Pallido-Luysio-Nigral atrophy revealed by rapidly progressive hemidystonia: a clinical, radiologic, functional, and neuropathologic study.
Identifieur interne : 003F02 ( PubMed/Curation ); précédent : 003F01; suivant : 003F03Pallido-Luysio-Nigral atrophy revealed by rapidly progressive hemidystonia: a clinical, radiologic, functional, and neuropathologic study.
Auteurs : L. Vercueil [France] ; A. Hammouti ; M L Andriantseheno ; M. Mohr ; C. Tranchant ; P. Maquet ; C. Marescaux ; F. SellalSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 2000.
English descriptors
- KwdEn :
- Age of Onset, Atrophy, Brain (metabolism), Brain (pathology), Brain (radionuclide imaging), Cerebrovascular Circulation, Diagnosis, Differential, Disease Progression, Dystonic Disorders (diagnosis), Dystonic Disorders (etiology), Dystonic Disorders (pathology), Dystonic Disorders (physiopathology), Fatal Outcome, Functional Laterality, Globus Pallidus (pathology), Globus Pallidus (radionuclide imaging), Humans, Magnetic Resonance Imaging, Male, Middle Aged, Neurodegenerative Diseases (complications), Neurodegenerative Diseases (diagnosis), Neurodegenerative Diseases (physiopathology), Substantia Nigra (pathology), Substantia Nigra (radionuclide imaging), Thalamus (pathology), Tomography, Emission-Computed, Tomography, Emission-Computed, Single-Photon, Videotape Recording.
- MESH :
- complications : Neurodegenerative Diseases.
- diagnosis : Dystonic Disorders, Neurodegenerative Diseases.
- etiology : Dystonic Disorders.
- metabolism : Brain.
- pathology : Brain, Dystonic Disorders, Globus Pallidus, Substantia Nigra, Thalamus.
- physiopathology : Dystonic Disorders, Neurodegenerative Diseases.
- radionuclide imaging : Brain, Globus Pallidus, Substantia Nigra.
- Age of Onset, Atrophy, Cerebrovascular Circulation, Diagnosis, Differential, Disease Progression, Fatal Outcome, Functional Laterality, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Tomography, Emission-Computed, Tomography, Emission-Computed, Single-Photon, Videotape Recording.
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.
PubMed: 11009203
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Hammouti</name></author><author><name sortKey="Andriantseheno, M L" sort="Andriantseheno, M L" uniqKey="Andriantseheno M" first="M L" last="Andriantseheno">M L Andriantseheno</name></author><author><name sortKey="Mohr, M" sort="Mohr, M" uniqKey="Mohr M" first="M" last="Mohr">M. Mohr</name></author><author><name sortKey="Tranchant, C" sort="Tranchant, C" uniqKey="Tranchant C" first="C" last="Tranchant">C. Tranchant</name></author><author><name sortKey="Maquet, P" sort="Maquet, P" uniqKey="Maquet P" first="P" last="Maquet">P. Maquet</name></author><author><name sortKey="Marescaux, C" sort="Marescaux, C" uniqKey="Marescaux C" first="C" last="Marescaux">C. Marescaux</name></author><author><name sortKey="Sellal, F" sort="Sellal, F" uniqKey="Sellal F" first="F" last="Sellal">F. Sellal</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2000">2000</date><idno type="RBID">pubmed:11009203</idno><idno type="pmid">11009203</idno><idno type="wicri:Area/PubMed/Corpus">003F02</idno><idno type="wicri:Area/PubMed/Curation">003F02</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Pallido-Luysio-Nigral atrophy revealed by rapidly progressive hemidystonia: a clinical, radiologic, functional, and neuropathologic study.</title><author><name sortKey="Vercueil, L" sort="Vercueil, L" uniqKey="Vercueil L" first="L" last="Vercueil">L. Vercueil</name><affiliation wicri:level="1"><nlm:affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies, Hôpitaux Universitaires de Strasbourg, France.</nlm:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies, Hôpitaux Universitaires de Strasbourg</wicri:regionArea></affiliation></author><author><name sortKey="Hammouti, A" sort="Hammouti, A" uniqKey="Hammouti A" first="A" last="Hammouti">A. Hammouti</name></author><author><name sortKey="Andriantseheno, M L" sort="Andriantseheno, M L" uniqKey="Andriantseheno M" first="M L" last="Andriantseheno">M L Andriantseheno</name></author><author><name sortKey="Mohr, M" sort="Mohr, M" uniqKey="Mohr M" first="M" last="Mohr">M. Mohr</name></author><author><name sortKey="Tranchant, C" sort="Tranchant, C" uniqKey="Tranchant C" first="C" last="Tranchant">C. Tranchant</name></author><author><name sortKey="Maquet, P" sort="Maquet, P" uniqKey="Maquet P" first="P" last="Maquet">P. Maquet</name></author><author><name sortKey="Marescaux, C" sort="Marescaux, C" uniqKey="Marescaux C" first="C" last="Marescaux">C. Marescaux</name></author><author><name sortKey="Sellal, F" sort="Sellal, F" uniqKey="Sellal F" first="F" last="Sellal">F. Sellal</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="ISSN">0885-3185</idno><imprint><date when="2000" type="published">2000</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Age of Onset</term><term>Atrophy</term><term>Brain (metabolism)</term><term>Brain (pathology)</term><term>Brain (radionuclide imaging)</term><term>Cerebrovascular Circulation</term><term>Diagnosis, Differential</term><term>Disease Progression</term><term>Dystonic Disorders (diagnosis)</term><term>Dystonic Disorders (etiology)</term><term>Dystonic Disorders (pathology)</term><term>Dystonic Disorders (physiopathology)</term><term>Fatal Outcome</term><term>Functional Laterality</term><term>Globus Pallidus (pathology)</term><term>Globus Pallidus (radionuclide imaging)</term><term>Humans</term><term>Magnetic Resonance Imaging</term><term>Male</term><term>Middle Aged</term><term>Neurodegenerative Diseases (complications)</term><term>Neurodegenerative Diseases (diagnosis)</term><term>Neurodegenerative Diseases (physiopathology)</term><term>Substantia Nigra (pathology)</term><term>Substantia Nigra (radionuclide imaging)</term><term>Thalamus (pathology)</term><term>Tomography, Emission-Computed</term><term>Tomography, Emission-Computed, Single-Photon</term><term>Videotape Recording</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Neurodegenerative Diseases</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Dystonic Disorders</term><term>Neurodegenerative Diseases</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Dystonic