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An autopsy case of preclinical multiple system atrophy ( MSA ‐ C )
Author(s) -
Kon Tomoya,
Mori Fumiaki,
Tanji Kunikazu,
Miki Yasuo,
Wakabayashi Koichi
Publication year - 2013
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/neup.12037
Subject(s) - medicine
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.