CONTINUOUS INTRATHECAL BACLOFEN INFUSION FOR SYMPTOMATIC GENERALIZED DYSTONIA

FIVE PATIENTS WITH generalized dystonia who were refractory to oral me dications were treated by continuous intrathecal baclofen infusion. Dy stonia was related to cerebral palsy in three patients and to Hallervo rden-Spatz disease in two. Responsiveness to intrathecally administere d baclofen was evaluated after bolus injections in one patient and dur ing continuous infusions via an external micropump in four. Patients w ho responded to trial injections were subsequently implanted with a pr ogrammable pump for continuous infusion of baclofen. Dystonia in the t hree patients with cerebral palsy was substantially improved by contin uous intrathecal baclofen infusion in doses of 500 to 800 mu g/d. Bene fit has persisted for >19 months of continuous infusion. Dystonia in t he two patients with Hallervorden-Spatz disease was not improved, alth ough the screening trial was limited by side effects in one patient an d by meningitis in the other. We conclude that continuous intrathecal baclofen infusion is beneficial therapy for some patients with general ized dystonia and that additional investigations are indicated.