Cerebellar presentation of multiple system atrophy
Identifieur interne : 002B39 ( Istex/Corpus ); précédent : 002B38; suivant : 002B40Cerebellar presentation of multiple system atrophy
Auteurs : G. K. Wenning ; E. Kraft ; R. Beck ; C. J. Fowler ; C. J. Mathias ; Quinn ; A. E. HardingSource :
- Movement Disorders [ 0885-3185 ] ; 1997-01.
English descriptors
Abstract
Early diagnosis of multiple‐system atrophy (MSA) is important in patients presenting with late‐onset cerebellar ataxia because it has a less favourable prognosis than other degenerative ataxic disorders. We report cerebellar presentation of MSA in a series of 16 patients, 3 of whom later developed parkinsonism. Two‐thirds of them had early evidence of impaired postural reflexes with a history of recurrent falls. Some of these had a narrow‐based, unsteady gait, unlike the more classic broad‐based gait ataxia of cerebellar disease. On review of the patients' histories, genitourinary dysfunction (particularly impotence) was present at the onset of, or preceding, cerebellar ataxia in 60% of patients, but this had often been attributed to age, or to urological or gynaecological causes. External striated anal or urethral sphincter electromyography (EMG) demonstrated features of chronic denervation and reinnervation in 14 (93%) of 15 patients, consistent with degeneration in Onuf's nucleus as occurs in MSA. Autonomic function tests were abnormal in 9 (64%) of 14 patients. Our data suggest that close enquiry into genitourinary function and analysis of the gait disorder can be useful pointers to a diagnosis of MSA in patients with an unexplained adult‐onset progressive cerebellar syndrome, and that sphincter EMG is the most useful investigation in this context.
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DOI: 10.1002/mds.870120121
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<affiliation xml:id="af3" countryCode="GB" type="organization"><unparsedAffiliation>Autonomic Unit, National Hospital for Neurology and Neurosurgery, London, England</unparsedAffiliation>
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<keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">Multiple system atrophy</keyword>
<keyword xml:id="kwd2">Cerebellar ataxia</keyword>
<keyword xml:id="kwd3">Autonomic failure</keyword>
<keyword xml:id="kwd4">Urinary dysfunction</keyword>
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<abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title>
<p>Early diagnosis of multiple‐system atrophy (MSA) is important in patients presenting with late‐onset cerebellar ataxia because it has a less favourable prognosis than other degenerative ataxic disorders. We report cerebellar presentation of MSA in a series of 16 patients, 3 of whom later developed parkinsonism. Two‐thirds of them had early evidence of impaired postural reflexes with a history of recurrent falls. Some of these had a narrow‐based, unsteady gait, unlike the more classic broad‐based gait ataxia of cerebellar disease. On review of the patients' histories, genitourinary dysfunction (particularly impotence) was present at the onset of, or preceding, cerebellar ataxia in 60% of patients, but this had often been attributed to age, or to urological or gynaecological causes. External striated anal or urethral sphincter electromyography (EMG) demonstrated features of chronic denervation and reinnervation in 14 (93%) of 15 patients, consistent with degeneration in Onuf's nucleus as occurs in MSA. Autonomic function tests were abnormal in 9 (64%) of 14 patients. Our data suggest that close enquiry into genitourinary function and analysis of the gait disorder can be useful pointers to a diagnosis of MSA in patients with an unexplained adult‐onset progressive cerebellar syndrome, and that sphincter EMG is the most useful investigation in this context.</p>
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<noteGroup><note xml:id="fn1"><p>Deceased</p>
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<!--Version 0.6 générée le 3-12-2015--><mods version="3.6"><titleInfo lang="en"><title>Cerebellar presentation of multiple system atrophy</title>
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<titleInfo type="abbreviated" lang="en"><title>CEREBELLAR PRESENTATION OF MSA</title>
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<titleInfo type="alternative" contentType="CDATA" lang="en"><title>Cerebellar presentation of multiple system atrophy</title>
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<name type="personal"><namePart type="given">G. K.</namePart>
<namePart type="family">Wenning</namePart>
<affiliation>University Deparment of Clinical Neurology, Institute of Neurology, Hospital for Neurology and Neurosurgery, London, England</affiliation>
<role><roleTerm type="text">author</roleTerm>
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</name>
