Expert recommendation on the diagnosis and management of dementia with Lewy bodies (2026)

doi:10.3969/j.issn.1672-6731.2026.08.002

Abstract

Abstract:

Dementia with Lewy bodies (DLB) is the second most common type of neurodegenerative dementia after Alzheimer's disease (AD) and represents a major component of the Lewy body disease (LBD) spectrum. In China, the first and second versions of the expert consensus were issued in 2015 and 2021, respectively. With the growing understanding of the clinical features and biomarkers of DLB, and considering recent advances in multidisciplinary diagnostic and therapeutic models as well as clinical practice in China, this updated consensus systematically integrates the latest domestic and international evidence. It provides comprehensive updates on the epidemiology, etiology and pathophysiological mechanisms, clinical manifestations, accessory examinations, diagnosis and differential diagnosis, and treatment strategies of DLB. "Expert recommendation on the diagnosis and management of dementia with Lewy bodies (2026)" emphasizes a diagnostic framework combining core clinical features with indicative biomarkers, as well as the value of multimodal biomarkers in early identification. In addition, it proposes a treatment strategy centered on symptomatic management and multidisciplinary care. The aim is to provide more standardized and practical guidance for clinical diagnosis and treatment, thereby improving the recognition and management of DLB in China.

Key words: Lewy body disease, Dementia, Biomarkers, Diagnosis, Therapy

摘要：

路易体痴呆是仅次于阿尔茨海默病的神经变性痴呆类型，也是路易体病谱系的重要组成部分。国内已于2015和2021年发布两版专家共识。随着对路易体痴呆临床特征及生物学标志物研究的深入，结合国内多学科诊疗模式的发展和相关临床实践的进展，在既往专家共识的基础上，系统整合近年国内外研究证据，对路易体痴呆流行病学、病因与病理生理学机制、临床表现、辅助检查、诊断与鉴别诊断、治疗等内容进行更新与完善，形成《路易体痴呆诊断与治疗推荐意见2026》。重点强调核心特征与提示性生物学标志物相结合的诊断体系，以及多模态生物学标志物在早期识别中的应用价值，同时提出以对症治疗和多学科综合管理为核心的治疗策略，旨在为临床提供更规范、实用的诊断与治疗依据，进一步提升我国路易体痴呆的识别与管理水平。

关键词: Lewy体病, 痴呆, 生物标记, 诊断, 治疗

CLC Number:

R741

Hua-long WANG, Xin-yi XIE, Ning SU, Bo HONG, Ling YUE, Shao-wei ZHANG, Li-min ZHANG, Guo-ping PENG, Xia LI, Gang WANG. Expert recommendation on the diagnosis and management of dementia with Lewy bodies (2026)[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2026, 26(8): 794-806.

王华龙, 谢心怡, 苏宁, 洪波, 岳玲, 张少伟, 张立敏, 彭国平, 李霞, 王刚. 路易体痴呆诊断与治疗推荐意见2026[J]. 中国现代神经疾病杂志, 2026, 26(8): 794-806.

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URL: https://journal11.magtechjournal.com/cjcnn/EN/10.3969/j.issn.1672-6731.2026.08.002

https://journal11.magtechjournal.com/cjcnn/EN/Y2026/V26/I8/794

Figures/Tables 6

References 72

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证据级别与推荐等级	标准
证据级别
A级	证据来源于多个随机对照临床试验或高质量Meta分析
B级	证据来源于单个随机对照临床试验或未随机分组的大样本临床试验
C级	证据来源于未经严格论证的专家意见、小样本临床研究或回顾性研究
推荐等级
Ⅰ级	证据或高度一致的专家共识认为治疗有效或获益
Ⅱ级	对治疗有效或获益的证据不一致 Ⅱb级，治疗有效或获益待考证 Ⅱa级，倾向于认为治疗有效或获益
Ⅲ级	证据或高度一致的专家共识认为治疗无效，甚至有害

