Cerebellar presentation of multiple system atrophy
Identifieur interne : 005392 ( Main/Exploration ); précédent : 005391; suivant : 005393Cerebellar presentation of multiple system atrophy
Auteurs : G. K. Wenning [Royaume-Uni] ; E. Kraft [Royaume-Uni] ; R. Beck [Royaume-Uni] ; C. J. Fowler [Royaume-Uni] ; C. J. Mathias [Royaume-Uni] ; Quinn [Royaume-Uni] ; A. E. Harding [Royaume-Uni]Source :
- Movement Disorders [ 0885-3185 ] ; 1997-01.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Homme.
English descriptors
- KwdEn :
- Adult, Aged, Anal sphincter, Ataxia, Autonomic Nervous System Diseases (diagnosis), Autonomic failure, Cerebellar Ataxia (diagnosis), Cerebellar ataxia, Cerebellum, Electromyography, Female, Human, Humans, Inaugural sign, Male, Middle Aged, Multiple system atrophy, Musculus sphincter urethral, Neurologic Examination, Olivopontocerebellar Atrophies (diagnosis), Parkinson Disease (diagnosis), Reflex, Abnormal (physiology), Urinary dysfunction, Urination Disorders (diagnosis).
- MESH :
- diagnosis : Autonomic Nervous System Diseases, Cerebellar Ataxia, Olivopontocerebellar Atrophies, Parkinson Disease, Urination Disorders.
- physiology : Reflex, Abnormal.
- Adult, Aged, Electromyography, Female, Humans, Male, Middle Aged, Neurologic Examination.
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.
Url:
DOI: 10.1002/mds.870120121
Affiliations:
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Le document en format XML
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K." last="Wenning">G. K. Wenning</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>University Deparment of Clinical Neurology, Institute of Neurology, Hospital for Neurology and Neurosurgery, London</wicri:cityArea></affiliation></author><author><name sortKey="Kraft, E" sort="Kraft, E" uniqKey="Kraft E" first="E." last="Kraft">E. Kraft</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>University Deparment of Clinical Neurology, Institute of Neurology, Hospital for Neurology and Neurosurgery, London</wicri:cityArea></affiliation></author><author><name sortKey="Beck, R" sort="Beck, R" uniqKey="Beck R" first="R." last="Beck">R. Beck</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>Department of Uro‐neurology, Hospital for Neurology and Neurosurgery, London</wicri:cityArea></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 wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>Department of Uro‐neurology, Hospital for Neurology and Neurosurgery, London</wicri:cityArea></affiliation></author><author><name sortKey="Mathias, C J" sort="Mathias, C J" uniqKey="Mathias C" first="C. J." last="Mathias">C. J. Mathias</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>Autonomic Unit, National Hospital for Neurology and Neurosurgery, London</wicri:cityArea></affiliation></author><author><name sortKey="Quinn" sort="Quinn" uniqKey="Quinn" last="Quinn">Quinn</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>University Deparment of Clinical Neurology, Institute of Neurology, Hospital for Neurology and Neurosurgery, London</wicri:cityArea></affiliation></author><author><name sortKey="Harding, A E" sort="Harding, A E" uniqKey="Harding A" first="A. E." last="Harding">A. E. Harding</name><affiliation wicri:level="2"><country>Royaume-Uni</country><placeName><region type="country">Angleterre</region></placeName><wicri:cityArea>University Deparment of Clinical Neurology, Institute of Neurology, Hospital for Neurology and Neurosurgery, London</wicri:cityArea></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</title><title level="j" type="sub">Official Journal of the Movement Disorder Society</title><title level="j" type="abbrev">Mov. Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="1997-01">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><idno type="ISSN">0885-3185</idno></series><idno type="istex">E257EF96081F1A007573E5215160BAE01173ECF4</idno><idno type="DOI">10.1002/mds.870120121</idno><idno type="ArticleID">MDS870120121</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Aged</term><term>Anal sphincter</term><term>Ataxia</term><term>Autonomic Nervous System Diseases (diagnosis)</term><term>Autonomic failure</term><term>Cerebellar Ataxia (diagnosis)</term><term>Cerebellar ataxia</term><term>Cerebellum</term><term>Electromyography</term><term>Female</term><term>Human</term><term>Humans</term><term>Inaugural sign</term><term>Male</term><term>Middle Aged</term><term>Multiple system atrophy</term><term>Musculus sphincter urethral</term><term>Neurologic Examination</term><term>Olivopontocerebellar Atrophies (diagnosis)</term><term>Parkinson Disease (diagnosis)</term><term>Reflex, Abnormal (physiology)</term><term>Urinary dysfunction</term><term>Urination Disorders (diagnosis)</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Autonomic Nervous System Diseases</term><term>Cerebellar Ataxia</term><term>Olivopontocerebellar Atrophies</term><term>Parkinson Disease</term><term>Urination Disorders</term></keywords><keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Reflex, Abnormal</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Aged</term><term>Electromyography</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Neurologic Examination</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Ataxie</term><term>Atrophie multisystématisée</term><term>Cervelet</term><term>Electromyographie</term><term>Homme</term><term>Signe inaugural</term><term>Sphincter anal</term><term>Sphincter strié urétral</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Homme</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml: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.</div></front></TEI><affiliations><list><country><li>Royaume-Uni</li></country><region><li>Angleterre</li></region></list><tree><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Wenning, G K" sort="Wenning, G K" uniqKey="Wenning G" first="G. K." last="Wenning">G. K. Wenning</name></region><name sortKey="Beck, R" sort="Beck, R" uniqKey="Beck R" first="R." last="Beck">R. Beck</name><name sortKey="Fowler, C J" sort="Fowler, C J" uniqKey="Fowler C" first="C. J." last="Fowler">C. J. Fowler</name><name sortKey="Harding, A E" sort="Harding, A E" uniqKey="Harding A" first="A. E." last="Harding">A. E. Harding</name><name sortKey="Kraft, E" sort="Kraft, E" uniqKey="Kraft E" first="E." last="Kraft">E. Kraft</name><name sortKey="Mathias, C J" sort="Mathias, C J" uniqKey="Mathias C" first="C. J." last="Mathias">C. J. Mathias</name><name sortKey="Quinn" sort="Quinn" uniqKey="Quinn" last="Quinn">Quinn</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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