Pallido‐luysio‐nigral atrophy revealed by rapidly progressive hemidystonia: A clinical, radiologic, functional, and neuropathologic study
Identifieur interne : 001734 ( Istex/Curation ); 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 [France] ; Pierre Maquet [Belgique] ; Christian Marescaux ; François SellalSource :
- Movement Disorders [ 0885-3185 ] ; 2000-09.
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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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Laurent Vercueil<affiliation><mods:affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</mods:affiliation><wicri:noCountry code="subField">Neuropsychologie et Explorations Fonctionnelles des Epilepsies</wicri:noCountry></affiliation><affiliation><mods:affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</mods:affiliation><wicri:noCountry code="subField">Neuropsychologie et Explorations Fonctionnelles des Epilepsies</wicri:noCountry></affiliation><affiliation><mods:affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</mods:affiliation><wicri:noCountry code="subField">Neuropsychologie et Explorations Fonctionnelles des Epilepsies</wicri:noCountry></affiliation><affiliation><mods:affiliation>Institut d'Anatomie Pathologique</mods:affiliation><wicri:noCountry code="no comma">Institut d'Anatomie Pathologique</wicri:noCountry></affiliation><affiliation><mods:affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</mods:affiliation><wicri:noCountry code="subField">Neuropsychologie et Explorations Fonctionnelles des Epilepsies</wicri:noCountry></affiliation><affiliation><mods:affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</mods:affiliation><wicri:noCountry code="subField">Neuropsychologie et Explorations Fonctionnelles des Epilepsies</wicri:noCountry></affiliation>Le document en format XML
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Andriantseheno</name><affiliation><mods:affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</mods: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><mods:affiliation>Institut d'Anatomie Pathologique</mods: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"><mods:affiliation>Service des Maladies du Système Nerveux et du Muscle, Hôpitaux Universitaires de Strasbourg, France</mods:affiliation><country xml:lang="fr">France</country><wicri:regionArea>Service des Maladies du Système Nerveux et du Muscle, Hôpitaux Universitaires de Strasbourg</wicri:regionArea></affiliation></author><author><name sortKey="Maquet, Pierre" sort="Maquet, Pierre" uniqKey="Maquet P" first="Pierre" last="Maquet">Pierre Maquet</name><affiliation wicri:level="1"><mods:affiliation>Unité Médicale du Cyclotron, CHU Sart Tilman, Liège, Belgique</mods:affiliation><country xml:lang="fr">Belgique</country><wicri:regionArea>Unité Médicale du Cyclotron, CHU Sart Tilman, Liège</wicri:regionArea></affiliation></author><author><name sortKey="Marescaux, Christian" sort="Marescaux, Christian" uniqKey="Marescaux C" first="Christian" last="Marescaux">Christian Marescaux</name><affiliation><mods:affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</mods: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><mods:affiliation>Service de Neurologie, Neuropsychologie et Explorations Fonctionnelles des Epilepsies</mods: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>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></record>Pour manipuler ce document sous Unix (Dilib)
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