Journal
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
Volume 62, Issue 8, Pages 812-819Publisher
AMER ASSN NEUROPATHOLOGISTS INC
DOI: 10.1093/jnen/62.8.812
Keywords
alpha-synuclein; alpha-synucleinopathy; cerebellum; dementia with Lewy bodies; multiple system atrophy; Parkinson disease; Purkinje cell
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alpha-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 alpha-synucleinopathies. Cerebellar degeneration with Purkinie 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-alpha-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 alpha-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 alpha-synuclein. Although no abnormal accumulation of alpha-synuclein was noted in the Purkinje cell somata, numerous alpha-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 alpha-synuclein pathology in PD and DLB, but not in MSA.
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