Weisman David, McKeith Ian
Department of Neurosciences and the Alzheimer's Disease Research Center, University of California, San Diego, CA 92093, USA.
Semin Neurol. 2007 Feb;27(1):42-7. doi: 10.1055/s-2006-956754.
Synucleinopathies, with and without dementia, encompass a wide range of diseases including Parkinson's disease, multiple system atrophy, rapid eye movement (REM) sleep behavior disorder, and dementia with Lewy bodies (DLB). DLB is a neurodegenerative disorder resulting in slowly progressive and unrelenting dementia until death. Prevalence studies suggest that it is the second most common dementing illness in the elderly. The neuropathologic findings of DLB show a wide anatomic range. Lewy bodies and Lewy-related pathology are found from the brain stem to the cortex and, in many cases, associated with concurrent Alzheimer's disease pathology. A recent international consortium on DLB has resulted in revised criteria for the clinical and pathological diagnosis of DLB incorporating new information about the core clinical features and improved methods for their assessment. The presentation of DLB is typically one of cortical and subcortical cognitive impairments, with worse visuospatial and executive dysfunction than Alzheimer's disease. There may be relative sparing of memory especially in the early stages. Core clinical features of DLB include fluctuating attention, recurrent visual hallucinations, and parkinsonism. Suggestive features include REM sleep behavior disorder, severe neuroleptic sensitivity, and low dopamine transporter uptake in the basal ganglia on functional neuroimaging. Additional supportive features that commonly occur in DLB, but with lower specificity, include repeated falls and syncope, transient, unexplained loss of consciousness, severe autonomic dysfunction, hallucinations in other modalities, systematized delusions, depression, relative preservation of medial temporal lobe structures on structural neuroimaging, reduced occipital activity on functional neuroimaging, prominent slow wave activity on electroencephalogram, and low uptake myocardial scintigraphy. Management of DLB includes pharmacological and nonpharmacological interventions for its cognitive, neuropsychiatric, motor, and sleep disturbances.
伴有或不伴有痴呆的突触核蛋白病涵盖多种疾病，包括帕金森病、多系统萎缩、快速眼动（REM）睡眠行为障碍和路易体痴呆（DLB）。DLB是一种神经退行性疾病，会导致缓慢进展且持续存在的痴呆，直至死亡。患病率研究表明，它是老年人中第二常见的痴呆性疾病。DLB的神经病理学发现显示出广泛的解剖范围。从脑干到皮质都能发现路易体和与路易体相关的病理学改变，并且在许多情况下，还伴有同时存在的阿尔茨海默病病理学改变。最近一个关于DLB的国际联盟制定了修订后的DLB临床和病理诊断标准，纳入了有关核心临床特征的新信息以及改进的评估方法。DLB的表现通常是皮质和皮质下认知障碍，与阿尔茨海默病相比，视觉空间和执行功能障碍更严重。记忆可能相对保留，尤其是在早期阶段。DLB的核心临床特征包括注意力波动、反复出现的视幻觉和帕金森综合征。提示性特征包括REM睡眠行为障碍、严重的抗精神病药物敏感性以及功能性神经影像学检查显示基底节区多巴胺转运体摄取降低。DLB中常见但特异性较低的其他支持性特征包括反复跌倒和晕厥、短暂的不明原因意识丧失、严重的自主神经功能障碍、其他形式的幻觉、系统性妄想、抑郁、结构神经影像学检查显示内侧颞叶结构相对保留、功能性神经影像学检查显示枕叶活动减少、脑电图上显著的慢波活动以及心肌闪烁显像摄取降低。DLB的管理包括针对其认知、神经精神、运动和睡眠障碍的药物和非药物干预措施。
