Institute of Neuroscience, The Medical School, Newcastle University, Framlington Place, Newcastle Upon Tyne, NE2 4HH, UK.
Department of Experimental Neurodegeneration, Center for Nanoscale Microscopy and Molecular Physiology of the Brain, Center for Biostructural Imaging of Neurodegeneration, University Medical Center Göttingen, Göttingen, Germany.
Mol Neurodegener. 2019 Jan 21;14(1):5. doi: 10.1186/s13024-019-0306-8.
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.
路易体痴呆症 (DLB) 是一种与年龄相关的神经退行性疾病,会导致认知能力逐渐下降,从而干扰正常生活和日常活动。从神经病理学角度来看,DLB 的特征是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)。DLB 患者常见与 PD 相似的锥体外系运动特征,但这些特征并非 DLB 临床诊断的必要条件。由于许多 PD 患者随着疾病的进展会出现痴呆,因此关于将 DLB 与 PD 痴呆 (PDD) 分开的问题一直存在争议,共识报告提出了一些指导方针,以帮助临床医生识别和管理这两种综合征。在这里,我们根据目前的理解提出了一些基本概念和定义,这些概念和定义应该指导医学界解决悬而未决的问题,希望这将有助于我们提高对 DLB 和其他突触核蛋白病的诊断,并为其制定新的治疗策略。