McKeith I G
Department of Old Age Psychiatry, Institute for the Health of the Elderly, Wolfson Research Centre, Newcastle General Hospital, Westgate Road, Newcastle upon Tyne NE4 6BE, UK.
Ann N Y Acad Sci. 2000;920:1-8. doi: 10.1111/j.1749-6632.2000.tb06898.x.
Lewy bodies are spherical, intracytoplasmic, eosinophilic, neuronal inclusions comprising abnormally truncated and phosphorylated intermediate neurofilament proteins, alpha-synuclein, ubiquitin, and associated enzymes. The clinical presentation of LB disease varies according to the site of LB formation and associated neuronal loss. Three main clinicopathological syndromes have been described--movement disorder, autonomic failure, and dementia. Parkinsonism is the most common presentation of LB disease developing in middle life. In older patients, a mixture of cognitive, autonomic, and motor dysfunction is more common. Dementia with LB (DLB) is a relatively recently described clinicopathological syndrome that accounts for up to 20% of all cases of dementia in old age. Patients, typically in their seventh and eighth decades, have LB pathology in cortical neurons as well as in the brain stem. LB disease should be considered in the differential diagnosis of a wide range of clinical presentations including episodic disturbances of consciousness, syncope, sleep disorders, and unexplained delirium.
路易小体是球形的、胞质内的、嗜酸性的神经元内含物，由异常截短和磷酸化的中间神经丝蛋白、α-突触核蛋白、泛素及相关酶组成。路易小体病的临床表现因路易小体形成部位及相关神经元丢失情况而异。已描述了三种主要的临床病理综合征——运动障碍、自主神经功能衰竭和痴呆。帕金森综合征是中年期发生的路易小体病最常见的表现形式。在老年患者中，认知、自主神经和运动功能障碍混合出现更为常见。路易小体痴呆（DLB）是一种相对较新描述的临床病理综合征，占老年痴呆所有病例的20%。患者通常为七、八十岁，其皮质神经元和脑干中均有路易小体病理改变。在包括意识发作性障碍、晕厥、睡眠障碍和不明原因谵妄等多种临床表现的鉴别诊断中，应考虑路易小体病。
