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Quantitative pathological changes in the cerebellum of multiple system atrophy

机译：多系统萎缩小脑的定量病理变化

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Multiple system atrophy (MSA) is a rare neurodegenerative disorder associated with parkinsonism, ataxia, and autonomic dysfunction. Its pathology is primarily subcortical comprising vacuolation, neuronal loss, gliosis, and alpha-synuclein-immunoreactive glial cytoplasmic inclusions (GC). To quantify cerebellar pathology in MSA, the density and spatial pattern of the pathological changes were studied in alpha-synuclein-immunolabelled sections of the cerebellar hemisphere in 10 MSA and 10 control cases. In MSA, densities of Purkinje cells (PC) were decreased and vacuoles in the granule cell layer (GL) increased compared with controls. In six MSA cases, GO were present in cerebellar white matter. In the molecular layer (ML) and GL of MSA, vacuoles were clustered, the clusters exhibiting a regular distribution parallel to the edge of the folia. Purkinje cells were randomly or regularly distributed with large gaps between surviving cells. Densities of glial cells and surviving neurons in the ML and surviving cells and vacuoles in the GL were negatively correlated consistent with gliosis and vacuolation in response to neuronal loss. Principal components analysis (PCA) suggested vacuole densities in the ML and vacuole density and cell losses in the GL were the main source of neuropathological variation among cases. The data suggest that: (1) cell losses and vacuolation of the GCL and loss of PC were the most significant pathological changes in the cases studied, (2) pathological changes were topographically distributed, and (3) cerebellar pathology could influence cerebral function in MSA via the cerebello-dentato-thalamic tract.
机译：多系统萎缩症（MSA）是与帕金森氏症，共济失调和自主神经功能障碍相关的罕见神经退行性疾病。其病理学主要是皮层下，包括空泡，神经元丢失，神经胶质增生和α-突触核蛋白免疫反应性神经胶质细胞质包涵体（GC）。为了量化MSA中的小脑病理，在10例MSA和10例对照病例中，在小脑半球的α-突触核蛋白免疫接种切片中研究了病理变化的密度和空间模式。在MSA中，与对照组相比，浦肯野细胞（PC）的密度降低，颗粒细胞层（GL）中的液泡增加。在6例MSA病例中，GO存在于小脑白质中。在MSA的分子层（ML）和GL中，液泡成簇，这些簇表现出平行于叶边缘的规则分布。浦肯野细胞随机或规则分布，存活的细胞之间有较大的间隙。 ML中神经胶质细胞和存活神经元的密度与GL中存活细胞和液泡的密度呈负相关，与神经胶质细胞增生和液泡响应神经元丢失相关。主成分分析（PCA）表明，ML中的液泡密度，GL中的液泡密度和细胞损失是病例间神经病理学变化的主要来源。数据表明：（1）在研究的病例中，细胞丢失，GCL空泡和PC丢失是最主要的病理变化;（2）病理变化在地形上是分布的;（3）小脑病理可能会影响脑功能。通过小脑-齿状-丘脑道的MSA。

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