<name type="personal"><namePart type="given">E.</namePart>
<namePart type="family">Kraft</namePart>
<affiliation>University Deparment of Clinical Neurology, Institute of Neurology, Hospital for Neurology and Neurosurgery, London, England</affiliation>
<role><roleTerm type="text">author</roleTerm>
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<name type="personal"><namePart type="given">R.</namePart>
<namePart type="family">Beck</namePart>
<affiliation>Department of Uro‐neurology, Hospital for Neurology and Neurosurgery, London, England</affiliation>
<role><roleTerm type="text">author</roleTerm>
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<name type="personal"><namePart type="given">C. J.</namePart>
<namePart type="family">Fowler</namePart>
<affiliation>Department of Uro‐neurology, Hospital for Neurology and Neurosurgery, London, England</affiliation>
<role><roleTerm type="text">author</roleTerm>
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<name type="personal"><namePart type="given">C. J.</namePart>
<namePart type="family">Mathias</namePart>
<affiliation>Autonomic Unit, National Hospital for Neurology and Neurosurgery, London, England</affiliation>
<role><roleTerm type="text">author</roleTerm>
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<name type="personal"><namePart type="termsOfAddress">Dr.</namePart>
<namePart type="family">Quinn</namePart>
<affiliation>University Deparment of Clinical Neurology, Institute of Neurology, Hospital for Neurology and Neurosurgery, London, England</affiliation>
<description>Correspondence: University Department of Clinical Neurology, Institute of Neurology, London WC1N 3BG, England</description>
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<name type="personal"><namePart type="given">A. E.</namePart>
<namePart type="family">Harding</namePart>
<affiliation>University Deparment of Clinical Neurology, Institute of Neurology, Hospital for Neurology and Neurosurgery, London, England</affiliation>
<description>Deceased</description>
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<dateIssued encoding="w3cdtf">1997-01</dateIssued>
<dateCaptured encoding="w3cdtf">1995-01-03</dateCaptured>
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<abstract lang="en">Early diagnosis of multiple‐system atrophy (MSA) is important in patients presenting with late‐onset cerebellar ataxia because it has a less favourable prognosis than other degenerative ataxic disorders. We report cerebellar presentation of MSA in a series of 16 patients, 3 of whom later developed parkinsonism. Two‐thirds of them had early evidence of impaired postural reflexes with a history of recurrent falls. Some of these had a narrow‐based, unsteady gait, unlike the more classic broad‐based gait ataxia of cerebellar disease. On review of the patients' histories, genitourinary dysfunction (particularly impotence) was present at the onset of, or preceding, cerebellar ataxia in 60% of patients, but this had often been attributed to age, or to urological or gynaecological causes. External striated anal or urethral sphincter electromyography (EMG) demonstrated features of chronic denervation and reinnervation in 14 (93%) of 15 patients, consistent with degeneration in Onuf's nucleus as occurs in MSA. Autonomic function tests were abnormal in 9 (64%) of 14 patients. Our data suggest that close enquiry into genitourinary function and analysis of the gait disorder can be useful pointers to a diagnosis of MSA in patients with an unexplained adult‐onset progressive cerebellar syndrome, and that sphincter EMG is the most useful investigation in this context.</abstract>
<subject lang="en"><genre>Keywords</genre>
<topic>Multiple system atrophy</topic>
<topic>Cerebellar ataxia</topic>
<topic>Autonomic failure</topic>
<topic>Urinary dysfunction</topic>
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<relatedItem type="host"><titleInfo><title>Movement Disorders</title>
<subTitle>Official Journal of the Movement Disorder Society</subTitle>
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<titleInfo type="abbreviated"><title>Mov. Disord.</title>
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<subject><genre>article category</genre>
<topic>Brief Report</topic>
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<identifier type="ISSN">0885-3185</identifier>
<identifier type="eISSN">1531-8257</identifier>
<identifier type="DOI">10.1002/(ISSN)1531-8257</identifier>
<identifier type="PublisherID">MDS</identifier>
<part><date>1997</date>
<detail type="volume"><caption>vol.</caption>
<number>12</number>
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<accessCondition type="use and reproduction" contentType="copyright">Copyright © 1997 Movement Disorder Society</accessCondition>
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