School of Medicine and Surgery, University of Milano-Bicocca, 20900 Monza, Italy.
Institute of Molecular Bioimaging and Physiology, National Research Council, 20054 Segrate (MI), Italy.
Int J Mol Sci. 2021 Apr 12;22(8):3960. doi: 10.3390/ijms22083960.
Dementia with Lewy bodies (DLB) is one of the most common causes of dementia and belongs to the group of α-synucleinopathies. Due to its clinical overlap with other neurodegenerative disorders and its high clinical heterogeneity, the clinical differential diagnosis of DLB from other similar disorders is often difficult and it is frequently underdiagnosed. Moreover, its genetic etiology has been studied only recently due to the unavailability of large cohorts with a certain diagnosis and shows genetic heterogeneity with a rare contribution of pathogenic mutations and relatively common risk factors. The rapid increase in the reported cases of DLB highlights the need for an easy, efficient and accurate diagnosis of the disease in its initial stages in order to halt or delay the progression. The currently used diagnostic methods proposed by the International DLB consortium rely on a list of criteria that comprises both clinical observations and the use of biomarkers. Herein, we summarize the up-to-now reported knowledge on the genetic architecture of DLB and discuss the use of prodromal biomarkers as well as recent promising candidates from alternative body fluids and new imaging techniques.
路易体痴呆(DLB)是最常见的痴呆症病因之一,属于α-突触核蛋白病的一种。由于其与其他神经退行性疾病的临床表现存在重叠,且具有高度的临床异质性,因此,DLB 与其他类似疾病的临床鉴别诊断往往较为困难,并且常常被漏诊。此外,由于缺乏具有明确诊断的大样本队列,其遗传病因学直到最近才得到研究,并且表现出遗传异质性,致病性突变的罕见贡献和相对常见的风险因素。DLB 报告病例的快速增加凸显了在疾病早期阶段进行简便、高效和准确诊断的必要性,以便阻止或延缓疾病进展。目前国际 DLB 联盟提出的诊断方法依赖于一系列标准,包括临床观察和生物标志物的使用。本文总结了迄今为止关于 DLB 的遗传结构的研究进展,并讨论了前驱期生物标志物的应用以及来自其他体液和新影像学技术的有前途的候选标志物。