title: Neuropathological features of multiple system atrophy with cognitive impairment.
abstract: Cognitive impairment (CI) is an exclusion criterion for the diagnosis of multiple system atrophy (MSA), according to the second consensus statement. This view was recently challenged by patients with pathologically confirmed MSA who were reported to have dementia. With an aim to investigate the pathological substrate of CI in MSA, quantitative assessment of the glial and neuronal cytoplasmic inclusions and semiquantitative assessment of neuronal loss in the cortical and limbic regions was performed. No differences in the severity of these MSA-related pathological findings were identified between nine MSA cases with CI and nine MSA cases with normal cognition. Alzheimer's-related pathological changes, cerebral amyloid angiopathy, and cerebrovascular disease did not differ between the two MSA groups. MSA-specific α-synuclein and secondary pathological conditions were not more severe in MSA cases with CI, suggesting that although CI may be intrinsic to the MSA disease process, further investigation into the pathological basis of cognitive impairment in MSA is warranted.
authors.affiliation: Queen Square Brain Bank for Neurological Disorders, Department of Molecular Neuroscience, UCL Institute of Neurology, London, UK.
authors.forename: Y T
authors.initials: YT
authors.lastname: Asi
authors.forename: Helen
authors.initials: H
authors.lastname: Ling
authors.forename: Z
authors.initials: Z
authors.lastname: Ahmed
authors.forename: A J
authors.initials: AJ
authors.lastname: Lees
authors.forename: T
authors.initials: T
authors.lastname: Revesz
authors.forename: J L
authors.initials: JL
authors.lastname: Holton
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D001921:Brain / Q000473:pathology*; D003071:Cognition / Q000502:physiology*; D003072:Cognition Disorders / Q000209:etiology / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000150:complications / Q000473:pathology*; D009410:Nerve Degeneration / Q000150:complications / Q000473:pathology*; D009474:Neurons / Q000473:pathology*
keywords: MSA; cognitive impairment; dementia; neuropathology; α-synuclein