Pallido‐luysio‐nigral atrophy revealed by rapidly progressive hemidystonia: A clinical, radiologic, functional, and neuropathologic study
Identifieur interne : 001734 ( Istex/Corpus ); précédent : 001733; suivant : 001735Pallido‐luysio‐nigral atrophy revealed by rapidly progressive hemidystonia: A clinical, radiologic, functional, and neuropathologic study
Auteurs : Laurent Vercueil ; Abdallah Hammouti ; Marcellin L. Andriantseheno ; Michel Mohr ; Christine Tranchant ; Pierre Maquet ; Christian Marescaux ; François SellalSource :
- Movement Disorders [ 0885-3185 ] ; 2000-09.
English descriptors
- KwdEn :
Abstract
Pallido‐luysio‐nigral atrophy (PLNA) is a rare neurodegenerative disease in which the clinical and radiologic correlates have not yet been clearly established. A 62‐year‐old man insidiously developed dystonic postures, choreoathetoid movements, slowness, and stiffness, which initially affected the right hand and foot and progressively spread to the entire right side. T2‐weighted magnetic resonance imaging showed increased signal intensity in both left and right medial pallida and in the left substantia nigra. Tests using HMPAO‐SPECT and FDG‐PET demonstrated left cortical hyperperfusion and hypermetabolism, whereas the left lenticular nucleus was slightly hypometabolic. At age 65, abnormal movements and postures involved all four limbs and the axis causing major gait disturbances, and facial and bulbar muscles atrophied resulting in dysarthria, dysphagia, and impaired breathing. Diffuse amyotrophy and fasciculations also appeared. Death occurred at age 66, 4 years after onset. At autopsy, severe bilateral neuronal loss and gliosis restricted to the pallidum, the subthalamic nucleus, the substantia nigra, and the hypoglossal nucleus were noted, accounting for the diagnosis of PLNA with lower motor neuron involvement. Progressive hemidystonia with adult onset represents an unusual clinical presentation for this disorder. Moreover, this observation indicates that a diagnosis of PLNA should be considered for specific magnetic resonance imaging, SPECT, and/or PET data, and suggests that in PLNA, pallidal dysfunction might play a key role in the dystonic presentation.
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DOI: 10.1002/1531-8257(200009)15:5<947::AID-MDS1027>3.0.CO;2-L
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Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>John Wiley & Sons, Inc.</publisher><pubPlace>New York</pubPlace><date type="published" when="2000-09">2000-09</date><biblScope unit="vol">15</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="947">947</biblScope><biblScope unit="page" to="953">953</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">3CA132D641A5396B7E9868AAB0469669C6655C1C</idno><idno type="DOI">10.1002/1531-8257(200009)15:5<947::AID-MDS1027>3.0.CO;2-L</idno><idno type="ArticleID">MDS1027</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Brain metabolism</term><term>Hemidystonia</term><term>Pallidal degeneration</term><term>Pallido‐luysio‐nigral atrophy</term><term>Thalamocortical perfusion</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Pallido‐luysio‐nigral atrophy (PLNA) is a rare neurodegenerative disease in which the clinical and radiologic correlates have not yet been clearly established. A 62‐year‐old man insidiously developed dystonic postures, choreoathetoid movements, slowness, and stiffness, which initially affected the right hand and foot and progressively spread to the entire right side. T2‐weighted magnetic resonance imaging showed increased signal intensity in both left and right medial pallida and in the left substantia nigra. Tests using HMPAO‐SPECT and FDG‐PET demonstrated left cortical hyperperfusion and hypermetabolism, whereas the left lenticular nucleus was slightly hypometabolic. At age 65, abnormal movements and postures involved all four limbs and the axis causing major gait disturbances, and facial and bulbar muscles atrophied resulting in dysarthria, dysphagia, and impaired breathing. Diffuse amyotrophy and fasciculations also appeared. Death occurred at age 66, 4 years after onset. At autopsy, severe bilateral neuronal loss and gliosis restricted to the pallidum, the subthalamic nucleus, the substantia nigra, and the hypoglossal nucleus were noted, accounting for the diagnosis of PLNA with lower motor neuron involvement. Progressive hemidystonia with adult onset represents an unusual clinical presentation for this disorder. Moreover, this observation indicates that a diagnosis of PLNA should be considered for specific magnetic resonance imaging, SPECT, and/or PET data, and suggests that in PLNA, pallidal dysfunction might play a key role in the dystonic presentation.