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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Beck</name><affiliation><mods:affiliation>Department of Uro‐neurology, Hospital for Neurology and Neurosurgery, London, England</mods:affiliation></affiliation></author><author><name sortKey="Fowler, C J" sort="Fowler, C J" uniqKey="Fowler C" first="C. J." last="Fowler">C. J. Fowler</name><affiliation><mods:affiliation>Department of Uro‐neurology, Hospital for Neurology and Neurosurgery, London, England</mods:affiliation></affiliation></author><author><name sortKey="Mathias, C J" sort="Mathias, C J" uniqKey="Mathias C" first="C. J." last="Mathias">C. J. 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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.</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>G. K. Wenning</name><affiliations><json:string>University Deparment of Clinical Neurology, Institute of Neurology, Hospital for Neurology and Neurosurgery, London, England</json:string></affiliations></json:item><json:item><name>E. Kraft</name><affiliations><json:string>University Deparment of Clinical Neurology, Institute of Neurology, Hospital for Neurology and Neurosurgery, London, England</json:string></affiliations></json:item><json:item><name>R. Beck</name><affiliations><json:string>Department of Uro‐neurology, Hospital for Neurology and Neurosurgery, London, England</json:string></affiliations></json:item><json:item><name>C. J. Fowler</name><affiliations><json:string>Department of Uro‐neurology, Hospital for Neurology and Neurosurgery, London, England</json:string></affiliations></json:item><json:item><name>C. J. Mathias</name><affiliations><json:string>Autonomic Unit, National Hospital for Neurology and Neurosurgery, London, England</json:string></affiliations></json:item><json:item><name>Dr. Quinn</name><affiliations><json:string>University Deparment of Clinical Neurology, Institute of Neurology, Hospital for Neurology and Neurosurgery, London, England</json:string></affiliations></json:item><json:item><name>A. E. 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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><qualityIndicators><score>4.651</score><pdfVersion>1.3</pdfVersion><pdfPageSize>612 x 827.759 pts</pdfPageSize><refBibsNative>true</refBibsNative><abstractCharCount>1374</abstractCharCount><pdfWordCount>2203</pdfWordCount><pdfCharCount>13623</pdfCharCount><pdfPageCount>3</pdfPageCount><abstractWordCount>204</abstractWordCount></qualityIndicators><title>Cerebellar presentation of multiple system atrophy</title><genre><json:string>Serial article</json:string></genre><host><volume>12</volume><pages><total>3</total><last>117</last><first>115</first></pages><issn><json:string>0885-3185</json:string></issn><issue>1</issue><subject><json:item><value>Brief Report</value></json:item></subject><genre></genre><language><json:string>unknown</json:string></language><title>Movement Disorders</title><doi><json:string>10.1002/(ISSN)1531-8257</json:string></doi></host><publicationDate>1997</publicationDate><copyrightDate>1997</copyrightDate><doi><json:string>10.1002/mds.870120121</json:string></doi><id>E257EF96081F1A007573E5215160BAE01173ECF4</id><fulltext><json:item><original>true</original><mimetype>application/pdf</mimetype><extension>pdf</extension><uri>https://api.istex.fr/document/E257EF96081F1A007573E5215160BAE01173ECF4/fulltext/pdf</uri></json:item><json:item><original>false</original><mimetype>application/zip</mimetype><extension>zip</extension><uri>https://api.istex.fr/document/E257EF96081F1A007573E5215160BAE01173ECF4/fulltext/zip</uri></json:item><istex:fulltextTEI uri="https://api.istex.fr/document/E257EF96081F1A007573E5215160BAE01173ECF4/fulltext/tei"><teiHeader type="text"><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Cerebellar presentation of multiple system atrophy</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><availability><p>Wiley Subscription Services, Inc., A Wiley Company</p></availability><date>1997</date></publicationStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Cerebellar presentation of multiple system atrophy</title><author><persName><forename type="first">G. 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Disord.</title><idno type="pISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><idno type="DOI">10.1002/(ISSN)1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="1997-01"></date><biblScope unit="vol">12</biblScope><biblScope unit="issue">1</biblScope><biblScope unit="page" from="115">115</biblScope><biblScope unit="page" to="117">117</biblScope></imprint></monogr><idno type="istex">E257EF96081F1A007573E5215160BAE01173ECF4</idno><idno type="DOI">10.1002/mds.870120121</idno><idno type="ArticleID">MDS870120121</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>1997</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><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></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>Multiple system atrophy</term></item><item><term>Cerebellar ataxia</term></item><item><term>Autonomic failure</term></item><item><term>Urinary dysfunction</term></item></list></keywords></textClass><textClass><keywords scheme="Journal Subject"><list><head>Article category</head><item><term>Brief Report</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="1995-01-03">Received</change><change when="1996-03-18">Registration</change><change