Wakabayashi Koichi, Takahashi Hitoshi
Department of Neuropathology, Institute of Brain Science, School of Medicine, Hirosaki University, Japan.
Neuropathology. 2006 Aug;26(4):338-45. doi: 10.1111/j.1440-1789.2006.00713.x.
Multiple system atrophy (MSA) is a sporadic, adult-onset neurodegenerative disease, which is characterized by striatonigral degeneration, olivopontocerebellar atrophy, and preganglionic autonomic lesions in any combination. The histological hallmark is the presence of argyrophilic fibrillary inclusions in the oligodendrocytes, referred to as glial cytoplasmic inclusions (GCIs). Fibrillary inclusions are also found in the neuronal somata, axons, and nucleus. Neuronal cytoplasmic inclusions are frequently found in the pontine and inferior olivary nuclei. Since the discovery of alpha-synuclein as a major component of glial and neuronal inclusions in MSA, two neurodegenerative processes have been considered in this disease: one is due to the widespread occurrence of GCIs associated with oligodendroglia-myelin degeneration (oligodendrogliopathy) in the central nervous system, and the other is due to the filamentous aggregation of alpha-synuclein in the neurons in several brain regions. These two degenerative processes might synergistically cause neuronal depletion in MSA.
多系统萎缩（MSA）是一种散发性成人起病的神经退行性疾病，其特征为纹状体黑质变性、橄榄脑桥小脑萎缩以及节前自主神经病变，这些病变可任意组合出现。组织学特征是少突胶质细胞中存在嗜银纤维性包涵体，称为胶质细胞质包涵体（GCIs）。在神经元胞体、轴突和细胞核中也发现有纤维性包涵体。神经元细胞质包涵体常见于脑桥和下橄榄核。自从发现α-突触核蛋白是MSA中胶质和神经元包涵体的主要成分以来，该病被认为存在两种神经退行性变过程：一种是由于中枢神经系统中与少突胶质细胞-髓鞘变性（少突胶质细胞病变）相关的GCIs广泛出现，另一种是由于α-突触核蛋白在几个脑区的神经元中发生丝状聚集。这两种退行性变过程可能协同导致MSA中的神经元缺失。
