MovDisordV3, PascalFrancis, Corpus, bibRecord, 001863

Neurological manifestations in Wilson's disease : Report of 119 cases

Identifieur interne : 001863 ( PascalFrancis/Corpus ); précédent : 001862; suivant : 001864

Neurological manifestations in Wilson's disease : Report of 119 cases

Auteurs : Alexandre Machado ; HSIN FEN CHIEN ; Marta Mitiko Deguti ; Eduardo Cancado ; Raymundo Soares Azevedo ; Milberto Scaff ; Egberto Reis Barbosa

Source :

Movement disorders [ 0885-3185 ] ; 2006.

RBID : Pascal:07-0090876

Descripteurs français

Pascal (Inist)
- Système nerveux pathologie, Wilson maladie, Etude cas, Cuivre.

English descriptors

KwdEn :
- Case study, Copper, Nervous system diseases, Wilson disease.

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%).

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

A01	`01`	`1`		`@0 0885-3185`
A03		`1`		`@0 Mov. disord.`
A05				`@2 21`
A06				`@2 12`
A08	`01`	`1`	`ENG`	`@1 Neurological manifestations in Wilson's disease : Report of 119 cases`
A11	`01`	`1`		`@1 MACHADO (Alexandre)`
A11	`02`	`1`		`@1 HSIN FEN CHIEN`
A11	`03`	`1`		`@1 MITIKO DEGUTI (Marta)`
A11	`04`	`1`		`@1 CANCADO (Eduardo)`
A11	`05`	`1`		`@1 SOARES AZEVEDO (Raymundo)`
A11	`06`	`1`		`@1 SCAFF (Milberto)`
A11	`07`	`1`		`@1 REIS BARBOSA (Egberto)`
A14	`01`			`@1 Department of Neurology, University of São Paulo School of Medicine @2 São Paulo @3 BRA @Z 1 aut. @Z 2 aut. @Z 6 aut. @Z 7 aut.`
A14	`02`			`@1 Department of Hepatology and Gastroenterology, University of São Paulo School of Medicine @2 São Paulo @3 BRA @Z 3 aut. @Z 4 aut.`
A14	`03`			`@1 Department of Pathology, University of São Paulo School of Medicine @2 São Paulo @3 BRA @Z 5 aut.`
A20				`@1 2192-2196`
A21				`@1 2006`
A23	`01`			`@0 ENG`
A43	`01`			`@1 INIST @2 20953 @5 354000145356790260`
A44				`@0 0000 @1 © 2007 INIST-CNRS. All rights reserved.`
A45				`@0 29 ref.`
A47	`01`	`1`		`@0 07-0090876`
A60				`@1 P`
A61				`@0 A`
A64	`01`	`1`		`@0 Movement disorders`
A66	`01`			`@0 USA`
C01	`01`		`ENG`	@0 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%).
C02	`01`	`X`		`@0 002B17`
C02	`02`	`X`		`@0 002B17G`
C02	`03`	`X`		`@0 002B17E`
C03	`01`	`X`	`FRE`	`@0 Système nerveux pathologie @5 01`
C03	`01`	`X`	`ENG`	`@0 Nervous system diseases @5 01`
C03	`01`	`X`	`SPA`	`@0 Sistema nervioso patología @5 01`
C03	`02`	`X`	`FRE`	`@0 Wilson maladie @5 02`
C03	`02`	`X`	`ENG`	`@0 Wilson disease @5 02`
C03	`02`	`X`	`SPA`	`@0 Wilson enfermedad @5 02`
C03	`03`	`X`	`FRE`	`@0 Etude cas @5 09`
C03	`03`	`X`	`ENG`	`@0 Case study @5 09`
C03	`03`	`X`	`SPA`	`@0 Estudio caso @5 09`
C03	`04`	`X`	`FRE`	`@0 Cuivre @2 NC @5 78`
C03	`04`	`X`	`ENG`	`@0 Copper @2 NC @5 78`
C03	`04`	`X`	`SPA`	`@0 Cobre @2 NC @5 78`
C07	`01`	`X`	`FRE`	`@0 Appareil digestif pathologie @5 37`
C07	`01`	`X`	`ENG`	`@0 Digestive diseases @5 37`
C07	`01`	`X`	`SPA`	`@0 Aparato digestivo patología @5 37`
C07	`02`	`X`	`FRE`	`@0 Enzymopathie @5 38`
C07	`02`	`X`	`ENG`	`@0 Enzymopathy @5 38`
C07	`02`	`X`	`SPA`	`@0 Enzimopatía @5 38`
C07	`03`	`X`	`FRE`	`@0 Maladie héréditaire @5 39`
C07	`03`	`X`	`ENG`	`@0 Genetic disease @5 39`
C07	`03`	`X`	`SPA`	`@0 Enfermedad hereditaria @5 39`
C07	`04`	`X`	`FRE`	`@0 Métabolisme pathologie @5 40`
C07	`04`	`X`	`ENG`	`@0 Metabolic diseases @5 40`
C07	`04`	`X`	`SPA`	`@0 Metabolismo patología @5 40`
N21				`@1 057`
N44	`01`			`@1 OTO`
N82				`@1 OTO`

Format Inist (serveur)

NO :	PASCAL 07-0090876 INIST
ET :	Neurological manifestations in Wilson's disease : Report of 119 cases
AU :	MACHADO (Alexandre); HSIN FEN CHIEN; MITIKO DEGUTI (Marta); CANCADO (Eduardo); SOARES AZEVEDO (Raymundo); SCAFF (Milberto); REIS BARBOSA (Egberto)
AF :	Department of Neurology, University of São Paulo School of Medicine/São Paulo/Brésil (1 aut., 2 aut., 6 aut., 7 aut.); Department of Hepatology and Gastroenterology, University of São Paulo School of Medicine/São Paulo/Brésil (3 aut., 4 aut.); Department of Pathology, University of São Paulo School of Medicine/São Paulo/Brésil (5 aut.)
DT :	Publication en série; Niveau analytique
SO :	Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2006; Vol. 21; No. 12; Pp. 2192-2196; Bibl. 29 ref.
LA :	Anglais
EA :	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%).
CC :	002B17; 002B17G; 002B17E
FD :	Système nerveux pathologie; Wilson maladie; Etude cas; Cuivre
FG :	Appareil digestif pathologie; Enzymopathie; Maladie héréditaire; Métabolisme pathologie
ED :	Nervous system diseases; Wilson disease; Case study; Copper
EG :	Digestive diseases; Enzymopathy; Genetic disease; Metabolic diseases
SD :	Sistema nervioso patología; Wilson enfermedad; Estudio caso; Cobre
LO :	INIST-20953.354000145356790260
ID :	07-0090876

Links to Exploration step

Pascal:07-0090876

Le document en format XML

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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Neurological manifestations in Wilson's disease : Report of 119 cases</title>
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<author><name sortKey="Soares Azevedo, Raymundo" sort="Soares Azevedo, Raymundo" uniqKey="Soares Azevedo R" first="Raymundo" last="Soares Azevedo">Raymundo Soares Azevedo</name>
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<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%).</div>
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<sZ>4 aut.</sZ>
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<fA14 i1="03"><s1>Department of Pathology, University of São Paulo School of Medicine</s1>
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<fC01 i1="01" l="ENG"><s0>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%).</s0>
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<s5>39</s5>
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<s5>40</s5>
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<fC07 i1="04" i2="X" l="SPA"><s0>Metabolismo patología</s0>
<s5>40</s5>
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<server><NO>PASCAL 07-0090876 INIST</NO>
<ET>Neurological manifestations in Wilson's disease : Report of 119 cases</ET>
<AU>MACHADO (Alexandre); HSIN FEN CHIEN; MITIKO DEGUTI (Marta); CANCADO (Eduardo); SOARES AZEVEDO (Raymundo); SCAFF (Milberto); REIS BARBOSA (Egberto)</AU>
<AF>Department of Neurology, University of São Paulo School of Medicine/São Paulo/Brésil (1 aut., 2 aut., 6 aut., 7 aut.); Department of Hepatology and Gastroenterology, University of São Paulo School of Medicine/São Paulo/Brésil (3 aut., 4 aut.); Department of Pathology, University of São Paulo School of Medicine/São Paulo/Brésil (5 aut.)</AF>
<DT>Publication en série; Niveau analytique</DT>
<SO>Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2006; Vol. 21; No. 12; Pp. 2192-2196; Bibl. 29 ref.</SO>
<LA>Anglais</LA>
<EA>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%).</EA>
<CC>002B17; 002B17G; 002B17E</CC>
<FD>Système nerveux pathologie; Wilson maladie; Etude cas; Cuivre</FD>
<FG>Appareil digestif pathologie; Enzymopathie; Maladie héréditaire; Métabolisme pathologie</FG>
<ED>Nervous system diseases; Wilson disease; Case study; Copper</ED>
<EG>Digestive diseases; Enzymopathy; Genetic disease; Metabolic diseases</EG>
<SD>Sistema nervioso patología; Wilson enfermedad; Estudio caso; Cobre</SD>
<LO>INIST-20953.354000145356790260</LO>
<ID>07-0090876</ID>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Neurological manifestations in Wilson's disease : Report of 119 cases

Neurological manifestations in Wilson's disease : Report of 119 cases

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