Pallido‐luysio‐nigral atrophy revealed by rapidly progressive hemidystonia: A clinical, radiologic, functional, and neuropathologic study
Identifieur interne : 000523 ( France/Analysis ); précédent : 000522; suivant : 000524Pallido‐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 [France] ; Pierre Maquet [Belgique] ; Christian Marescaux ; François SellalSource :
- Movement Disorders [ 0885-3185 ] ; 2000-09.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Personne âgée.
English descriptors
- KwdEn :
- Age of Onset, Atrophy, Brain (metabolism), Brain (pathology), Brain (radionuclide imaging), Brain metabolism, Case study, Cerebrovascular Circulation, Diagnosis, Diagnosis, Differential, Disease Progression, Dystonia, Dystonic Disorders (diagnosis), Dystonic Disorders (etiology), Dystonic Disorders (pathology), Dystonic Disorders (physiopathology), Elderly, Emission tomography, Evolution, Fatal Outcome, Functional Laterality, Globus Pallidus (pathology), Globus Pallidus (radionuclide imaging), Hemidystonia, Humans, Inaugural sign, Locus niger, Magnetic Resonance Imaging, Male, Middle Aged, Neurodegenerative Diseases (complications), Neurodegenerative Diseases (diagnosis), Neurodegenerative Diseases (physiopathology), Nuclear magnetic resonance imaging, Pallidal degeneration, Pallido‐luysio‐nigral atrophy, Pallidum, Pathology, Photon, Positron, Substantia Nigra (pathology), Substantia Nigra (radionuclide imaging), Subthalamic nucleus, Thalamocortical perfusion, Thalamus (pathology), Tomography, Emission-Computed, Tomography, Emission-Computed, Single-Photon, Unilateral, 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.
Url:
DOI: 10.1002/1531-8257(200009)15:5<947::AID-MDS1027>3.0.CO;2-L
Affiliations:
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Andriantseheno</name><affiliation><wicri:noCountry code="subField">Neuropsychologie et Explorations Fonctionnelles des Epilepsies</wicri:noCountry></affiliation></author><author><name sortKey="Mohr, Michel" sort="Mohr, Michel" uniqKey="Mohr M" first="Michel" last="Mohr">Michel Mohr</name><affiliation><wicri:noCountry code="no comma">Institut d'Anatomie Pathologique</wicri:noCountry></affiliation></author><author><name sortKey="Tranchant, Christine" sort="Tranchant, Christine" uniqKey="Tranchant C" first="Christine" last="Tranchant">Christine Tranchant</name><affiliation wicri:level="1"><country xml:lang="fr">France</country><wicri:regionArea>Service des Maladies du Système Nerveux et du Muscle, Hôpitaux Universitaires de Strasbourg</wicri:regionArea><wicri:noRegion>Hôpitaux Universitaires de Strasbourg</wicri:noRegion><wicri:noRegion>Hôpitaux Universitaires de Strasbourg</wicri:noRegion></affiliation></author><author><name sortKey="Maquet, Pierre" sort="Maquet, Pierre" uniqKey="Maquet P" first="Pierre" last="Maquet">Pierre Maquet</name><affiliation wicri:level="1"><country xml:lang="fr">Belgique</country><wicri:regionArea>Unité Médicale du Cyclotron, CHU Sart Tilman, Liège</wicri:regionArea><wicri:noRegion>Liège</wicri:noRegion></affiliation></author><author><name sortKey="Marescaux, Christian" sort="Marescaux, Christian" uniqKey="Marescaux C" first="Christian" last="Marescaux">Christian Marescaux</name><affiliation><wicri:noCountry code="subField">Neuropsychologie et Explorations Fonctionnelles des Epilepsies</wicri:noCountry></affiliation></author><author><name sortKey="Sellal, Francois" sort="Sellal, Francois" uniqKey="Sellal F" first="François" last="Sellal">François Sellal</name><affiliation><wicri:noCountry code="subField">Neuropsychologie et Explorations Fonctionnelles des Epilepsies</wicri:noCountry></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</title><title level="j" type="abbrev">Mov. 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>Age of Onset</term><term>Atrophy</term><term>Brain (metabolism)</term><term>Brain (pathology)</term><term>Brain (radionuclide imaging)</term><term>Brain metabolism</term><term>Case study</term><term>Cerebrovascular Circulation</term><term>Diagnosis</term><term>Diagnosis, Differential</term><term>Disease Progression</term><term>Dystonia</term><term>Dystonic Disorders (diagnosis)</term><term>Dystonic Disorders (etiology)</term><term>Dystonic Disorders (pathology)</term><term>Dystonic Disorders (physiopathology)</term><term>Elderly</term><term>Emission tomography</term><term>Evolution</term><term>Fatal Outcome</term><term>Functional Laterality</term><term>Globus Pallidus (pathology)</term><term>Globus Pallidus (radionuclide imaging)</term><term>Hemidystonia</term><term>Humans</term><term>Inaugural sign</term><term>Locus niger</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>Nuclear magnetic resonance imaging</term><term>Pallidal degeneration</term><term>Pallido‐luysio‐nigral atrophy</term><term>Pallidum</term><term>Pathology</term><term>Photon</term><term>Positron</term><term>Substantia Nigra (pathology)</term><term>Substantia Nigra (radionuclide imaging)</term><term>Subthalamic nucleus</term><term>Thalamocortical perfusion</term><term>Thalamus (pathology)</term><term>Tomography, Emission-Computed</term><term>Tomography, Emission-Computed, Single-Photon</term><term>Unilateral</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><keywords scheme="Pascal" xml:lang="fr"><term>Anatomopathologie</term><term>Atrophie</term><term>Diagnostic</term><term>Dystonie</term><term>Etude cas</term><term>Evolution</term><term>Imagerie RMN</term><term>Locus niger</term><term>Mâle</term><term>Noyau sousthalamique</term><term>Pallidum</term><term>Personne âgée</term><term>Photon</term><term>Positon</term><term>Signe inaugural</term><term>Tomoscintigraphie</term><term>Unilatéral</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Personne âgée</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><affiliations><list><country><li>Belgique</li><li>France</li></country></list><tree><noCountry><name sortKey="Andriantseheno, Marcellin L" sort="Andriantseheno, Marcellin L" uniqKey="Andriantseheno M" first="Marcellin L." last="Andriantseheno">Marcellin L. Andriantseheno</name><name sortKey="Hammouti, Abdallah" sort="Hammouti, Abdallah" uniqKey="Hammouti A" first="Abdallah" last="Hammouti">Abdallah Hammouti</name><name sortKey="Marescaux, Christian" sort="Marescaux, Christian" uniqKey="Marescaux C" first="Christian" last="Marescaux">Christian Marescaux</name><name sortKey="Mohr, Michel" sort="Mohr, Michel" uniqKey="Mohr M" first="Michel" last="Mohr">Michel Mohr</name><name sortKey="Sellal, Francois" sort="Sellal, Francois" uniqKey="Sellal F" first="François" last="Sellal">François Sellal</name><name sortKey="Vercueil, Laurent" sort="Vercueil, Laurent" uniqKey="Vercueil L" first="Laurent" last="Vercueil">Laurent Vercueil</name></noCountry><country name="France"><noRegion><name sortKey="Tranchant, Christine" sort="Tranchant, Christine" uniqKey="Tranchant C" first="Christine" last="Tranchant">Christine Tranchant</name></noRegion></country><country name="Belgique"><noRegion><name sortKey="Maquet, Pierre" sort="Maquet, Pierre" uniqKey="Maquet P" first="Pierre" last="Maquet">Pierre Maquet</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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