ParkinsonCanadaV1, Main, Exploration, bibRecord, 004303

Dopa-responsive dystonia : pathological and biochemical observations in a case

Identifieur interne : 004303 ( Main/Exploration ); précédent : 004302; suivant : 004304

Dopa-responsive dystonia : pathological and biochemical observations in a case

Auteurs : A. H. Rajput [Canada] ; W. R. G. Gibb ; X. H. Zhong ; K. S. Shannak ; S. Kish ; L. G. Chang ; O. Hornykiewicz

Source :

Annals of neurology [ 0364-5134 ] ; 1994.

RBID : Pascal:94-0376818

Descripteurs français

Pascal (Inist)
- Dystonie, Chimiothérapie, Efficacité traitement, Lévodopa, Anatomopathologie, Exploration, Encéphale, Analyse biochimique, Homme, Etude cas, Dopamine, Voie nigrostriatale.
Wicri :
- topic : Homme.

English descriptors

KwdEn :
- Biochemical analysis, Brain (vertebrata), Case study, Chemotherapy, Dopamine, Dystonia, Exploration, Human, Levodopa, Nigrostriatal pathway, Pathology, Treatment efficiency.

Abstract

We report the first neuropathological and neurochemical study of a patient with dopa-responsive dystonia. She had onset of foot dystonia at age 5 years and by age 8 years it was generalized with prominent right leg and arm involvement. On levodopa 750 mg daily she had complete symptomatic improvement that was sustained for 11 years until death. Pathological studies revealed normal numbers of hypopigmented substantia nigra neurons, normal tyrosine hydroxylase (TH) immunoreactivity and TH protein in the SN, no inclusion bodies or gliosis, and no evidence of a degenerative process in the striatum. Biochemical studies revealed reduced dopamine in the substantia nigra and striatum (8% in the putamen and 18% of control in the caudate) with a similar but not identical subregional distribution as in idiopathic Parkinson's disease

Affiliations:

Canada

Links toward previous steps (curation, corpus...)

to stream PascalFrancis, to step Corpus: 001019
to stream PascalFrancis, to step Curation: 000F06
to stream PascalFrancis, to step Checkpoint: 000F12
to stream Main, to step Merge: 004A74
to stream Main, to step Curation: 004303

Le document en format XML

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<term>Dystonia</term>
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<front><div type="abstract" xml:lang="en">We report the first neuropathological and neurochemical study of a patient with dopa-responsive dystonia. She had onset of foot dystonia at age 5 years and by age 8 years it was generalized with prominent right leg and arm involvement. On levodopa 750 mg daily she had complete symptomatic improvement that was sustained for 11 years until death. Pathological studies revealed normal numbers of hypopigmented substantia nigra neurons, normal tyrosine hydroxylase (TH) immunoreactivity and TH protein in the SN, no inclusion bodies or gliosis, and no evidence of a degenerative process in the striatum. Biochemical studies revealed reduced dopamine in the substantia nigra and striatum (8% in the putamen and 18% of control in the caudate) with a similar but not identical subregional distribution as in idiopathic Parkinson's disease</div>
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La maladie de Parkinson au Canada (serveur d'exploration)

Dopa-responsive dystonia : pathological and biochemical observations in a case

Dopa-responsive dystonia : pathological and biochemical observations in a case

Source :

Descripteurs français

English descriptors

Abstract

Links toward previous steps (curation, corpus...)

Le document en format XML

Pour manipuler ce document sous Unix (Dilib)

Pour mettre un lien sur cette page dans le réseau Wicri