证据级别与推荐等级	标准
证据级别
A级	证据来源于多个随机对照临床试验或高质量Meta分析
B级	证据来源于单个随机对照临床试验或未随机分组的大样本临床试验
C级	证据来源于未经严格论证的专家意见、小样本临床研究或回顾性研究
推荐等级
Ⅰ级	证据或高度一致的专家共识认为治疗有效或获益
Ⅱ级	对治疗有效或获益的证据不一致 Ⅱb级，治疗有效或获益待考证 Ⅱa级，倾向于认为治疗有效或获益
Ⅲ级	证据或高度一致的专家共识认为治疗无效，甚至有害

诊断标准	诊断核心要素	诊断依据与强度划分
国际路易体痴呆协作组1996年标准^[9]	满足波动性认知障碍、视幻觉、帕金森样症状中2项核心症状，并排除其他痴呆病因	以临床症状为主要依据
国际路易体痴呆协作组2005年标准^[10]	必要特征：进行性认知功能下降并影响社会职业功能和日常生活核心特征：认知波动，视幻觉，帕金森综合征增加支持性特征：RBD，对抗精神病药物敏感，PET/SPECT显示多巴胺能系统异常强调与阿尔茨海默病的鉴别，如路易体痴呆较少出现典型阿尔茨海默病的渐进性记忆力减退	很可能的路易体痴呆：2个及以上核心特征或者1个核心特征+ 1个及以上支持特征可能的路易体痴呆：1个核心特征或者1个及以上支持特征
国际路易体痴呆协作组2017年标准^[11]	必要特征：进行性认知功能下降并影响社会职业功能和日常生活核心特征：将RBD加入核心临床特征引入提示性生物学标志物：DAT⁃PET显示基底节区DAT摄取减少¹²³I⁃MIBG SPECT心肌显影显示¹²³I⁃MIBG摄取减少；PSG监测证实RBD增加支持性生物学标志物：强调排除混合性病理，如路易体痴呆与阿尔茨海默病共病	临床症状与生物学标志物相结合：很可能的路易体痴呆：2个及以上核心特征（±提示性生物学标志物）或1个核心特征+ 1个提示性生物学标志物可能的路易体痴呆：1个核心特征（无提示性生物学标志物）或1个及以上提示性生物学标志物（无核心特征）

诊断标准	诊断核心要素	诊断依据与强度划分
国际路易体痴呆协作组1996年标准^[9]	满足波动性认知障碍、视幻觉、帕金森样症状中2项核心症状，并排除其他痴呆病因	以临床症状为主要依据
国际路易体痴呆协作组2005年标准^[10]	必要特征：进行性认知功能下降并影响社会职业功能和日常生活核心特征：认知波动，视幻觉，帕金森综合征增加支持性特征：RBD，对抗精神病药物敏感，PET/SPECT显示多巴胺能系统异常强调与阿尔茨海默病的鉴别，如路易体痴呆较少出现典型阿尔茨海默病的渐进性记忆力减退	很可能的路易体痴呆：2个及以上核心特征或者1个核心特征+ 1个及以上支持特征可能的路易体痴呆：1个核心特征或者1个及以上支持特征
国际路易体痴呆协作组2017年标准^[11]	必要特征：进行性认知功能下降并影响社会职业功能和日常生活核心特征：将RBD加入核心临床特征引入提示性生物学标志物：DAT⁃PET显示基底节区DAT摄取减少¹²³I⁃MIBG SPECT心肌显影显示¹²³I⁃MIBG摄取减少；PSG监测证实RBD增加支持性生物学标志物：强调排除混合性病理，如路易体痴呆与阿尔茨海默病共病	临床症状与生物学标志物相结合：很可能的路易体痴呆：2个及以上核心特征（±提示性生物学标志物）或1个核心特征+ 1个提示性生物学标志物可能的路易体痴呆：1个核心特征（无提示性生物学标志物）或1个及以上提示性生物学标志物（无核心特征）

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