Movement Disorders (revue)

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Multiple system atrophy: An update

Identifieur interne : 004032 ( Main/Exploration ); précédent : 004031; suivant : 004033

Multiple system atrophy: An update

Auteurs : Gregor K. Wenning [Autriche] ; Felix Geser [Autriche] ; Michaela Stampfer-Kountchev [Autriche] ; François Tison [Autriche]

Source :

RBID : ISTEX:E4A65AD7BF3DD5CB1D2BDC0A82AF58121D1F120F

Descripteurs français

English descriptors

Abstract

Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder that usually manifests in the early sixth decade of life and progresses relentlessly with a mean survival of 9 years. Clinically, MSA is dominated by autonomic/urogenital failure, which may be associated with either levodopa (L‐dopa) ‐unresponsive parkinsonism in 80% of cases (MSA‐P subtype) or with cerebellar ataxia in 20% of cases (MSA‐C subtype). Pathologically, MSA is characterized by a neuronal multisystem degeneration and abnormal glial cytoplasmic inclusions containing α‐synuclein aggregates. Pharmacological treatment of motor features is disappointing except for a transient L‐dopa response in a minority of MSA‐P patients. In contrast, autonomic and urogenital features of MSA should be identified early on, because they can be treated effectively in many instances. Neuroprotective strategies are presently unavailable, however, two multicentre European trials have been launched to evaluate the effects of riluzole and human recombinant growth hormone on disease progression in MSA. Clearly, further randomised, controlled trials are required to identify effective symptomatic or neuroprotective agents in MSA. Several in vivo models have become available to allow a careful preselection of candidate agents. Several research groups have been formed in Europe (EMSA‐SG, NNIPPS) and United States (NAMSA‐SG), providing a framework for coordinated trial activity in MSA. © 2003 Movement Disorder Society

Url:
DOI: 10.1002/mds.10561


Affiliations:


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<div type="abstract" xml:lang="en">Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder that usually manifests in the early sixth decade of life and progresses relentlessly with a mean survival of 9 years. Clinically, MSA is dominated by autonomic/urogenital failure, which may be associated with either levodopa (L‐dopa) ‐unresponsive parkinsonism in 80% of cases (MSA‐P subtype) or with cerebellar ataxia in 20% of cases (MSA‐C subtype). Pathologically, MSA is characterized by a neuronal multisystem degeneration and abnormal glial cytoplasmic inclusions containing α‐synuclein aggregates. Pharmacological treatment of motor features is disappointing except for a transient L‐dopa response in a minority of MSA‐P patients. In contrast, autonomic and urogenital features of MSA should be identified early on, because they can be treated effectively in many instances. Neuroprotective strategies are presently unavailable, however, two multicentre European trials have been launched to evaluate the effects of riluzole and human recombinant growth hormone on disease progression in MSA. Clearly, further randomised, controlled trials are required to identify effective symptomatic or neuroprotective agents in MSA. Several in vivo models have become available to allow a careful preselection of candidate agents. Several research groups have been formed in Europe (EMSA‐SG, NNIPPS) and United States (NAMSA‐SG), providing a framework for coordinated trial activity in MSA. © 2003 Movement Disorder Society</div>
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