EPIDEMIOLOGICAL AND GENETIC STUDIES OF ALS/PD COMPLEX OF GUAM

为了阐明肌萎缩性肌萎缩性侧索硬化发病率高的病因， 侧索硬化症（ALS）和帕金森氏症痴呆症（PD）的岛屿上， 关岛，一项患者对照前瞻性研究（登记）建立于 1958. 除了患者及其家属外， 个体匹配的对照，他们各自的父母，兄弟姐妹，后代 和配偶。 登记处的目标是确定（1） ALS和PD患者的亲属是否有更高的发展风险 疾病比亲属的控制和（2）如果家庭发生 存在，以确定遗传参与病因学的程度 这种疾病 对登记处进行了25年的随访分析， 刚刚结束，结果已经公布。 本研究的其他目的是：1）调查遗传和 导致高发病率的流行病学因素 关岛的肌萎缩侧索硬化症和帕金森氏痴呆症; 2)评估各种已建立的遗传和 人类学标记之间的正常关岛人口和比较 他们与ALS/PD患者;和3）确定的影响， 由于瘫痪而导致的固定对骨密度的影响。

In an effort to elucidate the etiology of high incidence of Amyotrophic Lateral Sclerosis (ALS) and Parkinsonism Dementia (PD) on the island of Guam, a patient-control prospective study (Registry) was established in 1958. The registry includes, in addition to the patients and their individually matched controls, their respective parents, sibs, offspring and spouses. The objective of the registry has been to determine (1) whether relatives of ALS and PD patients have higher risk for developing the disease than relatives of controls and (2) if familial occurrence does exist, to determine the extent of genetic involvement in the etiology of the disease. A twenty-five year follow-up analysis of the registry has just been concluded and the results are published. Other objectives of this study are: 1) to investigate the genetic and epidemiological factors contributing to the very high incidence of Amyotrophic Lateral Sclerosis and Parkinsonism Dementia (ALS/PD) on Guam; 2) to evaluate the distribution of the various established genetic and anthropological markers among the normal Guamanian population and compare them with those of the ALS/PD patients; and 3) to ascertain the effects of immobilization due to paralysis on bone density.