MovDisordV3, PascalFrancis, Corpus, bibRecord, 001873

Wilson's disease tremor is associated with magnetic resonance imaging lesions in basal ganglia structures

Identifieur interne : 001873 ( PascalFrancis/Corpus ); précédent : 001872; suivant : 001874

Wilson's disease tremor is associated with magnetic resonance imaging lesions in basal ganglia structures

Auteurs : Martin Südmeyer ; Andreas Saleh ; Lars Wojtecki ; Mathias Cohnen ; Joachim Gross ; Markus Ploner ; Harald Hefter ; Lars Timmermann ; Alfons Schnitzler

Source :

Movement disorders [ 0885-3185 ] ; 2006.

RBID : Pascal:07-0090866

Descripteurs français

Pascal (Inist)
- Système nerveux pathologie, Wilson maladie, Tremblement, Imagerie RMN, Noyau gris central, Cuivre.

English descriptors

KwdEn :
- Basal ganglion, Copper, Nervous system diseases, Nuclear magnetic resonance imaging, Tremor, Wilson disease.

Abstract

Wilson's disease (WD) is an inherited disorder of copper metabolism yielding marked motor deficits, including a severely disabling tremor. As a structural correlate of the disease, a variety of cerebral abnormalities has been revealed. However, the relationship between motor deficits and cerebral lesions has remained largely unknown. Here, we investigated correlation between WD tremor and cerebral magnetic resonance imaging (MRI) findings. Cerebral MRI abnormalities in 6 symptomatic WD patients were compared to findings in 6 asymptomatic WD patients and 10 healthy controls. All patients were treated with long-term copper chelating therapy. Motor symptoms including tremor were determined by Unified Parkinson's Disease Rating Scale Part III (UPDRS-III). MRI findings in symptomatic WD patients revealed significant symmetric T2*-weighted hypointense signal alterations of globus pallidus, head of the caudate nucleus, and substantia nigra. In contrast, MRI of asymptomatic WD patients did not differ from healthy controls. Correlation analysis revealed a significant positive correlation between MRI basal ganglia lesions and UPDRS action tremor score. Our results demonstrate for the first time that Wilson's disease tremor is associated with lesions of the globus pallidus, the head of the caudate nucleus, and the substantia nigra.

Notice en format standard (ISO 2709)

Pour connaître la documentation sur le format Inist Standard.

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A11	`05`	`1`		`@1 GROSS (Joachim)`
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A14	`01`			`@1 Department of Neurology, Heinrich-Heine University @2 Düsseldorf @3 DEU @Z 1 aut. @Z 3 aut. @Z 5 aut. @Z 6 aut. @Z 7 aut. @Z 8 aut. @Z 9 aut.`
A14	`02`			`@1 Institute of Diagnostic Radiology, University Hospital @2 Düsseldorf @3 DEU @Z 2 aut. @Z 4 aut.`
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A44				`@0 0000 @1 © 2007 INIST-CNRS. All rights reserved.`
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C01	`01`		`ENG`	@0 Wilson's disease (WD) is an inherited disorder of copper metabolism yielding marked motor deficits, including a severely disabling tremor. As a structural correlate of the disease, a variety of cerebral abnormalities has been revealed. However, the relationship between motor deficits and cerebral lesions has remained largely unknown. Here, we investigated correlation between WD tremor and cerebral magnetic resonance imaging (MRI) findings. Cerebral MRI abnormalities in 6 symptomatic WD patients were compared to findings in 6 asymptomatic WD patients and 10 healthy controls. All patients were treated with long-term copper chelating therapy. Motor symptoms including tremor were determined by Unified Parkinson's Disease Rating Scale Part III (UPDRS-III). MRI findings in symptomatic WD patients revealed significant symmetric T2*-weighted hypointense signal alterations of globus pallidus, head of the caudate nucleus, and substantia nigra. In contrast, MRI of asymptomatic WD patients did not differ from healthy controls. Correlation analysis revealed a significant positive correlation between MRI basal ganglia lesions and UPDRS action tremor score. Our results demonstrate for the first time that Wilson's disease tremor is associated with lesions of the globus pallidus, the head of the caudate nucleus, and the substantia nigra.
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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`
C07	`05`	`X`	`FRE`	`@0 Mouvement involontaire @5 41`
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C07	`05`	`X`	`SPA`	`@0 Movimiento involuntario @5 41`
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C07	`07`	`X`	`FRE`	`@0 Encéphale @5 43`
C07	`07`	`X`	`ENG`	`@0 Encephalon @5 43`
C07	`07`	`X`	`SPA`	`@0 Encéfalo @5 43`
C07	`08`	`X`	`FRE`	`@0 Système nerveux central @5 44`
C07	`08`	`X`	`ENG`	`@0 Central nervous system @5 44`
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N21				`@1 057`
N44	`01`			`@1 OTO`
N82				`@1 OTO`

