教育講演4―1
多系統萎縮症の変性のはじまりは神経細胞からかグリア細胞からか
多系統萎縮症―シヌクレインと神経細胞変性―
吉田 眞理
愛知医科大学加齢医科学研究所〔〒480―1195 愛知県愛知郡長久手町大字岩作字雁又21〕
Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder that encompasses olivopontocerebellar atrophy (OPCA), striatonigral degeneration (SND) and Shy-Drager syndrome (SDS). The histopathological hallmarks are α-synuclein (AS) positive glial cytoplasmic inclusions (GCIs) in oligodendroglias. AS aggregation is also found in glial nuclear inclusions (GNIs), neuronal cytoplasmic inclusions (NCIs), neuronal nuclear inclusions (NNIs) and dystrophic neurties. Reviewing the pathological features of 102 MSA cases, OPCA-type was relatively more frequent and SND-type was less frequent in Japanese MSA cases, which suggested different phenotypic pattern of MSA might exist between races, compared to the relatively high frequency of SND-type in western countries. In early stage of MSA, NNIs, NCIs and diffuse homogenous stain of AS in neuronal nuclei and cytoplasm were observed in various vulnerable lesions including the pontine nuclei, putamen, substantia nigra, locus ceruleus, inferior olivary nucleus, intermediolateral column of thoracic cord, lower motor neurons and cortical pyramidal neurons, in additions to GCIs. These findings indicated that the primary nonfibrillar and fibrillar AS aggregation also occurred in neurons. Therefore both the direct involvement of neurons themselves and the oligodendroglia-myelin-axon mechanism may synergistically accelerate the degenerative process of MSA.
Full Text of this Article in Japanese PDF (494K)
(臨床神経, 51：838−842, 2011)
key words：多系統萎縮症，α-シヌクレイン，glial cytoplasmic inclusion（GCI），神経細胞核内封入体，神経細胞胞体内封入体
（受付日：2011年5月18日）
