Primary lateral sclerosis mimicking atypical parkinsonism
Identifieur interne : 001407 ( Istex/Corpus ); précédent : 001406; suivant : 001408Primary lateral sclerosis mimicking atypical parkinsonism
Auteurs : Ibrahim M. Norlinah ; Kailash P. Bhatia ; Karen Stergaard ; Robin Howard ; Gennarina Arabia ; Niall P. QuinnSource :
- Movement Disorders [ 0885-3185 ] ; 2007-10-31.
English descriptors
Abstract
Primary lateral sclerosis (PLS), the upper motor neurone variant of motor neurone disease, is characterized by progressive spinal or bulbar spasticity with minimal motor weakness. Rarely, PLS may present with clinical features resembling parkinsonism resulting in occasional misdiagnosis as one of the atypical parkinsonian syndromes. Here we describe five patients initially referred with a diagnosis of levodopa‐unresponsive atypical parkinsonism (n = 4) or primary progressive multiple sclerosis (n = 1), but subsequently found to have features consistent with PLS instead. Onset age varied from 49 to 67 years. Unilateral limb slowness or clumsiness was the initial complaint in four, and bulbar symptoms in one. Repeated finger/foot tapping was slow in all five, but without fatiguing or decrement. Spasticity with hyperreflexia, exaggerated jaw jerk and extensor plantar responses were eventually seen in all patients. Anterior horn cell involvement developed in three cases. Early gait disturbances resulting in falls were seen in all patients and none of them responded to dopaminergic medications. Two patients underwent dopamine transporter (DaT) SPECT scanning with normal results. Other features included emotional lability (n = 5) and cognitive impairment involving frontal subcortical systems (n = 1). In conclusion, these cases represent a subgroup of PLS patients in whom pyramidal slowness may be mistaken for akinesia, and spasticity misconstrued as rigidity, leading to an erroneous diagnosis of atypical parkinsonism. However, the absence of fatiguing and decrement on repeated finger/foot tapping should help to distinguish these patients from the true atypical parkinsonian syndromes. © 2007 Movement Disorder Society
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DOI: 10.1002/mds.21645
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ISTEX:6EE2C459D88B3BC7028060986EC265EFAA819B10Le document en format XML
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Quinn</name><affiliation><mods:affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</mods:affiliation></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="2007-10-31">2007-10-31</date><biblScope unit="vol">22</biblScope><biblScope unit="issue">14</biblScope><biblScope unit="page" from="2057">2057</biblScope><biblScope unit="page" to="2062">2062</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">6EE2C459D88B3BC7028060986EC265EFAA819B10</idno><idno type="DOI">10.1002/mds.21645</idno><idno type="ArticleID">MDS21645</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>akinesia</term><term>atypical parkinsonism</term><term>extrapyramidal</term><term>primary lateral sclerosis</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Primary lateral sclerosis (PLS), the upper motor neurone variant of motor neurone disease, is characterized by progressive spinal or bulbar spasticity with minimal motor weakness. Rarely, PLS may present with clinical features resembling parkinsonism resulting in occasional misdiagnosis as one of the atypical parkinsonian syndromes. Here we describe five patients initially referred with a diagnosis of levodopa‐unresponsive atypical parkinsonism (n = 4) or primary progressive multiple sclerosis (n = 1), but subsequently found to have features consistent with PLS instead. Onset age varied from 49 to 67 years. Unilateral limb slowness or clumsiness was the initial complaint in four, and bulbar symptoms in one. Repeated finger/foot tapping was slow in all five, but without fatiguing or decrement. Spasticity with hyperreflexia, exaggerated jaw jerk and extensor plantar responses were eventually seen in all patients. Anterior horn cell involvement developed in three cases. Early gait disturbances resulting in falls were seen in all patients and none of them responded to dopaminergic medications. Two patients underwent dopamine transporter (DaT) SPECT scanning with normal results. Other features included emotional lability (n = 5) and cognitive impairment involving frontal subcortical systems (n = 1). In conclusion, these cases represent a subgroup of PLS patients in whom pyramidal slowness may be mistaken for akinesia, and spasticity misconstrued as rigidity, leading to an erroneous diagnosis of atypical parkinsonism. However, the absence of fatiguing and decrement on repeated finger/foot tapping should help to distinguish these patients from the true atypical parkinsonian syndromes. © 2007 Movement Disorder Society</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Ibrahim M. Norlinah MB</name><affiliations><json:string>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</json:string><json:string>Neurology