</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Laurent Vercueil MD</name><affiliations><json:string>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</json:string></affiliations></json:item><json:item><name>Abdallah Hammouti MD</name><affiliations><json:string>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</json:string></affiliations></json:item><json:item><name>Marcellin L. Andriantseheno MD</name><affiliations><json:string>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</json:string></affiliations></json:item><json:item><name>Michel Mohr MD</name><affiliations><json:string>Institut d'Anatomie Pathologique</json:string></affiliations></json:item><json:item><name>Christine Tranchant MD</name><affiliations><json:string>Service des Maladies du Système Nerveux et du Muscle, Hôpitaux Universitaires de Strasbourg, France</json:string></affiliations></json:item><json:item><name>Pierre Maquet MD</name><affiliations><json:string>Unité Médicale du Cyclotron, CHU Sart Tilman, Liège, Belgique</json:string></affiliations></json:item><json:item><name>Christian Marescaux MD</name><affiliations><json:string>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</json:string></affiliations></json:item><json:item><name>François Sellal MD</name><affiliations><json:string>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>Pallido‐luysio‐nigral atrophy</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Pallidal degeneration</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Hemidystonia</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Thalamocortical perfusion</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Brain metabolism</value></json:item></subject><language><json:string>eng</json:string></language><abstract>Pallido‐luysio‐nigral atrophy (PLNA) is a rare neurodegenerative disease in which the clinical and radiologic correlates have not yet been clearly established. A 62‐year‐old man insidiously developed dystonic postures, choreoathetoid movements, slowness, and stiffness, which initially affected the right hand and foot and progressively spread to the entire right side. T2‐weighted magnetic resonance imaging showed increased signal intensity in both left and right medial pallida and in the left substantia nigra. Tests using HMPAO‐SPECT and FDG‐PET demonstrated left cortical hyperperfusion and hypermetabolism, whereas the left lenticular nucleus was slightly hypometabolic. At age 65, abnormal movements and postures involved all four limbs and the axis causing major gait disturbances, and facial and bulbar muscles atrophied resulting in dysarthria, dysphagia, and impaired breathing. Diffuse amyotrophy and fasciculations also appeared. Death occurred at age 66, 4 years after onset. At autopsy, severe bilateral neuronal loss and gliosis restricted to the pallidum, the subthalamic nucleus, the substantia nigra, and the hypoglossal nucleus were noted, accounting for the diagnosis of PLNA with lower motor neuron involvement. Progressive hemidystonia with adult onset represents an unusual clinical presentation for this disorder. Moreover, this observation indicates that a diagnosis of PLNA should be considered for specific magnetic resonance imaging, SPECT, and/or PET data, and suggests that in PLNA, pallidal dysfunction might play a key role in the dystonic presentation.</abstract><qualityIndicators><score>6.09</score><pdfVersion>1.3</pdfVersion><pdfPageSize>612 x 792 pts (letter)</pdfPageSize><refBibsNative>true</refBibsNative><abstractCharCount>1587</abstractCharCount><pdfWordCount>3450</pdfWordCount><pdfCharCount>23795</pdfCharCount><pdfPageCount>7</pdfPageCount><abstractWordCount>220</abstractWordCount></qualityIndicators><title>Pallido‐luysio‐nigral atrophy revealed by rapidly progressive hemidystonia: A clinical, radiologic, functional, and neuropathologic study</title><genre><json:string>Serial article</json:string></genre><host><volume>15</volume><pages><total>7</total><last>953</last><first>947</first></pages><issn><json:string>0885-3185</json:string></issn><issue>5</issue><subject><json:item><value>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>2000</publicationDate><copyrightDate>2000</copyrightDate><doi><json:string>10.1002/1531-8257(200009)15:5>947::AID-MDS1027>3.0.CO;2-L</json:string></doi><id>3CA132D641A5396B7E9868AAB0469669C6655C1C</id><fulltext><json:item><original>true</original><mimetype>application/pdf</mimetype><extension>pdf</extension><uri>https://api.istex.fr/document/3CA132D641A5396B7E9868AAB0469669C6655C1C/fulltext/pdf</uri></json:item><json:item><original>false</original><mimetype>application/zip</mimetype><extension>zip</extension><uri>https://api.istex.fr/document/3CA132D641A5396B7E9868AAB0469669C6655C1C/fulltext/zip</uri></json:item><istex:fulltextTEI uri="https://api.istex.fr/document/3CA132D641A5396B7E9868AAB0469669C6655C1C/fulltext/tei"><teiHeader type="text"><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Pallido‐luysio‐nigral atrophy revealed by rapidly progressive hemidystonia: A clinical, radiologic, functional, and neuropathologic study</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher>John Wiley & Sons, Inc.</publisher><pubPlace>New York</pubPlace><availability><p>John Wiley & Sons, Inc.