Unusual late-onset type of Hallervorden-Spatz disease
Identifieur interne : 002C93 ( Main/Exploration ); précédent : 002C92; suivant : 002C94Unusual late-onset type of Hallervorden-Spatz disease
Auteurs : K. Jellinger [Autriche] ; E. Neumayer [Autriche]Source :
- Zeitschrift für Neurologie [ 0012-1037 ] ; 1972-06-01.
Abstract
Summary: A woman aged 64 presented with progressive rigid-akinetic Parkinsonian syndrome with initial tremor and terminal mental confusion following l-DOPA treatment. Death occurred at age 70.5 after 7 years' illness. Neuropathological examination disclosed: 1. excessive hyperpigmentation of the globus pallidus and reticular part of the substantia nigra associated with severe neuroaxonal dystrophy in both these nuclei, and occurrence of axonal spheroids in other parts of the CNS (ansa lenticularis, intern putamen, subthalamic nucleus, and neocortex), and 2. severe bilateral atrophy and gliosis of the subthalamic nucleus and zona compacta of the substantia nigra with preservation of the other melanin-containing nuclei. From the histopathological findings a co-existence of an adult type of Hallervorden-Spatz disease (the oldest known case) with degeneration of the pigmented neurons in the substantia nigra is suggested, the latter probably responsible for the prominent clinical features. The nosological relationship of Hallervorden-Spatz disease with late onset to other degenerative nervous disorders is discussed.
Url:
DOI: 10.1007/BF00316039
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Jellinger</name><affiliation wicri:level="1"><country xml:lang="fr">Autriche</country><wicri:regionArea>Neurological Institute, University of Vienna, Vienna-Lainz</wicri:regionArea><wicri:noRegion>Vienna-Lainz</wicri:noRegion></affiliation><affiliation wicri:level="1"><country xml:lang="fr">Autriche</country><wicri:regionArea>Department of Neurology, Hospital for Chronic Diseases, Vienna-Lainz</wicri:regionArea><wicri:noRegion>Vienna-Lainz</wicri:noRegion></affiliation></author><author><name sortKey="Neumayer, E" sort="Neumayer, E" uniqKey="Neumayer E" first="E." last="Neumayer">E. 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Neurol.</title><idno type="ISSN">0012-1037</idno><idno type="eISSN">1432-1459</idno><imprint><publisher>Springer-Verlag</publisher><pubPlace>Berlin/Heidelberg</pubPlace><date type="published" when="1972-06-01">1972-06-01</date><biblScope unit="volume">203</biblScope><biblScope unit="issue">2</biblScope><biblScope unit="page" from="105">105</biblScope><biblScope unit="page" to="118">118</biblScope></imprint><idno type="ISSN">0012-1037</idno></series><idno type="istex">BBC2E8D0A38422EDF3611D0F9A6285708464976D</idno><idno type="DOI">10.1007/BF00316039</idno><idno type="ArticleID">Art2</idno><idno type="ArticleID">BF00316039</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0012-1037</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Summary: A woman aged 64 presented with progressive rigid-akinetic Parkinsonian syndrome with initial tremor and terminal mental confusion following l-DOPA treatment. Death occurred at age 70.5 after 7 years' illness. Neuropathological examination disclosed: 1. excessive hyperpigmentation of the globus pallidus and reticular part of the substantia nigra associated with severe neuroaxonal dystrophy in both these nuclei, and occurrence of axonal spheroids in other parts of the CNS (ansa lenticularis, intern putamen, subthalamic nucleus, and neocortex), and 2. severe bilateral atrophy and gliosis of the subthalamic nucleus and zona compacta of the substantia nigra with preservation of the other melanin-containing nuclei. From the histopathological findings a co-existence of an adult type of Hallervorden-Spatz disease (the oldest known case) with degeneration of the pigmented neurons in the substantia nigra is suggested, the latter probably responsible for the prominent clinical features. The nosological relationship of Hallervorden-Spatz disease with late onset to other degenerative nervous disorders is discussed.</div></front></TEI><affiliations><list><country><li>Autriche</li></country></list><tree><country name="Autriche"><noRegion><name sortKey="Jellinger, K" sort="Jellinger, K" uniqKey="Jellinger K" first="K." last="Jellinger">K. Jellinger</name></noRegion><name sortKey="Jellinger, K" sort="Jellinger, K" uniqKey="Jellinger K" first="K." last="Jellinger">K. Jellinger</name><name sortKey="Neumayer, E" sort="Neumayer, E" uniqKey="Neumayer E" first="E." last="Neumayer">E. Neumayer</name><name sortKey="Neumayer, E" sort="Neumayer, E" uniqKey="Neumayer E" first="E." last="Neumayer">E. Neumayer</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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