Disorders</term></keywords><keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Brain</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain</term><term>Dystonic Disorders</term><term>Globus Pallidus</term><term>Substantia Nigra</term><term>Thalamus</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Dystonic Disorders</term><term>Neurodegenerative Diseases</term></keywords><keywords scheme="MESH" qualifier="radionuclide imaging" xml:lang="en"><term>Brain</term><term>Globus Pallidus</term><term>Substantia Nigra</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Age of Onset</term><term>Atrophy</term><term>Cerebrovascular Circulation</term><term>Diagnosis, Differential</term><term>Disease Progression</term><term>Fatal Outcome</term><term>Functional Laterality</term><term>Humans</term><term>Magnetic Resonance Imaging</term><term>Male</term><term>Middle Aged</term><term>Tomography, Emission-Computed</term><term>Tomography, Emission-Computed, Single-Photon</term><term>Videotape Recording</term></keywords></textClass></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><pubmed><MedlineCitation Owner="NLM" Status="MEDLINE"><PMID Version="1">11009203</PMID><DateCreated><Year>2001</Year><Month>01</Month><Day>09</Day></DateCreated><DateCompleted><Year>2001</Year><Month>01</Month><Day>09</Day></DateCompleted><DateRevised><Year>2006</Year><Month>11</Month><Day>15</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0885-3185</ISSN><JournalIssue CitedMedium="Print"><Volume>15</Volume><Issue>5</Issue><PubDate><Year>2000</Year><Month>Sep</Month></PubDate></JournalIssue><Title>Movement disorders : official journal of the Movement Disorder Society</Title><ISOAbbreviation>Mov. Disord.</ISOAbbreviation></Journal><ArticleTitle>Pallido-Luysio-Nigral atrophy revealed by rapidly progressive hemidystonia: a clinical, radiologic, functional, and neuropathologic study.</ArticleTitle><Pagination><MedlinePgn>947-53</MedlinePgn></Pagination><Abstract><AbstractText>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.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Vercueil</LastName><ForeName>L</ForeName><Initials>L</Initials><AffiliationInfo><Affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies, Hôpitaux Universitaires de Strasbourg, France.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Hammouti</LastName><ForeName>A</ForeName><Initials>A</Initials></Author><Author ValidYN="Y"><LastName>Andriantseheno</LastName><ForeName>M L</ForeName><Initials>ML</Initials></Author><Author ValidYN="Y"><LastName>Mohr</LastName><ForeName>M</ForeName><Initials>M</Initials></Author><Author ValidYN="Y"><LastName>Tranchant</LastName><ForeName>C</ForeName><Initials>C</Initials></Author><Author ValidYN="Y"><LastName>Maquet</LastName><ForeName>P</ForeName><Initials>P</Initials></Author><Author ValidYN="Y"><LastName>Marescaux</LastName><ForeName>C</ForeName><Initials>C</Initials></Author><Author ValidYN="Y"><LastName>Sellal</LastName><ForeName>F</ForeName><Initials>F</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D002363">Case Reports</PublicationType><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>UNITED STATES</Country><MedlineTA>Mov Disord</MedlineTA><NlmUniqueID>8610688</NlmUniqueID><ISSNLinking>0885-3185</ISSNLinking></MedlineJournalInfo><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName MajorTopicYN="N" UI="D017668">Age of Onset</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D001284">Atrophy</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D001921">Brain</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000378">metabolism</QualifierName><QualifierName MajorTopicYN="N" UI="Q000473">pathology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000531">radionuclide imaging</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="Y" UI="D002560">Cerebrovascular Circulation</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D003937">Diagnosis, Differential</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D018450">Disease Progression</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D020821">Dystonic Disorders</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="Y" UI="Q000209">etiology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000473">pathology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D017809">Fatal Outcome</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D007839">Functional Laterality</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D005917">Globus Pallidus</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000473">pathology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000531">radionuclide imaging</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D006801">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008279">Magnetic Resonance Imaging</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008297">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008875">Middle Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D019636">Neurodegenerative Diseases</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000150">complications</QualifierName><QualifierName MajorTopicYN="Y" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D013378">Substantia Nigra</DescriptorName><QualifierName MajorTopicYN="Y" UI="Q000473">pathology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000531">radionuclide imaging</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D013788">Thalamus</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000473">pathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D014055">Tomography, Emission-Computed</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D015899">Tomography, Emission-Computed, Single-Photon</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D014743">Videotape Recording</DescriptorName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>2000</Year><Month>9</Month><Day>29</Day><Hour>11</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2001</Year><Month>2</Month><Day>28</Day><Hour>10</Hour><Minute>1</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2000</Year><Month>9</Month><Day>29</Day><Hour>11</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">11009203</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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