Unit, Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia</json:string></affiliations></json:item><json:item><name>Kailash P. Bhatia MD</name><affiliations><json:string>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</json:string></affiliations></json:item><json:item><name>Karen Østergaard MD</name><affiliations><json:string>Department of Neurology, Aarhus University Hospital, Aarhus C, Denmark</json:string></affiliations></json:item><json:item><name>Robin Howard PhD</name><affiliations><json:string>Department of Molecular Neuroscience, Institute of Neurology, London, United Kingdom</json:string></affiliations></json:item><json:item><name>Gennarina Arabia MD</name><affiliations><json:string>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</json:string></affiliations></json:item><json:item><name>Niall P. Quinn MD</name><affiliations><json:string>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>primary lateral sclerosis</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>atypical parkinsonism</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>akinesia</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>extrapyramidal</value></json:item></subject><language><json:string>eng</json:string></language><abstract>Primary lateral sclerosis (PLS), the upper motor neurone variant of motor neurone disease, is characterized by progressive spinal or bulbar spasticity with minimal motor weakness. Rarely, PLS may present with clinical features resembling parkinsonism resulting in occasional misdiagnosis as one of the atypical parkinsonian syndromes. Here we describe five patients initially referred with a diagnosis of levodopa‐unresponsive atypical parkinsonism (n = 4) or primary progressive multiple sclerosis (n = 1), but subsequently found to have features consistent with PLS instead. Onset age varied from 49 to 67 years. Unilateral limb slowness or clumsiness was the initial complaint in four, and bulbar symptoms in one. Repeated finger/foot tapping was slow in all five, but without fatiguing or decrement. Spasticity with hyperreflexia, exaggerated jaw jerk and extensor plantar responses were eventually seen in all patients. Anterior horn cell involvement developed in three cases. Early gait disturbances resulting in falls were seen in all patients and none of them responded to dopaminergic medications. Two patients underwent dopamine transporter (DaT) SPECT scanning with normal results. Other features included emotional lability (n = 5) and cognitive impairment involving frontal subcortical systems (n = 1). In conclusion, these cases represent a subgroup of PLS patients in whom pyramidal slowness may be mistaken for akinesia, and spasticity misconstrued as rigidity, leading to an erroneous diagnosis of atypical parkinsonism. However, the absence of fatiguing and decrement on repeated finger/foot tapping should help to distinguish these patients from the true atypical parkinsonian syndromes. © 2007 Movement Disorder Society</abstract><qualityIndicators><score>6.294</score><pdfVersion>1.3</pdfVersion><pdfPageSize>594 x 792 pts</pdfPageSize><refBibsNative>true</refBibsNative><abstractCharCount>1739</abstractCharCount><pdfWordCount>3306</pdfWordCount><pdfCharCount>21557</pdfCharCount><pdfPageCount>6</pdfPageCount><abstractWordCount>249</abstractWordCount></qualityIndicators><title>Primary lateral sclerosis mimicking atypical parkinsonism</title><genre><json:string>Serial article</json:string></genre><host><volume>22</volume><pages><total>6</total><last>2062</last><first>2057</first></pages><issn><json:string>0885-3185</json:string></issn><issue>14</issue><subject><json:item><value>Research Article</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>2007</publicationDate><copyrightDate>2007</copyrightDate><doi><json:string>10.1002/mds.21645</json:string></doi><id>6EE2C459D88B3BC7028060986EC265EFAA819B10</id><fulltext><json:item><original>true</original><mimetype>application/pdf</mimetype><extension>pdf</extension><uri>https://api.istex.fr/document/6EE2C459D88B3BC7028060986EC265EFAA819B10/fulltext/pdf</uri></json:item><json:item><original>false</original><mimetype>application/zip</mimetype><extension>zip</extension><uri>https://api.istex.fr/document/6EE2C459D88B3BC7028060986EC265EFAA819B10/fulltext/zip</uri></json:item><istex:fulltextTEI uri="https://api.istex.fr/document/6EE2C459D88B3BC7028060986EC265EFAA819B10/fulltext/tei"><teiHeader type="text"><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Primary lateral sclerosis mimicking atypical parkinsonism</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>2007</date></publicationStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Primary lateral sclerosis mimicking atypical parkinsonism</title><author><persName><forename type="first">Ibrahim M.</forename><surname>Norlinah</surname><roleName type="degree">MB</roleName></persName><affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</affiliation><affiliation>Neurology Unit, Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia</affiliation></author><author><persName><forename type="first">Kailash P.