Autosomal dominant dopa-responsive dystonia

Orpha code: 98808OMIM code: 619911

Definition

A rare neurometabolic disorder characterized by childhood-onset dystonia that shows a dramatic and sustained response to low doses of levodopa (L-dopa) and that may be associated with parkinsonism at an older age.

Disease data
Classification

Disease

Synonyms
Autosomal dominant Segawa syndrome
Autosomalny dominujący zespół Segawa
DRD z deficytem GTPCH1
Dystonia reagująca na lewodopę z deficytem GTPCH1
DYT5a
DYT5a
GTPCH1-deficient DRD
GTPCH1-deficient dopa-responsive dystonia
HPD with marked diurnal fluctuation
Hereditary progressive dystonia with marked diurnal fluctuation
ORPHA code
98808
OMIM code
619911
ICD10 code
G24.1
ICD11 code
-

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl