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文摘
查莫罗人遭受两个神经综合症称为ALS和parkinsonism-dementia关岛的复杂(PDC)。我们报告死亡率数据在1991年到1995年这些综合症,每隔5年进行比较与那些可以追溯到1951年。与预测疾病消失,症状仍然盛行。然而,发病的年龄和死亡年龄增加了对于这两个症状,建议推迟转移环境因素导致疾病。我们也报告临床、家族、neuropathologic,免疫组织化学结果一系列连续解剖Guamanian查莫罗人案件。12例诊断为PDC,当地称为“bodig”和三个肌萎缩性侧索硬化症,当地人称为“lytico。”All but three of these fifteen patients had a pronounced family history of similar illness. Eight of twelve bodig patients had siblings who were also affected with bodig; two of three lytico cases had siblings afflicted with lytico. The family histories are compatible with genetic transmission of each syndrome. The neuropathology of bodig is characterized by severe and widespread neurofibrillary tangle (NFT) development. NFTs are surrounded by reactive microglia and reactive astrocytes, and complement proteins and other molecules connected with inflammation are associated with them. Similar inflammatory responses also occur in Alzheimer's disease (AD) but have been largely attributed to the presence of senile plaques. These data indicate that tangles, as well as plaques, generate inflammatory reactions and that such reactions may exacerbate the fundamental pathology in bodig as well as in AD.