</forename><surname>Bhatia</surname><roleName type="degree">MD</roleName></persName><affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</affiliation></author><author><persName><forename type="first">Karen</forename><surname>Østergaard</surname><roleName type="degree">MD</roleName></persName><affiliation>Department of Neurology, Aarhus University Hospital, Aarhus C, Denmark</affiliation></author><author><persName><forename type="first">Robin</forename><surname>Howard</surname><roleName type="degree">PhD</roleName></persName><affiliation>Department of Molecular Neuroscience, Institute of Neurology, London, United Kingdom</affiliation></author><author><persName><forename type="first">Gennarina</forename><surname>Arabia</surname><roleName type="degree">MD</roleName></persName><affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</affiliation></author><author><persName><forename type="first">Niall P.</forename><surname>Quinn</surname><roleName type="degree">MD</roleName></persName><note type="correspondence"><p>Correspondence: Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London WC1N 3BG, United Kingdom</p></note><affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</affiliation></author></analytic><monogr><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="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="2007-10-31"></date><biblScope unit="vol">22</biblScope><biblScope unit="issue">14</biblScope><biblScope unit="page" from="2057">2057</biblScope><biblScope unit="page" to="2062">2062</biblScope></imprint></monogr><idno type="istex">6EE2C459D88B3BC7028060986EC265EFAA819B10</idno><idno type="DOI">10.1002/mds.21645</idno><idno type="ArticleID">MDS21645</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>2007</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>Primary lateral sclerosis (PLS), the upper motor neurone variant of motor neurone disease, is characterized by progressive spinal or bulbar spasticity with minimal motor weakness. Rarely, PLS may present with clinical features resembling parkinsonism resulting in occasional misdiagnosis as one of the atypical parkinsonian syndromes. Here we describe five patients initially referred with a diagnosis of levodopa‐unresponsive atypical parkinsonism (n = 4) or primary progressive multiple sclerosis (n = 1), but subsequently found to have features consistent with PLS instead. Onset age varied from 49 to 67 years. Unilateral limb slowness or clumsiness was the initial complaint in four, and bulbar symptoms in one. Repeated finger/foot tapping was slow in all five, but without fatiguing or decrement. Spasticity with hyperreflexia, exaggerated jaw jerk and extensor plantar responses were eventually seen in all patients. Anterior horn cell involvement developed in three cases. Early gait disturbances resulting in falls were seen in all patients and none of them responded to dopaminergic medications. Two patients underwent dopamine transporter (DaT) SPECT scanning with normal results. Other features included emotional lability (n = 5) and cognitive impairment involving frontal subcortical systems (n = 1). In conclusion, these cases represent a subgroup of PLS patients in whom pyramidal slowness may be mistaken for akinesia, and spasticity misconstrued as rigidity, leading to an erroneous diagnosis of atypical parkinsonism. However, the absence of fatiguing and decrement on repeated finger/foot tapping should help to distinguish these patients from the true atypical parkinsonian syndromes. © 2007 Movement Disorder Society</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>primary lateral sclerosis</term></item><item><term>atypical parkinsonism</term></item><item><term>akinesia</term></item><item><term>extrapyramidal</term></item></list></keywords></textClass><textClass><keywords scheme="Journal Subject"><list><head>Article category</head><item><term>Research Article</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="2006-12-15">Received</change><change when="2007-05-29">Registration</change><change when="2007-10-31">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/6EE2C459D88B3BC7028060986EC265EFAA819B10/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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href="file:MDS.MDS21645.pdf"></link></linkGroup></publicationMeta><contentMeta><countGroup><count type="figureTotal" number="0"></count><count type="tableTotal" number="3"></count><count type="referenceTotal" number="17"></count><count type="wordTotal" number="3809"></count></countGroup><titleGroup><title type="main" xml:lang="en">Primary lateral sclerosis mimicking atypical parkinsonism</title><title type="short" xml:lang="en">PLS Mimicking Atypical Parkinsonism</title></titleGroup><creators><creator xml:id="au1" creatorRole="author" affiliationRef="#af1 #af2"><personName><givenNames>Ibrahim M.</givenNames><familyName>Norlinah</familyName><degrees>MB</degrees></personName></creator><creator xml:id="au2" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Kailash P.</givenNames><familyName>Bhatia</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au3" creatorRole="author" affiliationRef="#af3"><personName><givenNames>Karen</givenNames><familyName>Østergaard</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au4" creatorRole="author" affiliationRef="#af4"><personName><givenNames>Robin</givenNames><familyName>Howard</familyName><degrees>PhD</degrees></personName></creator><creator xml:id="au5" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Gennarina</givenNames><familyName>Arabia</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au6" creatorRole="author" affiliationRef="#af1" corresponding="yes"><personName><givenNames>Niall P.</givenNames><familyName>Quinn</familyName><degrees>MD</degrees></personName><contactDetails><email>n.quinn@ion.ucl.ac.uk</email></contactDetails></creator></creators><affiliationGroup><affiliation xml:id="af1" countryCode="GB" type="organization"><unparsedAffiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</unparsedAffiliation></affiliation><affiliation xml:id="af2" countryCode="MY" type="organization"><unparsedAffiliation>Neurology Unit, Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia</unparsedAffiliation></affiliation><affiliation xml:id="af3" countryCode="DK" type="organization"><unparsedAffiliation>Department of Neurology, Aarhus University Hospital, Aarhus C, Denmark</unparsedAffiliation></affiliation><affiliation xml:id="af4" countryCode="GB" type="organization"><unparsedAffiliation>Department of Molecular Neuroscience, Institute of Neurology, London, United Kingdom</unparsedAffiliation></affiliation></affiliationGroup><keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">primary lateral sclerosis</keyword><keyword xml:id="kwd2">atypical parkinsonism</keyword><keyword xml:id="kwd3">akinesia</keyword><keyword xml:id="kwd4">extrapyramidal</keyword></keywordGroup><supportingInformation><p> This article includes supplementary video clips, available online at <url href="http://www.interscience.wiley.com/jpages/0885-3185/suppmat"> http://www.interscience.wiley.com/jpages/0885‐3185/suppmat </url> . </p><supportingInfoItem><mediaResource alt="supporting information" href="urn-x:wiley:08853185:media:mds21645:mds21645-MDS21645"></mediaResource><caption>Case 1There is slowness of finger taps but with no definite fatiguing or decrement. There is obvious wasting of the left first dorsal interosseus.Case 2Segment 1. Repeated finger taps are slow, but with no definite fatiguing or decrement.Segment 2. The patient walks with a narrow‐based, stiff gait. There is stooped posture with reduced arm swing bilaterally. There is postural instability on the pull test.</caption></supportingInfoItem></supportingInformation><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><p>Primary lateral sclerosis (PLS), the upper motor neurone variant of motor neurone disease, is characterized by progressive spinal or bulbar spasticity with minimal motor weakness. Rarely, PLS may present with clinical features resembling parkinsonism resulting in occasional misdiagnosis as one of the atypical parkinsonian syndromes. Here we describe five patients initially referred with a diagnosis of levodopa‐unresponsive atypical parkinsonism (n = 4) or primary progressive multiple sclerosis (n = 1), but subsequently found to have features consistent with PLS instead. Onset age varied from 49 to 67 years. Unilateral limb slowness or clumsiness was the initial complaint in four, and bulbar symptoms in one. Repeated finger/foot tapping was slow in all five, but without fatiguing or decrement. Spasticity with hyperreflexia, exaggerated jaw jerk and extensor plantar responses were eventually seen in all patients. Anterior horn cell involvement developed in three cases. Early gait disturbances resulting in falls were seen in all patients and none of them responded to dopaminergic medications. Two patients underwent dopamine transporter (DaT) SPECT scanning with normal results. Other features included emotional lability (n = 5) and cognitive impairment involving frontal subcortical systems (n = 1). In conclusion, these cases represent a subgroup of PLS patients in whom pyramidal slowness may be mistaken for akinesia, and spasticity misconstrued as rigidity, leading to an erroneous diagnosis of atypical parkinsonism. However, the absence of fatiguing and decrement on repeated finger/foot tapping should help to distinguish these patients from the true atypical parkinsonian syndromes. © 2007 Movement Disorder Society</p></abstract></abstractGroup></contentMeta></header></component></istex:document></istex:metadataXml><!--Version 0.6 générée le 4-12-2015--><mods version="3.6"><titleInfo lang="en"><title>Primary lateral sclerosis mimicking atypical parkinsonism</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>PLS Mimicking Atypical Parkinsonism</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Primary lateral sclerosis mimicking atypical parkinsonism</title></titleInfo><name type="personal"><namePart type="given">Ibrahim M.