Metaanalysis of chronic pallidal stimulation in children with DYT-1 dystonia

Introduction: DYT1-dystonia is an autosomal-dominant disease with a penetrance rate of 30–40% and usually starts in childhood between the age 8–12 severely disabling some patients over time. Medical therapy with anti-cholinergic drugs or dopamine is often ineffective and may produce severe side effects. Recent reports suggest that chronic bilateral pallidal stimulation leads to major improvement in these patients.