Dementia with Lewy bodies (DLB) is an age-associated neurodegenerative disorder producing progressive cognitive decline that interferes with normal life and daily activities. Neuropathologically, DLB is characterised by the accumulation of aggregated α-synuclein protein in Lewy bodies and Lewy neurites, similar to Parkinson’s disease (PD). Extrapyramidal motor features characteristic of PD, are common in DLB patients, but are not essential for the clinical diagnosis of DLB. Since many PD patients develop dementia as disease progresses, there has been controversy about the separation of DLB from PD dementia (PDD) and consensus reports have put forward guidelines to assist clinicians in the identification and management of both syndromes. Here, we present basic concepts and definitions, based on our current understanding, that should guide the community to address open questions that will, hopefully, lead us towards improved diagnosis and novel therapeutic strategies for DLB and other synucleinopathies.

中文翻译：

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路易体痴呆：更新和展望

路易体痴呆 (DLB) 是一种与年龄相关的神经退行性疾病，会导致进行性认知衰退，干扰正常生活和日常活动。神经病理学上，DLB 的特征在于路易体和路易神经突中聚集的 α-突触核蛋白的积累，类似于帕金森病 (PD)。PD 的锥体外系运动特征在 DLB 患者中很常见，但对于 DLB 的临床诊断不是必需的。由于许多 PD 患者随着疾病的进展发展为痴呆，因此关于 DLB 与 PD 痴呆 (PDD) 的分离一直存在争议，共识报告提出了帮助临床医生识别和管理这两种综合征的指南。在这里，我们根据我们目前的理解提出基本概念和定义，