PDF(Pubmed)
Abstract：
Dementia with Lewy bodies (DLB), Parkinson\'s disease dementia (PDD), and Parkinson\'s disease (PD) collectively known as Lewy body diseases (LBDs) are neuropathologically characterised by α-synuclein deposits (Lewy bodies and Lewy neurites). However, LBDs also exhibit pathology associated with Alzheimer\'s disease (AD) (i.e. hyperphosphorylated tau and amyloid β (Aβ). Aβ can be deposited in the walls of blood vessels in the brains of individuals with AD, termed cerebral amyloid angiopathy (CAA). The aim of this study was to investigate the type and distribution of CAA in DLB, PDD, and PD and determine if this differs from AD. CAA type, severity, and topographical distribution was assessed in 94 AD, 30 DLB, 17 PDD, and 11 PD cases, and APOE genotype evaluated in a subset of cases where available. 96.3% AD cases, 70% DLB cases and 82.4% PDD cases exhibited CAA (type 1 or type 2). However only 45.5% PD cases had CAA. Type 1 CAA accounted for 37.2% of AD cases, 10% of DLB cases, and 5.9% of PDD cases, and was not observed in PD cases. There was a hierarchical topographical distribution in regions affected by CAA where AD and DLB displayed the same distribution pattern that differed from PDD and PD. APOE ε4 was associated with severity of CAA in AD cases. Topographical patterns and severity of CAA in DLB more closely resembled AD rather than PDD, and as type 1 CAA is associated with clinical dementia in AD, further investigations are warranted into whether the increased presence of type 1 CAA in DLB compared to PDD are related to the onset of cognitive symptoms and is a distinguishing factor between LBDs. Possible alignment of the the topographical distribution of CAA and microbleeds in DLB warrants further investigation. CAA in DLB more closely resembles AD rather than PDD or PD, and should be taken into consideration when stratifying patients for clinical trials or designing disease modifying therapies.
摘要：
路易体痴呆（DLB），帕金森病痴呆（PDD），和帕金森病(PD)统称为路易体病(LBD),其神经病理学特征在于α-突触核蛋白沉积(路易体和路易神经突)。然而,LBD还表现出与阿尔茨海默病(AD)相关的病理学(即过度磷酸化的tau和淀粉样蛋白β(Aβ)。Aβ可以沉积在AD患者大脑的血管壁中，称为脑淀粉样血管病（CAA）。本研究的目的是调查DLB中CAA的类型和分布。PDD,和PD，并确定这是否不同于AD。CAA类型,严重程度,在公元94年评估了地形分布，30DLB,17PDD,和11例PD病例，和APOE基因型在可用病例的子集进行评估。96.3%AD病例，70%的DLB病例和82.4%的PDD病例表现为CAA(1型或2型)。然而，只有45.5%的PD病例患有CAA。1型CAA占AD病例的37.2%,10%的DLB病例，和5.9%的PDD病例，在PD病例中未观察到。在受CAA影响的区域中存在分层的地形分布，其中AD和DLB显示出与PDD和PD不同的相同分布模式。APOEε4与AD患者的CAA严重程度相关。DLB中CAA的地形模式和严重程度更类似于AD而不是PDD，由于1型CAA与AD的临床痴呆有关，与PDD相比,DLB中1型CAA的增加是否与认知症状的发作有关,并且是LBD之间的区别因素,因此需要进一步研究.CAA的地形分布和DLB中微出血的可能对齐值得进一步研究。DLB中的CAA更类似于AD，而不是PDD或PD，并且在对患者进行分层以进行临床试验或设计疾病改善疗法时应予以考虑。
