Lewy Body Dementias: Dementia With Lewy Bodies and Parkinson Disease Dementia.
Lewy Body Dementias: Dementia With Lewy Bodies and Parkinson Disease Dementia.
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DOI：
10.1212/con.0000000000000309
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发表时间：
2016-04-01
期刊：
影响因子：
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通讯作者：
Gomperts, Stephen N
中科院分区：
文献类型：
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作者：
Gomperts, Stephen N
审查目的：本文概述了路易体痴呆（DLB）和帕金森病痴呆（PDD）的临床特征、神经病理学表现、诊断标准和治疗。DLB和PDD是常见的，临床上相似的综合征，具有共同的特征性神经病理学变化，包括路易体和神经突中α-突触核蛋白的沉积以及被盖多巴胺细胞群和基底前脑胆碱能细胞群的丧失，通常伴有不同程度的共存阿尔茨海默病病理学。DLB和PDD的临床星座包括与帕金森综合征相关的进行性认知障碍、幻视以及注意力和觉醒的波动。目前的临床诊断标准强调这些特征，并权衡单光子发射计算机断层扫描（SPECT）成像测量的多巴胺细胞丢失和快速眼动（REM）睡眠行为障碍（突触核蛋白病的风险因素）的证据。痴呆相对于帕金森综合征的发生时间是DLB和PDD之间的主要临床区别，在明确的特发性帕金森病（至少1年的运动症状后）背景下出现的痴呆表示PDD，而相对于帕金森综合征的早期认知障碍表示DLB。这些综合征之间的区别仍然是一个活跃的研究问题。这些疾病的治疗仍然是对症的，并依赖于药理学和nonpharmacologicstrategies.SUMMARY：DLB和PDD是重要的和常见的痴呆综合征，重叠在其临床特征，神经病理学和管理。它们被认为存在于路易体病的一个谱中，并且在它们的分化中存在一些争议。鉴于需要优化认知，锥体外系功能和精神健康，管理可能是复杂的，应该是系统的。
PURPOSE OF REVIEW: This article provides an overview of the clinical features, neuropathologic findings, diagnostic criteria, and management of dementia with Lewy bodies (DLB) and Parkinson disease dementia (PDD), together known as the Lewy body dementias.RECENT FINDINGS: DLB and PDD are common, clinically similar syndromes that share characteristic neuropathologic changes, including deposition of alpha-synuclein in Lewy bodies and neurites and loss of tegmental dopamine cell populations and basal forebrain cholinergic populations, often with a variable degree of coexisting Alzheimer pathology. The clinical constellations of DLB and PDD include progressive cognitive impairment associated with parkinsonism, visual hallucinations, and fluctuations of attention and wakefulness. Current clinical diagnostic criteria emphasize these features and also weigh evidence for dopamine cell loss measured with single-photon emission computed tomography (SPECT) imaging and for rapid eye movement (REM) sleep behavior disorder, a risk factor for the synucleinopathies. The timing of dementia relative to parkinsonism is the major clinical distinction between DLB and PDD, with dementia arising in the setting of well-established idiopathic Parkinson disease (after at least 1 year of motor symptoms) denoting PDD, while earlier cognitive impairment relative to parkinsonism denotes DLB. The distinction between these syndromes continues to be an active research question. Treatment for these illnesses remains symptomatic and relies on both pharmacologic and nonpharmacologic strategies.SUMMARY: DLB and PDD are important and common dementia syndromes that overlap in their clinical features, neuropathology, and management. They are believed to exist on a spectrum of Lewy body disease, and some controversy persists in their differentiation. Given the need to optimize cognition, extrapyramidal function, and psychiatric health, management can be complex and should be systematic.