Rett syndrome and associated movement disorders
Identifieur interne : 006276 ( Main/Exploration ); précédent : 006275; suivant : 006277Rett syndrome and associated movement disorders
Auteurs : Patricia M. Fitzgerald [États-Unis] ; Jankovic [États-Unis] ; Alan K. Percy [États-Unis]Source :
- Movement Disorders [ 0885-3185 ] ; 1990.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Enfant.
English descriptors
- KwdEn :
- Adolescent, Adult, Age, Behavioral disorder, Bruxism, Case study, Child, Child, Preschool, Chorea, Clinical investigation, Degenerative disease, Diagnosis, Dystonia, Extrapyramidal syndrome, Female, Gait disturbance, Humans, Infant, Involuntary movement, Motor system disorder, Movement Disorders (etiology), Movement Disorders (physiopathology), Myoclonus, Nervous system diseases, Oculogyric crisis, Parkinsonism, Psychomotor retardation, Rett Syndrome (complications), Rett Syndrome (physiopathology), Rett syndrome, Stereotyped Behavior, Stereotypies.
- MESH :
- complications : Rett Syndrome.
- etiology : Movement Disorders.
- physiopathology : Movement Disorders, Rett Syndrome.
- Adolescent, Adult, Child, Child, Preschool, Female, Humans, Infant, Stereotyped Behavior.
Abstract
Rett syndrome, a progressive neurodegenerative disorder described only in female subjects, is manifested by a wide spectrum of behavioral and motor abnormalities. We studied 32 patients with this disorder, ages 30 months to 28 years old, and characterized their extrapyramidal disturbance. The most common motor abnormalities were stereotyped movements and gait disturbance, seen in all patients. Bruxism, oculogyric crises, parkinsonism, and dystonia were also common, but myoclonus and choreoathetosis were seen only infrequently. The hyperkinetic movement disorders tended to dominate in younger patients, while bradykinetic disorders were more evident in the older patients. This study provides evidence that movement disorders seen in Rett syndrome reflect age‐related neurodegenerative changes in the basal ganglia.
Url:
DOI: 10.1002/mds.870050303
Affiliations:
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Le document en format XML
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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="1990">1990</date><biblScope unit="vol">5</biblScope><biblScope unit="issue">3</biblScope><biblScope unit="page" from="195">195</biblScope><biblScope unit="page" to="202">202</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">0543E5C43B9E3B4B8134B9F22FDECA0089EFBCA9</idno><idno type="DOI">10.1002/mds.870050303</idno><idno type="ArticleID">MDS870050303</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Age</term><term>Behavioral disorder</term><term>Bruxism</term><term>Case study</term><term>Child</term><term>Child, Preschool</term><term>Chorea</term><term>Clinical investigation</term><term>Degenerative disease</term><term>Diagnosis</term><term>Dystonia</term><term>Extrapyramidal syndrome</term><term>Female</term><term>Gait disturbance</term><term>Humans</term><term>Infant</term><term>Involuntary movement</term><term>Motor system disorder</term><term>Movement Disorders (etiology)</term><term>Movement Disorders (physiopathology)</term><term>Myoclonus</term><term>Nervous system diseases</term><term>Oculogyric crisis</term><term>Parkinsonism</term><term>Psychomotor retardation</term><term>Rett Syndrome (complications)</term><term>Rett Syndrome (physiopathology)</term><term>Rett syndrome</term><term>Stereotyped Behavior</term><term>Stereotypies</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Rett Syndrome</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Movement Disorders</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Movement Disorders</term><term>Rett Syndrome</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adolescent</term><term>Adult</term><term>Child</term><term>Child, Preschool</term><term>Female</term><term>Humans</term><term>Infant</term><term>Stereotyped Behavior</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Age</term><term>Diagnostic</term><term>Enfant</term><term>Etude cas</term><term>Exploration clinique</term><term>Extrapyramidal syndrome</term><term>Maladie dégénérative</term><term>Mouvement involontaire</term><term>Retard psychomoteur</term><term>Rett syndrome</term><term>Système nerveux pathologie</term><term>Trouble comportement</term><term>Trouble moteur</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Enfant</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Rett syndrome, a progressive neurodegenerative disorder described only in female subjects, is manifested by a wide spectrum of behavioral and motor abnormalities. We studied 32 patients with this disorder, ages 30 months to 28 years old, and characterized their extrapyramidal disturbance. The most common motor abnormalities were stereotyped movements and gait disturbance, seen in all patients. Bruxism, oculogyric crises, parkinsonism, and dystonia were also common, but myoclonus and choreoathetosis were seen only infrequently. The hyperkinetic movement disorders tended to dominate in younger patients, while bradykinetic disorders were more evident in the older patients. This study provides evidence that movement disorders seen in Rett syndrome reflect age‐related neurodegenerative changes in the basal ganglia.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country><region><li>Texas</li></region></list><tree><country name="États-Unis"><region name="Texas"><name sortKey="Fitzgerald, Patricia M" sort="Fitzgerald, Patricia M" uniqKey="Fitzgerald P" first="Patricia M." last="Fitzgerald">Patricia M. Fitzgerald</name></region><name sortKey="Jankovic" sort="Jankovic" uniqKey="Jankovic" last="Jankovic">Jankovic</name><name sortKey="Percy, Alan K" sort="Percy, Alan K" uniqKey="Percy A" first="Alan K." last="Percy">Alan K. Percy</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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