Format Inist (serveur)

NO :	PASCAL 07-0090866 INIST
ET :	Wilson's disease tremor is associated with magnetic resonance imaging lesions in basal ganglia structures
AU :	SÜDMEYER (Martin); SALEH (Andreas); WOJTECKI (Lars); COHNEN (Mathias); GROSS (Joachim); PLONER (Markus); HEFTER (Harald); TIMMERMANN (Lars); SCHNITZLER (Alfons)
AF :	Department of Neurology, Heinrich-Heine University/Düsseldorf/Allemagne (1 aut., 3 aut., 5 aut., 6 aut., 7 aut., 8 aut., 9 aut.); Institute of Diagnostic Radiology, University Hospital/Düsseldorf/Allemagne (2 aut., 4 aut.)
DT :	Publication en série; Niveau analytique
SO :	Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2006; Vol. 21; No. 12; Pp. 2134-2139; Bibl. 24 ref.
LA :	Anglais
EA :	Wilson's disease (WD) is an inherited disorder of copper metabolism yielding marked motor deficits, including a severely disabling tremor. As a structural correlate of the disease, a variety of cerebral abnormalities has been revealed. However, the relationship between motor deficits and cerebral lesions has remained largely unknown. Here, we investigated correlation between WD tremor and cerebral magnetic resonance imaging (MRI) findings. Cerebral MRI abnormalities in 6 symptomatic WD patients were compared to findings in 6 asymptomatic WD patients and 10 healthy controls. All patients were treated with long-term copper chelating therapy. Motor symptoms including tremor were determined by Unified Parkinson's Disease Rating Scale Part III (UPDRS-III). MRI findings in symptomatic WD patients revealed significant symmetric T2*-weighted hypointense signal alterations of globus pallidus, head of the caudate nucleus, and substantia nigra. In contrast, MRI of asymptomatic WD patients did not differ from healthy controls. Correlation analysis revealed a significant positive correlation between MRI basal ganglia lesions and UPDRS action tremor score. Our results demonstrate for the first time that Wilson's disease tremor is associated with lesions of the globus pallidus, the head of the caudate nucleus, and the substantia nigra.
CC :	002B17; 002B17G; 002B24A06
FD :	Système nerveux pathologie; Wilson maladie; Tremblement; Imagerie RMN; Noyau gris central; Cuivre
FG :	Appareil digestif pathologie; Enzymopathie; Maladie héréditaire; Métabolisme pathologie; Mouvement involontaire; Trouble neurologique; Encéphale; Système nerveux central
ED :	Nervous system diseases; Wilson disease; Tremor; Nuclear magnetic resonance imaging; Basal ganglion; Copper
EG :	Digestive diseases; Enzymopathy; Genetic disease; Metabolic diseases; Involuntary movement; Neurological disorder; Encephalon; Central nervous system
SD :	Sistema nervioso patología; Wilson enfermedad; Temblor; Imaginería RMN; Núcleo basal; Cobre
LO :	INIST-20953.354000145356790160
ID :	07-0090866

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Pascal:07-0090866

Le document en format XML

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<front><div type="abstract" xml:lang="en">Wilson's disease (WD) is an inherited disorder of copper metabolism yielding marked motor deficits, including a severely disabling tremor. As a structural correlate of the disease, a variety of cerebral abnormalities has been revealed. However, the relationship between motor deficits and cerebral lesions has remained largely unknown. Here, we investigated correlation between WD tremor and cerebral magnetic resonance imaging (MRI) findings. Cerebral MRI abnormalities in 6 symptomatic WD patients were compared to findings in 6 asymptomatic WD patients and 10 healthy controls. All patients were treated with long-term copper chelating therapy. Motor symptoms including tremor were determined by Unified Parkinson's Disease Rating Scale Part III (UPDRS-III). MRI findings in symptomatic WD patients revealed significant symmetric T2*-weighted hypointense signal alterations of globus pallidus, head of the caudate nucleus, and substantia nigra. In contrast, MRI of asymptomatic WD patients did not differ from healthy controls. Correlation analysis revealed a significant positive correlation between MRI basal ganglia lesions and UPDRS action tremor score. Our results demonstrate for the first time that Wilson's disease tremor is associated with lesions of the globus pallidus, the head of the caudate nucleus, and the substantia nigra.</div>
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<fA11 i1="01" i2="1"><s1>SÜDMEYER (Martin)</s1>
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<fC01 i1="01" l="ENG"><s0>Wilson's disease (WD) is an inherited disorder of copper metabolism yielding marked motor deficits, including a severely disabling tremor. As a structural correlate of the disease, a variety of cerebral abnormalities has been revealed. However, the relationship between motor deficits and cerebral lesions has remained largely unknown. Here, we investigated correlation between WD tremor and cerebral magnetic resonance imaging (MRI) findings. Cerebral MRI abnormalities in 6 symptomatic WD patients were compared to findings in 6 asymptomatic WD patients and 10 healthy controls. All patients were treated with long-term copper chelating therapy. Motor symptoms including tremor were determined by Unified Parkinson's Disease Rating Scale Part III (UPDRS-III). MRI findings in symptomatic WD patients revealed significant symmetric T2*-weighted hypointense signal alterations of globus pallidus, head of the caudate nucleus, and substantia nigra. In contrast, MRI of asymptomatic WD patients did not differ from healthy controls. Correlation analysis revealed a significant positive correlation between MRI basal ganglia lesions and UPDRS action tremor score. Our results demonstrate for the first time that Wilson's disease tremor is associated with lesions of the globus pallidus, the head of the caudate nucleus, and the substantia nigra.</s0>
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<server><NO>PASCAL 07-0090866 INIST</NO>
<ET>Wilson's disease tremor is associated with magnetic resonance imaging lesions in basal ganglia structures</ET>
<AU>SÜDMEYER (Martin); SALEH (Andreas); WOJTECKI (Lars); COHNEN (Mathias); GROSS (Joachim); PLONER (Markus); HEFTER (Harald); TIMMERMANN (Lars); SCHNITZLER (Alfons)</AU>
<AF>Department of Neurology, Heinrich-Heine University/Düsseldorf/Allemagne (1 aut., 3 aut., 5 aut., 6 aut., 7 aut., 8 aut., 9 aut.); Institute of Diagnostic Radiology, University Hospital/Düsseldorf/Allemagne (2 aut., 4 aut.)</AF>
<DT>Publication en série; Niveau analytique</DT>
<SO>Movement disorders; ISSN 0885-3185; Etats-Unis; Da. 2006; Vol. 21; No. 12; Pp. 2134-2139; Bibl. 24 ref.</SO>
<LA>Anglais</LA>
<EA>Wilson's disease (WD) is an inherited disorder of copper metabolism yielding marked motor deficits, including a severely disabling tremor. As a structural correlate of the disease, a variety of cerebral abnormalities has been revealed. However, the relationship between motor deficits and cerebral lesions has remained largely unknown. Here, we investigated correlation between WD tremor and cerebral magnetic resonance imaging (MRI) findings. Cerebral MRI abnormalities in 6 symptomatic WD patients were compared to findings in 6 asymptomatic WD patients and 10 healthy controls. All patients were treated with long-term copper chelating therapy. Motor symptoms including tremor were determined by Unified Parkinson's Disease Rating Scale Part III (UPDRS-III). MRI findings in symptomatic WD patients revealed significant symmetric T2*-weighted hypointense signal alterations of globus pallidus, head of the caudate nucleus, and substantia nigra. In contrast, MRI of asymptomatic WD patients did not differ from healthy controls. Correlation analysis revealed a significant positive correlation between MRI basal ganglia lesions and UPDRS action tremor score. Our results demonstrate for the first time that Wilson's disease tremor is associated with lesions of the globus pallidus, the head of the caudate nucleus, and the substantia nigra.</EA>
<CC>002B17; 002B17G; 002B24A06</CC>
<FD>Système nerveux pathologie; Wilson maladie; Tremblement; Imagerie RMN; Noyau gris central; Cuivre</FD>
<FG>Appareil digestif pathologie; Enzymopathie; Maladie héréditaire; Métabolisme pathologie; Mouvement involontaire; Trouble neurologique; Encéphale; Système nerveux central</FG>
<ED>Nervous system diseases; Wilson disease; Tremor; Nuclear magnetic resonance imaging; Basal ganglion; Copper</ED>
<EG>Digestive diseases; Enzymopathy; Genetic disease; Metabolic diseases; Involuntary movement; Neurological disorder; Encephalon; Central nervous system</EG>
<SD>Sistema nervioso patología; Wilson enfermedad; Temblor; Imaginería RMN; Núcleo basal; Cobre</SD>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Wilson's disease tremor is associated with magnetic resonance imaging lesions in basal ganglia structures

Wilson's disease tremor is associated with magnetic resonance imaging lesions in basal ganglia structures

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