Bencze János, Seo Woosung, Hye Abdul, Aarsland Dag, Hortobágyi Tibor
Department of Pathology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary.
MTA-DE Cerebrovascular and Neurodegenerative Research Group, Department of Neurology, University of Debrecen, Debrecen, Hungary.
Free Neuropathol. 2020 Feb 18;1:7. doi: 10.17879/freeneuropathology-2020-2613. eCollection 2020 Jan.
Dementia is one of the major burdens of our aging society. According to certain predictions, the number of patients will double every 20 years. Although Alzheimer's disease (AD), as the most frequent neurodegenerative dementia, has been extensively analysed, less is known about dementia with Lewy bodies (DLB). Neuropathological hallmarks of DLB are the deposition of intracellular Lewy bodies (LB) and Lewy neurites (LN). DLB belongs to the α-synucleinopathies, as the major component of these inclusions is pathologically aggregated α-synuclein. Depending on the localisation of LBs and LNs in the central nervous system cognitive and motor symptoms can occur. In our work, we will systematically review the possible etiology and epidemiology, pathological (both macroscopic and microscopic) features, structural and functional imaging findings, with a special emphasis on the clinico-pathological correlations. Finally, we summarize the latest clinical symptoms-based diagnostic criteria and the novel therapeutic approaches. Since DLB is frequently accompanied with AD pathology, highlighting possible differential diagnostic approaches is an integral part of our paper. Although our present knowledge is insufficient, the rapid development of diagnostic and research methods provide hope for better diagnosis and more efficient treatment, contributing to a better quality of life.
痴呆症是老龄化社会的主要负担之一。根据某些预测,患者数量每20年将翻一番。尽管阿尔茨海默病(AD)作为最常见的神经退行性痴呆已得到广泛分析,但关于路易体痴呆(DLB)的了解却较少。DLB的神经病理学特征是细胞内路易小体(LB)和路易神经突(LN)的沉积。DLB属于α-突触核蛋白病,因为这些包涵体的主要成分是病理性聚集的α-突触核蛋白。根据LB和LN在中枢神经系统中的定位,可出现认知和运动症状。在我们的工作中,我们将系统地回顾可能的病因和流行病学、病理(宏观和微观)特征、结构和功能影像学发现,特别强调临床病理相关性。最后,我们总结了最新的基于临床症状的诊断标准和新的治疗方法。由于DLB常伴有AD病理,突出可能的鉴别诊断方法是我们论文的一个组成部分。尽管我们目前的知识不足,但诊断和研究方法的快速发展为更好的诊断和更有效的治疗带来了希望,有助于提高生活质量。