Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare genetic neurological disorder characterized by subacute encephalopathy with confusion, seizures, and movement disorder, often following a history of febrile illness. Imaging may reveal bilateral lesions in the basal ganglia. The disease usually becomes symptomatic in childhood and is life-threatening if left untreated, but symptoms can be reversed and progression prevented by treatment with high doses of biotin and thiamine. Disease data Classification Disease Synonyms BBGD BBGD BTBGD Choroba zwojów podstawnych wrażliwa na biotynę-tiaminę BTBGD Biotin-responsive basal ganglia disease ORPHA code 65284 OMIM code 607483 ICD10 code G93.8 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl