Volume 29, Issue 7 p. 857-867
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Cognitive impairment in multiple system atrophy: A position statement by the neuropsychology task force of the MDS multiple system atrophy (MODIMSA) study group

Iva Stankovic MD

Iva Stankovic MD

Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia

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Florian Krismer MD

Florian Krismer MD

Department of Neurology, Innsbruck Medical University, Austria

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Aleksandar Jesic MD

Aleksandar Jesic MD

Neurology Clinic, Clinical Centre of Vojvodina, School of Medicine Novi Sad, University of Novi Sad, Novi Sad, Serbia

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Angelo Antonini MD, PhD

Angelo Antonini MD, PhD

Department for Parkinson's Disease, “Fondazione Ospedale San Camillo,” I.R.C.C.S., Venice, Italy

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Thomas Benke MD

Thomas Benke MD

Department of Neurology, Innsbruck Medical University, Austria

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Richard G. Brown MD, PhD

Richard G. Brown MD, PhD

King's College London, Institute of Psychiatry, Department of Psychology, London, United Kingdom

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David J. Burn MD, FRCP

David J. Burn MD, FRCP

Institute for Ageing and Health, Newcastle University, Newcastle upon Tyne, United Kingdom

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Janice L. Holton FRCPath, PhD

Janice L. Holton FRCPath, PhD

Queen Square Brain Bank for Neurological Disorders, Institute of Neurology, University College London, London, United Kingdom

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Horacio Kaufmann MD

Horacio Kaufmann MD

Dysautonomia Center, Langone Medical Center, New York University, New York, USA

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Vladimir S. Kostic MD, PhD

Vladimir S. Kostic MD, PhD

Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia

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Helen Ling BScMed, BMBS, MSc

Helen Ling BScMed, BMBS, MSc

Queen Square Brain Bank for Neurological Disorders, Institute of Neurology, University College London, London, United Kingdom

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Wassilios G. Meissner MD, PhD

Wassilios G. Meissner MD, PhD

Univ. de Bordeaux, Institut des Maladies Neurodégénératives, Bordeaux, France

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Werner Poewe MD

Werner Poewe MD

Department of Neurology, Innsbruck Medical University, Austria

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Marija Semnic MD

Marija Semnic MD

Neurology Clinic, Clinical Centre of Vojvodina, School of Medicine Novi Sad, University of Novi Sad, Novi Sad, Serbia

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Klaus Seppi MD

Klaus Seppi MD

Department of Neurology, Innsbruck Medical University, Austria

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Atsushi Takeda MD, PhD

Atsushi Takeda MD, PhD

Department of Neurology, National Hospital Organization Sendai Nishitaga Hospital, Sendai, Japan

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Daniel Weintraub MD

Daniel Weintraub MD

Departments of Psychiatry and Neurology, Perelman School of Medicine at the University of Pennsylvania, and Parkinson's Disease Research, Education and Clinical Center, Philadelphia Veterans Affairs Medical Center, Philadelphia, Pennsylvania, USA

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Gregor K. Wenning MD, PhD, MSc

Corresponding Author

Gregor K. Wenning MD, PhD, MSc

Department of Neurology, Innsbruck Medical University, Austria

Correspondence to: Dr. Gregor K. Wenning, Department of Neurology, Medical University, Anichstrasse 35, A-6020 Innsbruck, Austria, E-mail: [email protected]Search for more papers by this author
on behalf of the Movement Disorders Society MSA (MODIMSA) Study Group

on behalf of the Movement Disorders Society MSA (MODIMSA) Study Group

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First published: 18 April 2014
Citations: 181

The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health.

Funding agencies: This study was supported by funds of the Austrian Science Fund (FWF): F04404-B19. Dr. Brown acknowledges support from the National Institute for Health Research (NIHR) Mental Health Biomedical Research Centre and Dementia Biomedical Research Unit at South London, and Maudsley NHS Foundation Trust and King's College London.

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

Consensus diagnostic criteria for multiple system atrophy consider dementia as a nonsupporting feature, despite emerging evidence demonstrating that cognitive impairments are an integral part of the disease. Cognitive disturbances in multiple system atrophy occur across a wide spectrum from mild single domain deficits to impairments in multiple domains and even to frank dementia in some cases. Frontal-executive dysfunction is the most common presentation, while memory and visuospatial functions also may be impaired. Imaging and neuropathological findings support the concept that cognitive impairments in MSA originate from striatofrontal deafferentation, with additional contributions from intrinsic cortical degeneration and cerebellar pathology. Based on a comprehensive evidence-based review, the authors propose future avenues of research that ultimately may lead to diagnostic criteria for cognitive impairment and dementia associated with multiple system atrophy. © 2014 International Parkinson and Movement Disorder Society