Severe generalised dystonia associated with a mosaic pattern of striatal gliosis
Identifieur interne : 005F31 ( Main/Exploration ); précédent : 005F30; suivant : 005F32Severe generalised dystonia associated with a mosaic pattern of striatal gliosis
Auteurs : Gibb [Royaume-Uni] ; L. Kilford [Royaume-Uni] ; C. D. Marsden [Royaume-Uni]Source :
- Movement Disorders [ 0885-3185 ] ; 1992.
English descriptors
- KwdEn :
- Adolescent, Astrocytes (pathology), Calbindin, Chlormethiazole (poisoning), Corpus Striatum (pathology), Drug Overdose (pathology), Dystonia, Dystonia Musculorum Deformans (pathology), Glial Fibrillary Acidic Protein (analysis), Gliosis (pathology), Humans, Huntington's disease, Hypoxia, Male, Neuroacanthocytosis, Putamen (pathology), Striatum.
- MESH :
- chemical , analysis : Glial Fibrillary Acidic Protein.
- chemical , poisoning : Chlormethiazole.
- pathology : Astrocytes, Corpus Striatum, Drug Overdose, Dystonia Musculorum Deformans, Gliosis, Putamen.
- Adolescent, Humans, Male.
Abstract
A mosaic pattern of striatal pathology is described in a male who developed severe generalised dystonia from the age of 10 years, and died at the age of 18 years. There was no family history of dystonia, and extensive investigations during his life failed to identify a cause for the dystonia. The caudate nucleus and putamen showed a network of cell loss and gliosis surrounding islands of preserved striatum. Dorsal parts showed confluent gliosis, and ventral parts were spared. The pattern suggested a correlation with patch‐matrix organisation, but there was no correlation with the distribution of calbindin immunoreactive cells, which are present in the matrix of the classical striosome‐matrix organisation. The pathological findings were unlike those in status marmoratus, perinatal hypoxia‐ischaemia, Huntington's disease, and neuroacanthocytosis, but similar to those reported in a 44‐year‐old man with predominantly cranial dystonia.
Url:
DOI: 10.1002/mds.870070305
Affiliations:
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Marsden</name><affiliation wicri:level="3"><country xml:lang="fr">Royaume-Uni</country><wicri:regionArea>Parkinson's Disease Society Brain Bank, Institute of Neurology and the National Hospital for Neurology and Neurosurgery, London</wicri:regionArea><placeName><settlement type="city">Londres</settlement><region type="country">Angleterre</region><region type="région" nuts="1">Grand Londres</region></placeName></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</title><title level="j" type="sub">Official Journal of the Movement Disorder Society</title><title level="j" type="abbrev">Mov. Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="1992">1992</date><biblScope unit="vol">7</biblScope><biblScope unit="issue">3</biblScope><biblScope unit="page" from="217">217</biblScope><biblScope unit="page" to="223">223</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">B66D370AE7A3B9394170AF5607BA97381091FC9B</idno><idno type="DOI">10.1002/mds.870070305</idno><idno type="ArticleID">MDS870070305</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term><term>Astrocytes (pathology)</term><term>Calbindin</term><term>Chlormethiazole (poisoning)</term><term>Corpus Striatum (pathology)</term><term>Drug Overdose (pathology)</term><term>Dystonia</term><term>Dystonia Musculorum Deformans (pathology)</term><term>Glial Fibrillary Acidic Protein (analysis)</term><term>Gliosis (pathology)</term><term>Humans</term><term>Huntington's disease</term><term>Hypoxia</term><term>Male</term><term>Neuroacanthocytosis</term><term>Putamen (pathology)</term><term>Striatum</term></keywords><keywords scheme="MESH" type="chemical" qualifier="analysis" xml:lang="en"><term>Glial Fibrillary Acidic Protein</term></keywords><keywords scheme="MESH" type="chemical" qualifier="poisoning" xml:lang="en"><term>Chlormethiazole</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Astrocytes</term><term>Corpus Striatum</term><term>Drug Overdose</term><term>Dystonia Musculorum Deformans</term><term>Gliosis</term><term>Putamen</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adolescent</term><term>Humans</term><term>Male</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">A mosaic pattern of striatal pathology is described in a male who developed severe generalised dystonia from the age of 10 years, and died at the age of 18 years. There was no family history of dystonia, and extensive investigations during his life failed to identify a cause for the dystonia. The caudate nucleus and putamen showed a network of cell loss and gliosis surrounding islands of preserved striatum. Dorsal parts showed confluent gliosis, and ventral parts were spared. The pattern suggested a correlation with patch‐matrix organisation, but there was no correlation with the distribution of calbindin immunoreactive cells, which are present in the matrix of the classical striosome‐matrix organisation. The pathological findings were unlike those in status marmoratus, perinatal hypoxia‐ischaemia, Huntington's disease, and neuroacanthocytosis, but similar to those reported in a 44‐year‐old man with predominantly cranial dystonia.</div></front></TEI><affiliations><list><country><li>Royaume-Uni</li></country><region><li>Angleterre</li><li>Grand Londres</li></region><settlement><li>Londres</li></settlement></list><tree><country name="Royaume-Uni"><region name="Angleterre"><name sortKey="Gibb" sort="Gibb" uniqKey="Gibb" last="Gibb">Gibb</name></region><name sortKey="Kilford, L" sort="Kilford, L" uniqKey="Kilford L" first="L." last="Kilford">L. Kilford</name><name sortKey="Marsden, C D" sort="Marsden, C D" uniqKey="Marsden C" first="C. D." last="Marsden">C. D. Marsden</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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