Unusual late-onset type of Hallervorden-Spatz disease
Identifieur interne : 001F90 ( Main/Corpus ); précédent : 001F89; suivant : 001F91Unusual late-onset type of Hallervorden-Spatz disease
Auteurs : K. Jellinger ; E. NeumayerSource :
- 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><mods:affiliation>Neurological Institute, University of Vienna, Vienna-Lainz, Austria</mods:affiliation></affiliation><affiliation><mods:affiliation>Department of Neurology, Hospital for Chronic Diseases, Vienna-Lainz, Austria</mods:affiliation></affiliation></author><author><name sortKey="Neumayer, E" sort="Neumayer, E" uniqKey="Neumayer E" first="E." last="Neumayer">E. Neumayer</name><affiliation><mods:affiliation>Neurological Institute, University of Vienna, Vienna-Lainz, Austria</mods:affiliation></affiliation><affiliation><mods:affiliation>Department of Neurology, Hospital for Chronic Diseases, Vienna-Lainz, Austria</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">Zeitschrift für Neurologie</title><title level="j" type="abbrev">Z. 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Neurol.</title><idno type="JournalID">415</idno><idno type="pISSN">0012-1037</idno><idno type="eISSN">1432-1459</idno><idno type="IssueArticleCount">10</idno><idno type="VolumeIssueCount">4</idno><imprint><publisher>Springer-Verlag</publisher><pubPlace>Berlin/Heidelberg</pubPlace><date type="published" when="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></monogr><idno type="istex">BBC2E8D0A38422EDF3611D0F9A6285708464976D</idno><idno type="DOI">10.1007/BF00316039</idno><idno type="ArticleID">Art2</idno><idno type="ArticleID">BF00316039</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>1972-06-21</date></creation><langUsage><language ident="en">en</language></langUsage><abstract xml:lang="en"><p>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.</p></abstract><abstract xml:lang="de"><p>Zusammenfassung: Eine 64jährige Frau bot klinisch ein progressives rigid-akinetisches Parkinson-Syndrom mit initialem Tremor und terminalem Verwirrtheitszustand im Anschluß an l-DOPA-Behandlung. Der Tod trat nach 7jähriger Krankheitsdauer im Alter von 70,5 Jahren ein. Neuropathologisch ergab sich 1. eine starke Hyperpigmentation von Pallidum und Zona reticularis nigrae mit schwerer neuroaxonaler Dystrophie in beiden Kerngebieten sowie Auftreten von Axonschollen in anderen Gebieten des ZNS (Ansa lenticularis, inneres Putamen, Corpus Luysii und Inselrinde) sowie 2. schwere symmetrische Atrophie und Gliose des Corpus subthalamicum und der Zona compacta nigrae bei Erhaltung der übrigen melaninhaltigen Hirnstammkerne. Aus dem histopathologischen Befund wird das Zusammentreffen einer adulten Form der Hallervorden-Spatzschen Krankheit (älteste bekannte Beobachtung) mit einer Degeneration der pigmentierten Neurone der Substantia nigra angenommen. Die letztgenannten Veränderungen waren offenbar für das hervorstechende klinische Syndrom verantwortlich. Die nosologischen Beziehungen von Spätformen der Hallervorden-Spatzschen Krankheit zu anderen degenerativen Erkrankungen des Nervensystems werden erörtert.</p></abstract><textClass><keywords scheme="Journal Subject"><list><head>Medicine & Public Health</head><item><term>Neurosciences</term></item><item><term>Neuroradiology</term></item><item><term>Neurology</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="1972-06-21">Created</change><change when="1972-06-01">Published</change><change xml:id="refBibs-istex" who="#ISTEX-API" when="2016-3-2">References added</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><original>false</original><mimetype>text/plain</mimetype><extension>txt</extension><uri>https://api.istex.fr/document/BBC2E8D0A38422EDF3611D0F9A6285708464976D/fulltext/txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Springer, Publisher found" wicri:toSee="no header"><istex:xmlDeclaration>version="1.0" encoding="UTF-8"</istex:xmlDeclaration><istex:docType PUBLIC="-//Springer-Verlag//DTD A++ V2.4//EN" URI="http://devel.springer.de/A++/V2.4/DTD/A++V2.4.dtd" name="istex:docType"></istex:docType><istex:document><Publisher><PublisherInfo><PublisherName>Springer-Verlag</PublisherName><PublisherLocation>Berlin/Heidelberg</PublisherLocation></PublisherInfo><Journal><JournalInfo JournalProductType="ArchiveJournal" NumberingStyle="Unnumbered"><JournalID>415</JournalID><JournalPrintISSN>0012-1037</JournalPrintISSN><JournalElectronicISSN>1432-1459</JournalElectronicISSN><JournalTitle>Zeitschrift für Neurologie</JournalTitle><JournalAbbreviatedTitle>Z. 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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.