McKeith Ian
Institute for Ageing and Health, Newcastle General Hospital, Newcastle upon Tyne, UK.
Dialogues Clin Neurosci. 2004 Sep;6(3):333-41. doi: 10.31887/DCNS.2004.6.3/imckeith.
Dementia with Lewy bodies (DLB) is the second most common cause of neurodegenerative dementia in older people, accounting for 10% to 15% of all cases, it occupies part of a spectrum that includes Parkinson's disease and primary autonomic failure. All these diseases share a neuritic pathology based upon abnormal aggregation of the synaptic protein α-synuciein. It is important to identify DLB patients accurately because they have specific symptoms, impairments, and functional disabilities thai differ from other common dementia syndromes such as Alzheimer's disease, vascular cognitive impairment, and frontotemporal dementia. Clinical diagnostic criteria for DLB have been validated against autopsy, but fail to detect a substantial minority of cases with atypical presentations that are often due to the presence of mixed pathology. DLB patients frequently have severe neuroleptic sensitivity reactions, which are associated with significantly increased morbidity and mortality. Cholinesterase inhibitor treatment is usually well tolerated and substantially improves cognitive and neuropsychiatrie symptoms. Although virtually unrecognized 20 years ago, DLB could within this decade become one of the most treatable neurodegenerative disorders of late life.
路易体痴呆(DLB)是老年人神经退行性痴呆的第二大常见病因,占所有病例的10%至15%,它属于一个谱系的一部分,该谱系包括帕金森病和原发性自主神经功能衰竭。所有这些疾病都有基于突触蛋白α-突触核蛋白异常聚集的神经病理改变。准确识别DLB患者很重要,因为他们有特定的症状、损害和功能残疾,这些与其他常见的痴呆综合征不同,如阿尔茨海默病、血管性认知障碍和额颞叶痴呆。DLB的临床诊断标准已通过尸检验证,但未能检测出相当一部分具有非典型表现的病例,这些病例通常是由于存在混合病理。DLB患者经常有严重的抗精神病药物敏感反应,这与发病率和死亡率显著增加有关。胆碱酯酶抑制剂治疗通常耐受性良好,并能显著改善认知和神经精神症状。尽管20年前几乎未被认识到,但在这十年内,DLB可能会成为晚年最可治疗的神经退行性疾病之一。