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Fumiaki Mori, Yue-Shan Piao, Shintaro Hayashi, Hideo Fujiwara, Masato Hasegawa, Makoto Yoshimoto, Takeshi Iwatsubo, Hitoshi Takahashi, Koichi Wakabayashi, α-Synuclein Accumulates in Purkinje Cells in Lewy Body Disease but not in Multiple System Atrophy, Journal of Neuropathology & Experimental Neurology, Volume 62, Issue 8, August 2003, Pages 812–819, https://doi.org/10.1093/jnen/62.8.812
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Abstract
α-Synuclein has an important role in the pathogenesis of Parkinson disease (PD), dementia with Lewy bodies (DLB), and multiple system atrophy (MSA), comprising a new disease concept, that of α-synucleinopathies. Cerebellar degeneration with Purkinje cell depletion is present in the majority of MSA cases. By contrast, cerebellar pathology has not been demonstrated unequivocally in either PD or DLB. Recent immunohistochemical studies using anti-α-synuclein antibodies have shown that LB-type degeneration in PD and DLB is more widespread than previously recognized. To determine whether cerebellar Purkinje cells might be involved in α-synuclein pathology, we carried out immunohistochemical examinations of the cerebella of patients with PD (n = 10), DLB (n = 7), MSA (n = 10), Alzheimer disease and other tauopathies (n = 9), and age-matched control subjects (n = 10), using antibodies specific for α-synuclein. Although no abnormal accumulation of α-synuclein was noted in the Purkinje cell somata, numerous α-synuclein-positive, round inclusions were found in the cerebellar white matter in all the patients with PD and DLB. Immunohistochemical and ultrastructural examinations revealed that the majority of these inclusions was located in the Purkinje cell axons and consisted of granulo-filamentous structures. No such inclusions were observed in MSA, tauopathies, or controls. These findings indicate that Purkinje cells are also the victims of α-synuclein pathology in PD and DLB, but not in MSA.
