Infusion of intrathecal baclofen for generalized dystonia in cerebral palsy

Generalized dystonia occurs in 15 to 25% of persons with cerebral palsy (CP) and responds poorly to medical and surgical treatments. Object. After the authors observed a woman whose dystonic CP was dramatically improved by continuous infusion of intrathecal baclofen; they designed this pilot study to evaluate the effect of this treatment on a group of patients with dystonic CP. Methods. The authors assessed the short-term response to intrathecal baclofen infusion in 12 patients with dystonic CP. An intrathecal catheter was inserted percutaneously and connected to an external microinfusion pump. The infusion began at a rate of 100 mu g/day and was increased by 50 mu g every 12 hours until the dystonia abated, adverse effects occurred, or the dose reached 900 mu g/day with no improvement. Two observers, one blinded and one not blinded to the patient's treatment status, viewed videotapes made before and after the infusions and graded the dystonia in eight body regions, using a 5-point scale. Overall and regional scores were compared by using Wilcoxon signed-rank tests. Conclusions. Dystonia diminished in 10 of 12 patients whose average daily dose of intrathecal baclofen was 575 mu g. Overall dystonia scores and scores for the extremities, trunk, and cervical regions were significantly better after infusion (p = 0.003). The two observers' scores were not significantly different. Programmable infusion pumps were subsequently implanted in eight patients for long-term therapy and improvement was sustained in six (p < 0.05). Intrathecal baclofen infusion is a promising treatment option for generalized dystonia associated with CP. The effects of intrathecal baclofen infusion on dystonia can be evaluated by using short-term continuous infusions.