when="1997-01">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/E257EF96081F1A007573E5215160BAE01173ECF4/fulltext/txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Wiley, elements deleted: body"><istex:xmlDeclaration>version="1.0" encoding="UTF-8" standalone="yes"</istex:xmlDeclaration><istex:document><component version="2.0" type="serialArticle" xml:lang="en"><header><publicationMeta level="product"><publisherInfo><publisherName>Wiley Subscription Services, Inc., A Wiley Company</publisherName><publisherLoc>Hoboken</publisherLoc></publisherInfo><doi registered="yes">10.1002/(ISSN)1531-8257</doi><issn type="print">0885-3185</issn><issn type="electronic">1531-8257</issn><idGroup><id type="product" value="MDS"></id></idGroup><titleGroup><title type="main" xml:lang="en" sort="MOVEMENT DISORDERS">Movement Disorders</title><title type="subtitle">Official Journal of the Movement Disorder Society</title><title type="short">Mov. 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K.</givenNames><familyName>Wenning</familyName></personName></creator><creator xml:id="au2" creatorRole="author" affiliationRef="#af1"><personName><givenNames>E.</givenNames><familyName>Kraft</familyName></personName></creator><creator xml:id="au3" creatorRole="author" affiliationRef="#af2"><personName><givenNames>R.</givenNames><familyName>Beck</familyName></personName></creator><creator xml:id="au4" creatorRole="author" affiliationRef="#af2"><personName><givenNames>C. J.</givenNames><familyName>Fowler</familyName></personName></creator><creator xml:id="au5" creatorRole="author" affiliationRef="#af3"><personName><givenNames>C. J.</givenNames><familyName>Mathias</familyName></personName></creator><creator xml:id="au6" creatorRole="author" affiliationRef="#af1" corresponding="yes"><personName><honorifics>Dr.</honorifics><givenNames>N. 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E.</givenNames><familyName>Harding</familyName></personName></creator></creators><affiliationGroup><affiliation xml:id="af1" countryCode="GB" type="organization"><unparsedAffiliation>University Deparment of Clinical Neurology, Institute of Neurology, Hospital for Neurology and Neurosurgery, London, England</unparsedAffiliation></affiliation><affiliation xml:id="af2" countryCode="GB" type="organization"><unparsedAffiliation>Department of Uro‐neurology, Hospital for Neurology and Neurosurgery, London, England</unparsedAffiliation></affiliation><affiliation xml:id="af3" countryCode="GB" type="organization"><unparsedAffiliation>Autonomic Unit, National Hospital for Neurology and Neurosurgery, London, England</unparsedAffiliation></affiliation></affiliationGroup><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></keywordGroup><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></abstract></abstractGroup></contentMeta><noteGroup><note xml:id="fn1"><p>Deceased</p></note></noteGroup></header></component></istex:document></istex:metadataXml><!--Version 0.6 générée le 3-12-2015--><mods version="3.6"><titleInfo lang="en"><title>Cerebellar presentation of multiple system atrophy</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>CEREBELLAR PRESENTATION OF MSA</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Cerebellar presentation of multiple system atrophy</title></titleInfo><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></role></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></role></name><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></role></name><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></role></name><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></role></name><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><role><roleTerm type="text">author</roleTerm></role></name><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><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre authority="originalCategForm">article</genre><originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><place><placeTerm type="text">Hoboken</placeTerm></place><dateIssued encoding="w3cdtf">1997-01</dateIssued><dateCaptured encoding="w3cdtf">1995-01-03</dateCaptured><dateValid encoding="w3cdtf">1996-03-18</dateValid><copyrightDate encoding="w3cdtf">1997</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType><extent unit="figures">1</extent><extent unit="tables">1</extent><extent unit="references">13</extent></physicalDescription><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></subject><relatedItem type="host"><titleInfo><title>Movement Disorders</title><subTitle>Official Journal of the Movement Disorder Society</subTitle></titleInfo><titleInfo type="abbreviated"><title>Mov. Disord.</title></titleInfo><subject><genre>article category</genre><topic>Brief Report</topic></subject><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></detail><detail type="issue"><caption>no.</caption><number>1</number></detail><extent unit="pages"><start>115</start><end>117</end><total>3</total></extent></part></relatedItem><identifier type="istex">E257EF96081F1A007573E5215160BAE01173ECF4</identifier><identifier type="DOI">10.1002/mds.870120121</identifier><identifier type="ArticleID">MDS870120121</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 1997 Movement Disorder Society</accessCondition><recordInfo><recordOrigin>Wiley Subscription Services, Inc., A Wiley Company</recordOrigin><recordContentSource>WILEY</recordContentSource></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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