Hudson A J, Rice G P
Department of Clinical Neurological Sciences, University Hospital, London, Ontario.
Can J Neurol Sci. 1990 Nov;17(4):427-33. doi: 10.1017/s0317167100031024.
Guamanian amyotrophic lateral sclerosis with parkinsonism-dementia (ALS/PD) has been the subject of intensive study since its discovery in 1947 because of its extraordinarily high incidence in a small ethnic group (Chamorros) whose dietary lack and customs have suggested possible causes. As yet, these and other suspected causes have eluded proof. Because of marked similarities between Guamanian ALS/PD and late onset post-encephalitic (encephalitis lethargica) parkinsonism and ALS it is suggested that they have a common cause. The parkinsonism and ALS in the two disorders are clinically very similar and neuropathological studies have shown a very similar distribution of neurofibrillary tangles in neurons. Some clinical differences, such as ocular features in the post-encephalitic cases and dementia in Guamanian ALS/PD, can be explained by differences in the severity of infection and the interval between the encephalitis and onset of sequelae. Although unproven, influenza A (HswilN1 strain) has long been suspected as the cause of encephalitis lethargica because of simultaneous pandemics of the two diseases in the 1920s. Because influenza A can persistently infect cells and has a marked propensity to mutate it is an optimal candidate among other RNA viruses for delayed nervous system infection as a possible cause of ALS/PD.
自1947年发现以来，关岛肌萎缩侧索硬化症合并帕金森痴呆综合征（ALS/PD）一直是深入研究的对象，因为它在一个小种族群体（查莫罗人）中的发病率极高，而该群体的饮食缺乏和习俗提示了可能的病因。然而，这些以及其他可疑病因尚未得到证实。由于关岛ALS/PD与晚发性脑炎后（昏睡性脑炎）帕金森综合征和ALS之间存在明显相似性，有人认为它们有共同的病因。这两种疾病中的帕金森综合征和ALS在临床上非常相似，神经病理学研究表明神经元中的神经原纤维缠结分布非常相似。一些临床差异，如脑炎后病例的眼部特征和关岛ALS/PD中的痴呆，可以用感染严重程度以及脑炎与后遗症发作之间的时间间隔差异来解释。虽然未经证实，但由于20世纪20年代这两种疾病同时大流行，甲型流感（HswilN1毒株）长期以来一直被怀疑是昏睡性脑炎的病因。由于甲型流感可以持续感染细胞并且有明显的变异倾向，在其他RNA病毒中，它是导致ALS/PD的延迟性神经系统感染的最佳候选者。
