Bencs Viktor, Bencze János, Módis V László, Simon Viktória, Kálmán János, Hortobágyi Tibor
Általános Orvostudományi Kar, Neurológiai Tanszék, MTA-DE Cerebrovascularis és Neurodegeneratív Kutatócsoport,Debreceni Egyetem Debrecen.
Általános Orvostudományi Kar, Patológiai Intézet,Debreceni Egyetem Debrecen.
Orv Hetil. 2020 May 1;161(18):727-737. doi: 10.1556/650.2020.31715.
Dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD) are two major neurodegenerative diseases sharing common clinical, pathophysiological and morphologic features. The pathological hallmark of both diseases is the presence of Lewy-bodies (LB). The main constituent of these inclusions is the pathologically aggregated α-synuclein protein. In DLB, LBs are predominantly located in the cortex, whereas in PDD, the subcortical regions are predominantly affected. Furthermore, in DLB, coexisting Alzheimer's disease (AD), pathology with β-amyloid plaques and neurofibrillary tangles are more common. It is still debated whether DLB and PDD are two distinct entities or different phenotypes of the same disease. Clinical diagnosis is based on the temporal sequence of motor and cognitive symptoms. Dementia often precedes parkinsonism in DLB, while in PDD, cognitive decline generally appears after the onset of motor symptoms. Also, fluctuation of cognitive functions and neuroleptic sensitivity is more severe in DLB than PDD. The recent advancements of imaging techniques revealed that cortical damage, cholinergic deficit and concomitant AD pathology are more severe in DLB compared to PDD. The analysis of cerebrospinal fluid biomarkers shows higher oligomeric α-synuclein burden in PDD. Levodopa is less effective in DLB than in PDD and may increase the risk of psychosis. In this review, we comprehensively analyse the pathological, radiological and clinical features of DLB and PDD, highlighting the overlaps and differences. Orv Hetil. 2020; 161(18): 727-737.
路易体痴呆(DLB)和帕金森病痴呆(PDD)是两种主要的神经退行性疾病,具有共同的临床、病理生理和形态学特征。这两种疾病的病理标志都是路易小体(LB)的存在。这些包涵体的主要成分是病理聚集的α-突触核蛋白。在DLB中,LB主要位于皮质,而在PDD中,主要受影响的是皮质下区域。此外,在DLB中,共存的阿尔茨海默病(AD)病理以及β-淀粉样斑块和神经原纤维缠结更为常见。DLB和PDD是两种不同的实体还是同一疾病的不同表型仍存在争议。临床诊断基于运动和认知症状的时间顺序。在DLB中,痴呆通常先于帕金森ism出现,而在PDD中,认知功能下降一般在运动症状出现后出现。此外,DLB中认知功能的波动和对神经阻滞剂的敏感性比PDD更严重。最近成像技术的进展表明,与PDD相比,DLB中的皮质损伤、胆碱能缺陷和伴随的AD病理更严重。脑脊液生物标志物分析显示PDD中寡聚α-突触核蛋白负担更高。左旋多巴在DLB中的疗效不如PDD,并且可能增加精神病风险。在本综述中,我们全面分析了DLB和PDD的病理、放射学和临床特征,突出了重叠和差异。《匈牙利医学周报》。2020年;161(18):727 - 737。