</p></availability><date>2000</date></publicationStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Pallido‐luysio‐nigral atrophy revealed by rapidly progressive hemidystonia: A clinical, radiologic, functional, and neuropathologic study</title><author><persName><forename type="first">Laurent</forename><surname>Vercueil</surname><roleName type="degree">MD</roleName></persName><note type="correspondence"><p>Correspondence: Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies, Hôpitaux Universitaires, 1 place de l'Hôpital, 67091 Strasbourg Cedex, France</p></note><affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</affiliation></author><author><persName><forename type="first">Abdallah</forename><surname>Hammouti</surname><roleName type="degree">MD</roleName></persName><affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</affiliation></author><author><persName><forename type="first">Marcellin L.</forename><surname>Andriantseheno</surname><roleName type="degree">MD</roleName></persName><affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</affiliation></author><author><persName><forename type="first">Michel</forename><surname>Mohr</surname><roleName type="degree">MD</roleName></persName><affiliation>Institut d'Anatomie Pathologique</affiliation></author><author><persName><forename type="first">Christine</forename><surname>Tranchant</surname><roleName type="degree">MD</roleName></persName><affiliation>Service des Maladies du Système Nerveux et du Muscle, Hôpitaux Universitaires de Strasbourg, France</affiliation></author><author><persName><forename type="first">Pierre</forename><surname>Maquet</surname><roleName type="degree">MD</roleName></persName><affiliation>Unité Médicale du Cyclotron, CHU Sart Tilman, Liège, Belgique</affiliation></author><author><persName><forename type="first">Christian</forename><surname>Marescaux</surname><roleName type="degree">MD</roleName></persName><affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</affiliation></author><author><persName><forename type="first">François</forename><surname>Sellal</surname><roleName type="degree">MD</roleName></persName><affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</affiliation></author></analytic><monogr><title level="j">Movement Disorders</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>John Wiley & Sons, Inc.</publisher><pubPlace>New York</pubPlace><date type="published" when="2000-09"></date><biblScope unit="vol">15</biblScope><biblScope unit="issue">5</biblScope><biblScope unit="page" from="947">947</biblScope><biblScope unit="page" to="953">953</biblScope></imprint></monogr><idno type="istex">3CA132D641A5396B7E9868AAB0469669C6655C1C</idno><idno type="DOI">10.1002/1531-8257(200009)15:5<947::AID-MDS1027>3.0.CO;2-L</idno><idno type="ArticleID">MDS1027</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>2000</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>Pallido‐luysio‐nigral atrophy (PLNA) is a rare neurodegenerative disease in which the clinical and radiologic correlates have not yet been clearly established. A 62‐year‐old man insidiously developed dystonic postures, choreoathetoid movements, slowness, and stiffness, which initially affected the right hand and foot and progressively spread to the entire right side. T2‐weighted magnetic resonance imaging showed increased signal intensity in both left and right medial pallida and in the left substantia nigra. Tests using HMPAO‐SPECT and FDG‐PET demonstrated left cortical hyperperfusion and hypermetabolism, whereas the left lenticular nucleus was slightly hypometabolic. At age 65, abnormal movements and postures involved all four limbs and the axis causing major gait disturbances, and facial and bulbar muscles atrophied resulting in dysarthria, dysphagia, and impaired breathing. Diffuse amyotrophy and fasciculations also appeared. Death occurred at age 66, 4 years after onset. At autopsy, severe bilateral neuronal loss and gliosis restricted to the pallidum, the subthalamic nucleus, the substantia nigra, and the hypoglossal nucleus were noted, accounting for the diagnosis of PLNA with lower motor neuron involvement. Progressive hemidystonia with adult onset represents an unusual clinical presentation for this disorder. Moreover, this observation indicates that a diagnosis of PLNA should be considered for specific magnetic resonance imaging, SPECT, and/or PET data, and suggests that in PLNA, pallidal dysfunction might play a key role in the dystonic presentation.</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>Keywords</head><item><term>Pallido‐luysio‐nigral atrophy</term></item><item><term>Pallidal degeneration</term></item><item><term>Hemidystonia</term></item><item><term>Thalamocortical perfusion</term></item><item><term>Brain metabolism</term></item></list></keywords></textClass><textClass><keywords scheme="Journal Subject"><list><head>Article category</head><item><term>Article</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="1999-07-12">Received</change><change when="2000-04-18">Registration</change><change when="2000-09">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/3CA132D641A5396B7E9868AAB0469669C6655C1C/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>John Wiley & Sons, Inc.</publisherName><publisherLoc>New York</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="short">Mov. 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href="file:MDS.MDS1027.pdf"></link></linkGroup></publicationMeta><contentMeta><countGroup><count type="figureTotal" number="5"></count><count type="tableTotal" number="0"></count><count type="referenceTotal" number="30"></count><count type="wordTotal" number="3360"></count></countGroup><titleGroup><title type="main" xml:lang="en">Pallido‐luysio‐nigral atrophy revealed by rapidly progressive hemidystonia: A clinical, radiologic, functional, and neuropathologic study</title><title type="short" xml:lang="en">Pallido‐Luysio‐Nigral Atrophy and Hemidystonia</title></titleGroup><creators><creator xml:id="au1" creatorRole="author" affiliationRef="#af1" corresponding="yes"><personName><givenNames>Laurent</givenNames><familyName>Vercueil</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au2" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Abdallah</givenNames><familyName>Hammouti</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au3" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Marcellin L.</givenNames><familyName>Andriantseheno</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au4" creatorRole="author" affiliationRef="#af2"><personName><givenNames>Michel</givenNames><familyName>Mohr</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au5" creatorRole="author" affiliationRef="#af3"><personName><givenNames>Christine</givenNames><familyName>Tranchant</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au6" creatorRole="author" affiliationRef="#af4"><personName><givenNames>Pierre</givenNames><familyName>Maquet</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au7" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Christian</givenNames><familyName>Marescaux</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au8" creatorRole="author" affiliationRef="#af1"><personName><givenNames>François</givenNames><familyName>Sellal</familyName><degrees>MD</degrees></personName></creator></creators><affiliationGroup><affiliation xml:id="af1" countryCode="FR" type="organization"><unparsedAffiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</unparsedAffiliation></affiliation><affiliation xml:id="af2" countryCode="FR" type="organization"><unparsedAffiliation>Institut d'Anatomie Pathologique</unparsedAffiliation></affiliation><affiliation xml:id="af3" countryCode="FR" type="organization"><unparsedAffiliation>Service des Maladies du Système Nerveux et du Muscle, Hôpitaux Universitaires de Strasbourg, France</unparsedAffiliation></affiliation><affiliation xml:id="af4" countryCode="FR" type="organization"><unparsedAffiliation>Unité Médicale du Cyclotron, CHU Sart Tilman, Liège, Belgique</unparsedAffiliation></affiliation></affiliationGroup><keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">Pallido‐luysio‐nigral atrophy</keyword><keyword xml:id="kwd2">Pallidal degeneration</keyword><keyword xml:id="kwd3">Hemidystonia</keyword><keyword xml:id="kwd4">Thalamocortical perfusion</keyword><keyword xml:id="kwd5">Brain metabolism</keyword></keywordGroup><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><p>Pallido‐luysio‐nigral atrophy (PLNA) is a rare neurodegenerative disease in which the clinical and radiologic correlates have not yet been clearly established. A 62‐year‐old man insidiously developed dystonic postures, choreoathetoid movements, slowness, and stiffness, which initially affected the right hand and foot and progressively spread to the entire right side. T2‐weighted magnetic resonance imaging showed increased signal intensity in both left and right medial pallida and in the left substantia nigra. Tests using HMPAO‐SPECT and FDG‐PET demonstrated left cortical hyperperfusion and hypermetabolism, whereas the left lenticular nucleus was slightly hypometabolic. At age 65, abnormal movements and postures involved all four limbs and the axis causing major gait disturbances, and facial and bulbar muscles atrophied resulting in dysarthria, dysphagia, and impaired breathing. Diffuse amyotrophy and fasciculations also appeared. Death occurred at age 66, 4 years after onset. At autopsy, severe bilateral neuronal loss and gliosis restricted to the pallidum, the subthalamic nucleus, the substantia nigra, and the hypoglossal nucleus were noted, accounting for the diagnosis of PLNA with lower motor neuron involvement. Progressive hemidystonia with adult onset represents an unusual clinical presentation for this disorder. Moreover, this observation indicates that a diagnosis of PLNA should be considered for specific magnetic resonance imaging, SPECT, and/or PET data, and suggests that in PLNA, pallidal dysfunction might play a key role in the dystonic presentation.</p></abstract></abstractGroup></contentMeta></header></component></istex:document></istex:metadataXml><!