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Dopa-Responsive Dystonia

Disease ID: disease_node_14347

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DbxrefICD10CM:G24.1, MIM:128230, ORDO:98808
SubclassofDOID_0050736, DOID_543, DOID_0050737
Data SourceDOID
SynonymsAutosomal dominant Segawa syndrome, DOPA-RESPONSIVE DYSTONIA, AUTOSOMAL DOMINANT, DYSTONIA, DOPA-RESPONSIVE, AUTOSOMAL DOMINANT, DYSTONIA-PARKINSONISM WITH DIURNAL FLUCTUATION, GTPCH1-deficient DRD, GTPCH1-deficient dopa-responsive dystonia, HPD with marked diurnal fluctuation, Hereditary progressive dystonia with marked diurnal fluctuation, dystonia 5
Doid Labeldopa-responsive dystonia
Doid DescriptionA dystonia characterized by generalized dystonia, diurnal fluctuation of symptoms, and a dramatic therapeutic response to L-dopa that has_material_basis_in heterozygous mutation in the GCH1 gene on chromosome 14q13.
Has Material Basis InGENO_0000147, GENO_0000148
Disease Node Iddisease_node_14347
Doid IdDOID_0090043
LabelDopa-Responsive Dystonia
Doid Alternate IdsDOID_0060963