Neurological manifestations in Wilson's disease: Report of 119 cases
Identifieur interne : 002E95 ( Istex/Curation ); précédent : 002E94; suivant : 002E96Neurological manifestations in Wilson's disease: Report of 119 cases
Auteurs : Alexandre Machado [Brésil] ; Hsin Fen Chien [Brésil] ; Marta Mitiko Deguti [Brésil] ; Eduardo Cançado [Brésil] ; Raymundo Soares Azevedo [Brésil] ; Milberto Scaff [Brésil] ; Egberto Reis Barbosa [Brésil]Source :
- Movement Disorders [ 0885-3185 ] ; 2006-12.
English descriptors
Abstract
We describe the neurological manifestations of 119 patients with WD (93 index cases and 26 affected family members) seen between 1963 and 2004. The mean age at symptoms onset was 19.6 years (range, 7–37 years). Medical records were reviewed for the patient's first neurological examination. The most frequent neurological manifestations observed were dysarthria (91%), gait disturbance (75%), risus sardonicus (72%), dystonia (69%), rigidity (66%), tremor (60%), and dysphagia (50%). Less frequent manifestations were chorea (16%) and athetosis (14%). Rare neurological presentations were seizures (4.2%), and pyramidal signs (3%). © 2006 Movement Disorder Society
Url:
DOI: 10.1002/mds.21170
Links toward previous steps (curation, corpus...)
- to stream Istex, to step Corpus: Pour aller vers cette notice dans l'étape Curation :002E95
Links to Exploration step
ISTEX:D5510326E3372F83B9F844233921D6EA6F8F44CELe document en format XML
<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Neurological manifestations in Wilson's disease: Report of 119 cases</title>
<author><name sortKey="Machado, Alexandre" sort="Machado, Alexandre" uniqKey="Machado A" first="Alexandre" last="Machado">Alexandre Machado</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Neurology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Neurology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Fen Chien, Hsin" sort="Fen Chien, Hsin" uniqKey="Fen Chien H" first="Hsin" last="Fen Chien">Hsin Fen Chien</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Neurology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Neurology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Mitiko Deguti, Marta" sort="Mitiko Deguti, Marta" uniqKey="Mitiko Deguti M" first="Marta" last="Mitiko Deguti">Marta Mitiko Deguti</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Hepatology and Gastroenterology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Hepatology and Gastroenterology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Cancado, Eduardo" sort="Cancado, Eduardo" uniqKey="Cancado E" first="Eduardo" last="Cançado">Eduardo Cançado</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Hepatology and Gastroenterology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Hepatology and Gastroenterology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Soares Azevedo, Raymundo" sort="Soares Azevedo, Raymundo" uniqKey="Soares Azevedo R" first="Raymundo" last="Soares Azevedo">Raymundo Soares Azevedo</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Pathology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Pathology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Scaff, Milberto" sort="Scaff, Milberto" uniqKey="Scaff M" first="Milberto" last="Scaff">Milberto Scaff</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Neurology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Neurology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Reis Barbosa, Egberto" sort="Reis Barbosa, Egberto" uniqKey="Reis Barbosa E" first="Egberto" last="Reis Barbosa">Egberto Reis Barbosa</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Neurology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Neurology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">ISTEX</idno>
<idno type="RBID">ISTEX:D5510326E3372F83B9F844233921D6EA6F8F44CE</idno>
<date when="2006" year="2006">2006</date>
<idno type="doi">10.1002/mds.21170</idno>
<idno type="url">https://api.istex.fr/document/D5510326E3372F83B9F844233921D6EA6F8F44CE/fulltext/pdf</idno>
<idno type="wicri:Area/Istex/Corpus">002E95</idno>
<idno type="wicri:Area/Istex/Curation">002E95</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Neurological manifestations in Wilson's disease: Report of 119 cases</title>
<author><name sortKey="Machado, Alexandre" sort="Machado, Alexandre" uniqKey="Machado A" first="Alexandre" last="Machado">Alexandre Machado</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Neurology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Neurology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Fen Chien, Hsin" sort="Fen Chien, Hsin" uniqKey="Fen Chien H" first="Hsin" last="Fen Chien">Hsin Fen Chien</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Neurology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Neurology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Mitiko Deguti, Marta" sort="Mitiko Deguti, Marta" uniqKey="Mitiko Deguti M" first="Marta" last="Mitiko Deguti">Marta Mitiko Deguti</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Hepatology and Gastroenterology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Hepatology and Gastroenterology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Cancado, Eduardo" sort="Cancado, Eduardo" uniqKey="Cancado E" first="Eduardo" last="Cançado">Eduardo Cançado</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Hepatology and Gastroenterology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Hepatology and Gastroenterology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Soares Azevedo, Raymundo" sort="Soares Azevedo, Raymundo" uniqKey="Soares Azevedo R" first="Raymundo" last="Soares Azevedo">Raymundo Soares Azevedo</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Pathology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Pathology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Scaff, Milberto" sort="Scaff, Milberto" uniqKey="Scaff M" first="Milberto" last="Scaff">Milberto Scaff</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Neurology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Neurology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</affiliation>
</author>
<author><name sortKey="Reis Barbosa, Egberto" sort="Reis Barbosa, Egberto" uniqKey="Reis Barbosa E" first="Egberto" last="Reis Barbosa">Egberto Reis Barbosa</name>
<affiliation wicri:level="1"><mods:affiliation>Department of Neurology, University of São Paulo School of Medicine, São Paulo, Brazil</mods:affiliation>
<country xml:lang="fr">Brésil</country>
<wicri:regionArea>Department of Neurology, University of São Paulo School of Medicine, São Paulo</wicri:regionArea>
</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="2006-12">2006-12</date>
<biblScope unit="vol">21</biblScope>
<biblScope unit="issue">12</biblScope>
<biblScope unit="page" from="2192">2192</biblScope>
<biblScope unit="page" to="2196">2196</biblScope>
</imprint>
<idno type="ISSN">0885-3185</idno>
</series>
<idno type="istex">D5510326E3372F83B9F844233921D6EA6F8F44CE</idno>
<idno type="DOI">10.1002/mds.21170</idno>
<idno type="ArticleID">MDS21170</idno>
</biblStruct>
</sourceDesc>
<seriesStmt><idno type="ISSN">0885-3185</idno>
</seriesStmt>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Wilson's disease</term>
<term>neurological manifestations</term>
</keywords>
</textClass>
<langUsage><language ident="en">en</language>
</langUsage>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">We describe the neurological manifestations of 119 patients with WD (93 index cases and 26 affected family members) seen between 1963 and 2004. The mean age at symptoms onset was 19.6 years (range, 7–37 years). Medical records were reviewed for the patient's first neurological examination. The most frequent neurological manifestations observed were dysarthria (91%), gait disturbance (75%), risus sardonicus (72%), dystonia (69%), rigidity (66%), tremor (60%), and dysphagia (50%). Less frequent manifestations were chorea (16%) and athetosis (14%). Rare neurological presentations were seizures (4.2%), and pyramidal signs (3%). © 2006 Movement Disorder Society</div>
</front>
</TEI>
</record>
Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Santé/explor/MovDisordV3/Data/Istex/Curation
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 002E95 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/Istex/Curation/biblio.hfd -nk 002E95 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien |wiki= Wicri/Santé |area= MovDisordV3 |flux= Istex |étape= Curation |type= RBID |clé= ISTEX:D5510326E3372F83B9F844233921D6EA6F8F44CE |texte= Neurological manifestations in Wilson's disease: Report of 119 cases }}
This area was generated with Dilib version V0.6.23. |