--Version 0.6 générée le 3-12-2015--><mods version="3.6"><titleInfo lang="en"><title>Pallido‐luysio‐nigral atrophy revealed by rapidly progressive hemidystonia: A clinical, radiologic, functional, and neuropathologic study</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>Pallido‐Luysio‐Nigral Atrophy and Hemidystonia</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Pallido‐luysio‐nigral atrophy revealed by rapidly progressive hemidystonia: A clinical, radiologic, functional, and neuropathologic study</title></titleInfo><name type="personal"><namePart type="given">Laurent</namePart><namePart type="family">Vercueil</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</affiliation><description>Correspondence: Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies, Hôpitaux Universitaires, 1 place de l'Hôpital, 67091 Strasbourg Cedex, France</description><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Abdallah</namePart><namePart type="family">Hammouti</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Marcellin L.</namePart><namePart type="family">Andriantseheno</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Michel</namePart><namePart type="family">Mohr</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Institut d'Anatomie Pathologique</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Christine</namePart><namePart type="family">Tranchant</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service des Maladies du Système Nerveux et du Muscle, Hôpitaux Universitaires de Strasbourg, France</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Pierre</namePart><namePart type="family">Maquet</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Unité Médicale du Cyclotron, CHU Sart Tilman, Liège, Belgique</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Christian</namePart><namePart type="family">Marescaux</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">François</namePart><namePart type="family">Sellal</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre authority="originalCategForm">article</genre><originInfo><publisher>John Wiley & Sons, Inc.</publisher><place><placeTerm type="text">New York</placeTerm></place><dateIssued encoding="w3cdtf">2000-09</dateIssued><dateCaptured encoding="w3cdtf">1999-07-12</dateCaptured><dateValid encoding="w3cdtf">2000-04-18</dateValid><copyrightDate encoding="w3cdtf">2000</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">5</extent><extent unit="references">30</extent><extent unit="words">3360</extent></physicalDescription><abstract lang="en">Pallido‐luysio‐nigral atrophy (PLNA) is a rare neurodegenerative disease in which the clinical and radiologic correlates have not yet been clearly established. A 62‐year‐old man insidiously developed dystonic postures, choreoathetoid movements, slowness, and stiffness, which initially affected the right hand and foot and progressively spread to the entire right side. T2‐weighted magnetic resonance imaging showed increased signal intensity in both left and right medial pallida and in the left substantia nigra. Tests using HMPAO‐SPECT and FDG‐PET demonstrated left cortical hyperperfusion and hypermetabolism, whereas the left lenticular nucleus was slightly hypometabolic. At age 65, abnormal movements and postures involved all four limbs and the axis causing major gait disturbances, and facial and bulbar muscles atrophied resulting in dysarthria, dysphagia, and impaired breathing. Diffuse amyotrophy and fasciculations also appeared. Death occurred at age 66, 4 years after onset. At autopsy, severe bilateral neuronal loss and gliosis restricted to the pallidum, the subthalamic nucleus, the substantia nigra, and the hypoglossal nucleus were noted, accounting for the diagnosis of PLNA with lower motor neuron involvement. Progressive hemidystonia with adult onset represents an unusual clinical presentation for this disorder. Moreover, this observation indicates that a diagnosis of PLNA should be considered for specific magnetic resonance imaging, SPECT, and/or PET data, and suggests that in PLNA, pallidal dysfunction might play a key role in the dystonic presentation.</abstract><subject lang="en"><genre>Keywords</genre><topic>Pallido‐luysio‐nigral atrophy</topic><topic>Pallidal degeneration</topic><topic>Hemidystonia</topic><topic>Thalamocortical perfusion</topic><topic>Brain metabolism</topic></subject><relatedItem type="host"><titleInfo><title>Movement Disorders</title></titleInfo><titleInfo type="abbreviated"><title>Mov. Disord.</title></titleInfo><subject><genre>article category</genre><topic>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>2000</date><detail type="volume"><caption>vol.</caption><number>15</number></detail><detail type="issue"><caption>no.</caption><number>5</number></detail><extent unit="pages"><start>947</start><end>953</end><total>7</total></extent></part></relatedItem><identifier type="istex">3CA132D641A5396B7E9868AAB0469669C6655C1C</identifier><identifier type="DOI">10.1002/1531-8257(200009)15:5<947::AID-MDS1027>3.0.CO;2-L</identifier><identifier type="ArticleID">MDS1027</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 2000 Movement Disorder Society</accessCondition><recordInfo><recordOrigin>John Wiley & Sons, Inc.</recordOrigin><recordContentSource>WILEY</recordContentSource></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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