Occasional Review
Occasional Review
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R. D. Monie;A. M. Hunter;K. Rocchiccioli;J. P. White;I. A. Campbell;G. S. Kilpatrick;S. Glamorgan-S.-Glamo
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R. D. Monie;A. M. Hunter;K. Rocchiccioli;J. P. White;I. A. Campbell;G. S. Kilpatrick;S. Glamorgan-S.-Glamo
Lewy小体是一种独特的神经元包涵体，总是在帕金森病的黑质和其他特定脑区中发现。它主要由结构改变的神经丝组成，并且发生在神经元过度丢失的地方。它发生在一些老年人中，很少发生在其他中枢神经系统退行性疾病中。在273名死于帕金森病以外疾病的患者的大脑中，路易体的年龄特异性患病率在60岁至90岁之间从3-8%增加到12-8%。相关的病理结果表明，这些偶发性路易体病的病例是帕金森病的前驱病例，并证实了年龄（时间）在疾病演变中的重要性。鉴于这种疾病的常见和广泛发生，我们建议，在生命早期的内源性机制可能比环境因素更重要的发病机制路易体和帕金森氏病。在所有符合英国帕金森病协会脑库临床诊断标准（表1）的帕金森病病例中，如果通过病理检查确定并排除其他帕金森病，则称为路易体的神经元内含物存在于黑质的许多存活细胞中。因此，路易体提供了一种诊断标志物，并且对于病理学诊断来说与细胞损失的特异性分布一样重要。即使在黑质的一个双侧7 pm切片中没有Lewy小体，也排除了帕金森病。在没有帕金森病特征的个体中发现路易体的报告表明，这种疾病处于症状前期（偶发性路易体病）。2由于黑质外部位的细胞丢失和路易体形成，痴呆34和自主神经功能衰竭5不太常见，使帕金森病复杂化，或很少单独发生。路易体-帕金森病谱系的这些其他表现鼓励了包括特发性路易体病的病理术语（表2）。近年来，出现了一小群罕见疾病或常见疾病的罕见变体有时也与神经系统中的路易体有关，通常发生在特发性路易体病的某些相同区域（表2）。然而，这些不同，因为大多数是家族性的，发生在年轻的时候，并与其他病理病变。与帕金森氏病相反，路易体并不总是存在;例如，它们仅在Hallervorden-Spatz病的10-15%病例中报告。特发性路易体病的分布.
SUMMARY The Lewy body is a distinctive neuronal inclusion that is always found in the substantia nigra and other specific brain regions in Parkinson's disease. It is mainly composed of structurally altered neurofilament, and occurs wherever there is excessive loss of neurons. It occurs in some elderly individuals and rarely in other degenerative diseases of the central nervous system. In 273 brains of patients dying from disorders other than Parkinson's disease, the age-specific prevalence of Lewy bodies increased from 3-8% to 12-8% between the sixth and ninth decades. Associated pathological findings suggest that these cases of incidental Lewy body disease are presymptomatic cases of Parkinson's disease, and confirm the importance of age (time) in the evolution of the disease. In view of the common and widespread occurrence of this disorder we propose that endo-genous mechanisms operating in early life may be more important than environmental agents in the pathogenesis of Lewy bodies and Parkinson's disease. Neuronal inclusions called Lewy bodies are present in many surviving cells of the substantia nigra in all cases of Parkinson's disease fulfilling the UK Parkin-son's Disease Society Brain Bank clinical diagnostic criteria (table 1), if alternative Parkinsonian disorders are identified and excluded by pathological examination. Lewy bodies therefore provide a diagnostic marker and are as essential for the pathological diagnosis as the specific distribution of cell loss. The absence of Lewy bodies in even one bilateral 7 pm section of substantia nigra excludes Parkinson's disease .' Reports of Lewy bodies in individuals without Parkinsonian features suggest a presymptomatic phase of the disease (incidental Lewy body disease).2 Less commonly dementia34 and autonomic failure,5 due to cell loss and Lewy body formation in extra-nigral sites, complicate Parkinson's disease, or rarely occur alone. These other manifestations of the Lewy body-Parkinson's disease spectrum encourage the inclusive pathological term of idiopathic Lewy body disease (table 2). In recent years it has emerged that a small group of rare disorders or rare variants of common disorders are also sometimes associated with Lewy bodies in the nervous system, usually in some of the same areas affected in idiopathic Lewy body disease (table 2). These differ, however, because most are familial, occur at a young age and are associated with additional pathological lesions. In contrast to Parkinson's disease Lewy bodies are not invariably present; for example they are reported in only 10-15% of cases of Hallervorden-Spatz disease. IDIOPATHIC LEWY BODY DISEASE Distribution of …