</namePart><namePart type="family">Norlinah</namePart><namePart type="termsOfAddress">MB</namePart><affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</affiliation><affiliation>Neurology Unit, Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Kailash P.</namePart><namePart type="family">Bhatia</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Karen</namePart><namePart type="family">Østergaard</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Neurology, Aarhus University Hospital, Aarhus C, Denmark</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Robin</namePart><namePart type="family">Howard</namePart><namePart type="termsOfAddress">PhD</namePart><affiliation>Department of Molecular Neuroscience, Institute of Neurology, London, United Kingdom</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Gennarina</namePart><namePart type="family">Arabia</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Niall P.</namePart><namePart type="family">Quinn</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, United Kingdom</affiliation><description>Correspondence: Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, Queen Square, London WC1N 3BG, United Kingdom</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">2007-10-31</dateIssued><dateCaptured encoding="w3cdtf">2006-12-15</dateCaptured><dateValid encoding="w3cdtf">2007-05-29</dateValid><copyrightDate encoding="w3cdtf">2007</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="tables">3</extent><extent unit="references">17</extent><extent unit="words">3809</extent></physicalDescription><abstract lang="en">Primary lateral sclerosis (PLS), the upper motor neurone variant of motor neurone disease, is characterized by progressive spinal or bulbar spasticity with minimal motor weakness. Rarely, PLS may present with clinical features resembling parkinsonism resulting in occasional misdiagnosis as one of the atypical parkinsonian syndromes. Here we describe five patients initially referred with a diagnosis of levodopa‐unresponsive atypical parkinsonism (n = 4) or primary progressive multiple sclerosis (n = 1), but subsequently found to have features consistent with PLS instead. Onset age varied from 49 to 67 years. Unilateral limb slowness or clumsiness was the initial complaint in four, and bulbar symptoms in one. Repeated finger/foot tapping was slow in all five, but without fatiguing or decrement. Spasticity with hyperreflexia, exaggerated jaw jerk and extensor plantar responses were eventually seen in all patients. Anterior horn cell involvement developed in three cases. Early gait disturbances resulting in falls were seen in all patients and none of them responded to dopaminergic medications. Two patients underwent dopamine transporter (DaT) SPECT scanning with normal results. Other features included emotional lability (n = 5) and cognitive impairment involving frontal subcortical systems (n = 1). In conclusion, these cases represent a subgroup of PLS patients in whom pyramidal slowness may be mistaken for akinesia, and spasticity misconstrued as rigidity, leading to an erroneous diagnosis of atypical parkinsonism. However, the absence of fatiguing and decrement on repeated finger/foot tapping should help to distinguish these patients from the true atypical parkinsonian syndromes. © 2007 Movement Disorder Society</abstract><subject lang="en"><genre>Keywords</genre><topic>primary lateral sclerosis</topic><topic>atypical parkinsonism</topic><topic>akinesia</topic><topic>extrapyramidal</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><note type="content"> This article includes supplementary video clips, available online at http://www.interscience.wiley.com/jpages/0885‐3185/suppmat .Supporting Info Item: Case 1There is slowness of finger taps but with no definite fatiguing or decrement. There is obvious wasting of the left first dorsal interosseus.Case 2Segment 1. Repeated finger taps are slow, but with no definite fatiguing or decrement.Segment 2. The patient walks with a narrow‐based, stiff gait. There is stooped posture with reduced arm swing bilaterally. There is postural instability on the pull test. - </note><subject><genre>article category</genre><topic>Research Article</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>2007</date><detail type="volume"><caption>vol.</caption><number>22</number></detail><detail type="issue"><caption>no.</caption><number>14</number></detail><extent unit="pages"><start>2057</start><end>2062</end><total>6</total></extent></part></relatedItem><identifier type="istex">6EE2C459D88B3BC7028060986EC265EFAA819B10</identifier><identifier type="DOI">10.1002/mds.21645</identifier><identifier type="ArticleID">MDS21645</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 2007 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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