An update on the cerebellar subtype of multiple system atrophy

Cerebellum & Ataxias
Ludovico CiolliGregor K Wenning

Abstract

Multiple system atrophy is a rare and fatal neurodegenerative disorder characterized by progressive autonomic failure, ataxia and parkinsonism in any combination. The clinical manifestations reflect central autonomic and striatonigral degeneration as well as olivopontocerebellar atrophy. Glial cytoplasmic inclusions, composed of α-synuclein and other proteins are considered the cellular hallmark lesion. The cerebellar variant of MSA (MSA-C) denotes a distinctive motor subtype characterized by progressive adult onset sporadic gait ataxia, scanning dysarthria, limb ataxia and cerebellar oculomotor dysfunction. In addition, there is autonomic failure and variable degrees of parkinsonism. A range of other disorders may present with MSA-C like features and therefore the differential diagnosis of MSA-C is not always straightforward. Here we review key aspects of MSA-C including pathology, pathogenesis, diagnosis, clinical features and treatment, paying special attention to differential diagnosis in late onset sporadic cerebellar ataxias.

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Citations

Jun 20, 2015·Journal of Movement Disorders·Ruth H Walker
Sep 30, 2017·Archives of Clinical Neuropsychology : the Official Journal of the National Academy of Neuropsychologists·Adam Gerstenecker
Nov 11, 2014·The Cerebellum·Leonardo BaldarçaraChristophe Habas
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Jan 16, 2018·Contrast Media & Molecular Imaging·Young Jin JeongJae Woo Kim

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Methods Mentioned

BETA
laser-capture microdissection
transgenic

Software Mentioned

Fine
STR

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