Autosomal recessive dopa-responsive dystonia (DYT5b) is a very rare neurometabolic disorder characterized by a spectrum of symptoms ranging from those seen in dopa-responsive dystonia (DRD; see this term) to progressive infantile encephalopathy. The disease is Orphanet_101150 (Autosomal recessive dopa-responsive dystonia). Also known as: Autosomal recessive Segawa syndrome, DYT5b, Tyrosine hydroxylase deficiency, Tyrosine hydroxylase-deficient dopa-responsive dystonia.