Neuropathological features of multiple system atrophy with cognitive impairment

Authors

  • Y. T. Asi MSc, PhD,

    1. Queen Square Brain Bank for Neurological Disorders, Department of Molecular Neuroscience, UCL Institute of Neurology, London
    Search for more papers by this author
    • Joint first authors.

  • Helen Ling MRCP, PhD,

    1. Queen Square Brain Bank for Neurological Disorders, Department of Molecular Neuroscience, UCL Institute of Neurology, London
    2. Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, London
    Search for more papers by this author
    • Joint first authors.

  • Z. Ahmed PhD,

    1. Queen Square Brain Bank for Neurological Disorders, Department of Molecular Neuroscience, UCL Institute of Neurology, London
    Search for more papers by this author
  • A. J. Lees MD, FRCP, FMedSci,

    1. Queen Square Brain Bank for Neurological Disorders, Department of Molecular Neuroscience, UCL Institute of Neurology, London
    2. Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, London
    Search for more papers by this author
  • T. Revesz MD, FRCPath,

    1. Queen Square Brain Bank for Neurological Disorders, Department of Molecular Neuroscience, UCL Institute of Neurology, London
    Search for more papers by this author
  • J. L. Holton PhD, FRCPath

    Corresponding author
    1. Queen Square Brain Bank for Neurological Disorders, Department of Molecular Neuroscience, UCL Institute of Neurology, London
    • Correspondence to: Dr Janice Holton MBChB, PhD, FRCPath, Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London WC1N 3BG; janice.holton@ucl.ac.uk

    Search for more papers by this author

  • Funding agencies: This study was supported by grants from the Multiple System Atrophy Trust, the Reta Lila Weston Institute of Neurological Studies and Kuwait Ministry of Higher Education.

  • Relevant conflicts of interest/financial disclosures: Nothing to report.

  • Full financial disclosures and author roles may be found in the online version of this article.

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. © 2014 International Parkinson and Movement Disorder Society

Ancillary