Outcomes of deep brain stimulation surgery in the management of dystonia in glutaric aciduria type 1.

Abstract

Objectives: Glutaric aciduria type 1 (GA1) is a rare autosomal recessive organic acidaemia caused by deficiency of the glutaryl-CoA dehydrogenase enzyme. We describe the outcomes following deep brain stimulation (DBS) for the management of dystonia of children and adults with glutaric aciduria type 1 (GA1).