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关岛帕金森痴呆综合征

Parkinsonism-dementia complex of Guam.

作者信息

Steele John C

机构信息

Micronesian Health Study II, Tamuning, Guam.

出版信息

Mov Disord. 2005 Aug;20 Suppl 12:S99-S107. doi: 10.1002/mds.20547.

DOI:10.1002/mds.20547
PMID:16092098
Abstract

On Guam and in two other Pacific locales, indigenous residents and immigrants are prone to familial neurodegeneration that manifests as atypical parkinsonism, dementia, motor neuron disease, or a combination of these three phenotypes. This progressive and fatal disease of the Mariana islands, the Kii peninsula of Japan, and the coastal plain of West New Guinea is similar and the pathological features have close affiliation with universal tauopathies, including progressive supranuclear palsy, Alzheimer's disease, and amyotrophic lateral sclerosis. The Chamorros of Guam call the disease lytico-bodig, and neuroscientists refer to it as the amyotrophic lateral sclerosis/Parkinsonism-dementia complex. During recent decades, its prevalence has declined progressively, and the age at onset has steadily increased. In 2004, motor neuron disease, once 100 times more common than elsewhere is rare, atypical parkinsonism is declining, and only dementia remains unusually common in elderly females. The cause of this obscure malady remains uncertain, despite 60 years of international research, but its ending implicates environmental influences rather than genetic predisposition.

摘要

在关岛以及其他两个太平洋地区,当地居民和移民容易患家族性神经退行性疾病,其表现为非典型帕金森症、痴呆、运动神经元病,或这三种表型的组合。这种在马里亚纳群岛、日本纪伊半岛和新几内亚西部沿海平原出现的进行性致命疾病具有相似性,其病理特征与包括进行性核上性麻痹、阿尔茨海默病和肌萎缩侧索硬化症在内的普遍性tau蛋白病密切相关。关岛的查莫罗人将这种疾病称为“lytico-bodig”,神经科学家则将其称为肌萎缩侧索硬化症/帕金森症-痴呆综合征。近几十年来,其患病率逐渐下降,发病年龄稳步上升。2004年,曾经比其他地方常见100倍的运动神经元病变得罕见,非典型帕金森症在减少,只有痴呆在老年女性中仍然异常常见。尽管经过60年的国际研究,这种隐匿疾病的病因仍不确定,但其结局表明是环境影响而非遗传易感性所致。

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