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Journal of Child Neurology
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*BIOTIN
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Biotin-Responsive Basal Ganglia Disease: A Treatable and Reversible Neurological Disorder of Childhood

P.S. Bindu, DM

Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India, drpsbindu{at}yahoo.co.in

M.L. Noone, DM

Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India

A. Nalini, DM

Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India

Uday B. Muthane, DM

Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India

Jerry M. E. Kovoor, MD

Department of Neuroimaging & Interventional Radiology, National Institute of Mental Health and Neurosciences, Bangalore, India

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.

Key Words: biotin • basal ganglia • encephalopathy

Journal of Child Neurology, Vol. 24, No. 6, 750-752 (2009)
DOI: 10.1177/0883073808329525
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