关岛查莫罗人和北方 马里亚纳群岛，在纪伊半岛的日本人和 西新几内亚的Auyu和Jakai人。尽管，临床上的ALS和 在关岛看到的PD是两种不同的疾病，初步分析 表明将所有三种诊断（ALS，PD和ALS+PD）合并为 一个影响遗传分析的诊断，是合理的。年龄、性别 和出生队列特定责任的定义和隔离分析 在后勤和正常模式下对这一负债进行了评估， 发病时间。在任何一种模式下，纯粹的环境， 孟德尔显性和孟德尔隐性假说可以被拒绝， 但不能否定双等位基因加性主基因座假说。

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. Eventhough, clinically ALS and PD as seen on Guam are two distinct disorders, preliminary analyses indicated that combining all three diagnoses (ALS, PD, and ALS+PD) into one affected diagnosis for genetic analyses, 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.