Biotin-responsive basal ganglia disease: a treatable and reversible neurological disorder of childhood.
Biotin-responsive basal ganglia disease is a rare childhood neurological disorder of uncertain etiology that is treatable if suspected and diagnosed. Only few cases have been reported earlier in literature. We report a case of biotin-responsive basal ganglia disease suspected clinically, corroborated by neuroimaging and a dramatic response to biotin therapy.
Bindu
P S
PS
Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India. drpsbindu@yahoo.co.in
Noone
M L
ML
Nalini
A
A
Muthane
Uday B
UB
Kovoor
Jerry M E
JM
D001480:Basal Ganglia Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology; D001710:Biotin / Q000627:therapeutic use*; D001921:Brain / Q000473:pathology; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D020191:Myoclonic Epilepsies, Progressive / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology; D014803:Vitamin B Complex / Q000627:therapeutic use*
D014803:Vitamin B Complex; D001710:Biotin