Neurological manifestations in Wilson's disease : Report of 119 cases
Identifieur interne : 001863 ( PascalFrancis/Corpus ); précédent : 001862; suivant : 001864Neurological 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 BarbosaSource :
- Movement disorders [ 0885-3185 ] ; 2006.
Descripteurs français
- Pascal (Inist)
English descriptors
- KwdEn :
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.
pA |
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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:07-0090876Le document en format XML
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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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<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>
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<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>
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