</Para></Abstract><Abstract ID="Abs2" Language="De"><Heading>Zusammenfassung</Heading><Para>Eine 64jährige Frau bot klinisch ein progressives rigid-akinetisches Parkinson-Syndrom mit initialem Tremor und terminalem Verwirrtheitszustand im Anschluß an <Emphasis Type="SmallCaps">l</Emphasis>-DOPA-Behandlung. Der Tod trat nach 7jähriger Krankheitsdauer im Alter von 70,5 Jahren ein. Neuropathologisch ergab sich 1. eine starke Hyperpigmentation von Pallidum und Zona reticularis nigrae mit schwerer neuroaxonaler Dystrophie in beiden Kerngebieten sowie Auftreten von Axonschollen in anderen Gebieten des ZNS (Ansa lenticularis, inneres Putamen, Corpus Luysii und Inselrinde) sowie 2. schwere symmetrische Atrophie und Gliose des Corpus subthalamicum und der Zona compacta nigrae bei Erhaltung der übrigen melaninhaltigen Hirnstammkerne. Aus dem histopathologischen Befund wird das Zusammentreffen einer adulten Form der Hallervorden-Spatzschen Krankheit (älteste bekannte Beobachtung) mit einer Degeneration der pigmentierten Neurone der Substantia nigra angenommen. Die letztgenannten Veränderungen waren offenbar für das hervorstechende klinische Syndrom verantwortlich. Die nosologischen Beziehungen von Spätformen der Hallervorden-Spatzschen Krankheit zu anderen degenerativen Erkrankungen des Nervensystems werden erörtert.</Para></Abstract><KeywordGroup Language="En"><Heading>Key words</Heading><Keyword>Hallervorden-Spatz Disease</Keyword><Keyword>Pallido-Nigral Hyperpigmentation</Keyword><Keyword>Neuroaxonal Dystrophy</Keyword><Keyword>Parkinsonism</Keyword><Keyword>CNS Degenerations</Keyword></KeywordGroup></ArticleHeader><NoBody></NoBody></Article></Issue></Volume></Journal></Publisher></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Unusual late-onset type of Hallervorden-Spatz disease</title><subTitle>Clinico-pathological study of a case presenting as parkinsonism</subTitle></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Unusual late-onset type of Hallervorden-Spatz disease</title><subTitle>Clinico-pathological study of a case presenting as parkinsonism</subTitle></titleInfo><name type="personal"><namePart type="given">K.</namePart><namePart type="family">Jellinger</namePart><affiliation>Neurological Institute, University of Vienna, Vienna-Lainz, Austria</affiliation><affiliation>Department of Neurology, Hospital for Chronic Diseases, Vienna-Lainz, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">E.</namePart><namePart type="family">Neumayer</namePart><affiliation>Neurological Institute, University of Vienna, Vienna-Lainz, Austria</affiliation><affiliation>Department of Neurology, Hospital for Chronic Diseases, Vienna-Lainz, Austria</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="research-article" displayLabel="OriginalPaper"></genre><originInfo><publisher>Springer-Verlag</publisher><place><placeTerm type="text">Berlin/Heidelberg</placeTerm></place><dateCreated encoding="w3cdtf">1972-06-21</dateCreated><dateIssued encoding="w3cdtf">1972-06-01</dateIssued><copyrightDate encoding="w3cdtf">1972</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType></physicalDescription><abstract 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.</abstract><abstract lang="de">Zusammenfassung: Eine 64jährige Frau bot klinisch ein progressives rigid-akinetisches Parkinson-Syndrom mit initialem Tremor und terminalem Verwirrtheitszustand im Anschluß an l-DOPA-Behandlung. Der Tod trat nach 7jähriger Krankheitsdauer im Alter von 70,5 Jahren ein. Neuropathologisch ergab sich 1. eine starke Hyperpigmentation von Pallidum und Zona reticularis nigrae mit schwerer neuroaxonaler Dystrophie in beiden Kerngebieten sowie Auftreten von Axonschollen in anderen Gebieten des ZNS (Ansa lenticularis, inneres Putamen, Corpus Luysii und Inselrinde) sowie 2. schwere symmetrische Atrophie und Gliose des Corpus subthalamicum und der Zona compacta nigrae bei Erhaltung der übrigen melaninhaltigen Hirnstammkerne. Aus dem histopathologischen Befund wird das Zusammentreffen einer adulten Form der Hallervorden-Spatzschen Krankheit (älteste bekannte Beobachtung) mit einer Degeneration der pigmentierten Neurone der Substantia nigra angenommen. Die letztgenannten Veränderungen waren offenbar für das hervorstechende klinische Syndrom verantwortlich. Die nosologischen Beziehungen von Spätformen der Hallervorden-Spatzschen Krankheit zu anderen degenerativen Erkrankungen des Nervensystems werden erörtert.</abstract><note>Original Investigations</note><relatedItem type="host"><titleInfo><title>Zeitschrift für Neurologie</title></titleInfo><titleInfo type="abbreviated"><title>Z. 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