Succinic semialdehyde dehydrogenase deficiency

Orpha code: 22OMIM code: 271980

Definition

A rare neurometabolic disorder of gamma-aminobutyric acid (GABA) metabolism with a nonspecific clinical presentation (ranging from mild to severe) with the most frequent symptoms being cognitive impairment with prominent deficit in expressive language, hypotonia, ataxia, epilepsy, and behavioral dysregulation.

Disease data
Classification

Disease

Synonyms
4-hydroxybutyric aciduria
Niedobór dehydrogenazy semialdehydu bursztynowego
Gamma-hydroxybutyric aciduria
SSADH deficiency
ORPHA code
22
OMIM code
271980
ICD10 code
E72.8
ICD11 code
5C50.E1

No additional description.

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