title: An autopsy case of preclinical multiple system atrophy (MSA-C).
abstract: Multiple system atrophy (MSA) is divided into two clinical subtypes: MSA with predominant parkinsonian features (MSA-P) and MSA with predominant cerebellar dysfunction (MSA-C). We report a 71-year-old Japanese man without clinical signs of MSA, in whom post mortem examination revealed only slight gliosis in the pontine base and widespread occurrence of glial cytoplasmic inclusions in the central nervous system, with the greatest abundance in the pontine base and cerebellar white matter. Neuronal cytoplasmic inclusions (NCIs) and neuronal nuclear inclusions (NNIs) were almost restricted to the pontine and inferior olivary nuclei. It was noteworthy that most NCIs were located in the perinuclear area, and the majority of NNIs were observed adjacent to the inner surface of the nuclear membrane. To our knowledge, only four autopsy cases of preclinical MSA have been reported previously, in which neuronal loss was almost entirely restricted to the substantia nigra and/or putamen. Therefore, the present autopsy case of preclinical MSA-C is considered to be the first of its kind to have been reported. The histopathological features observed in preclinical MSA may represent the early pattern of MSA pathology.
authors.affiliation: Department of Neuropathology, Hirosaki University Graduate School of Medicine, Hirosaki, Japan; Department of Neurology, Aomori Prefectural Central Hospital, Aomori, Japan.
authors.forename: Tomoya
authors.initials: T
authors.lastname: Kon
authors.forename: Fumiaki
authors.initials: F
authors.lastname: Mori
authors.forename: Kunikazu
authors.initials: K
authors.lastname: Tanji
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Miki
authors.forename: Koichi
authors.initials: K
authors.lastname: Wakabayashi
mesh_terms: D000368:Aged; D001344:Autopsy; D001921:Brain / Q000473:pathology*; D006801:Humans; D033162:Incidental Findings; D002479:Inclusion Bodies / Q000473:pathology*; D008297:Male; D019578:Multiple System Atrophy / Q000473:pathology*
keywords: glial cytoplasmic inclusion; multiple system atrophy; neuronal cytoplasmic inclusion; neuronal nuclear inclusion; α-synuclein