EPIDEMIOLOGICAL AND GENETIC STUDIES OF ALS/PD COMPLEX OF GUAM

为阐明肌萎缩侧索硬化症和帕金森病高发的病因。 关岛，一项患者对照前瞻性研究(注册) 成立于1958年。登记处除病人外，还包括 和他们各自匹配的对照组，他们各自的父母，兄弟姐妹， 后代和配偶。登记处的目标一直是确定 (1)肌萎缩侧索硬化症和帕金森病患者的亲属是否有更高的风险 (2)是否有家族性 确实存在这种情况，以确定基因参与的程度 这种疾病的病原学。一项25年的随访分析 注册工作刚刚结束。 这项研究的其他目标是：1)研究遗传和 导致极高发病率的流行病学因素 关岛肌萎缩侧索硬化症和帕金森病痴呆(ALS/PD)； 2)评估各种已建立的遗传和基因的分布 关岛正常人群的人类学标记及其比较 与肌萎缩侧索硬化症/帕金森病患者的疗效比较；以及3)确定 瘫痪所致的制动对骨密度影响。

In an effort to elucitate the etiology of high incidence of ALS and 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. Other objectives of this study are: 1) to ivestigate 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 populations and compare them with those of the ALS/PD patients; and 3) to ascertain the effects of immobilization due to paralysis on bone density.