Intraventricular baclofen infusion for dystonia. Report of two cases.

Two children with generalized dystonia were given continuous intraventricular baclofen (IVB) infusions. The first was a 12-year-old girl with mixed dystonia and spasticity caused by methylmalonic aciduria whose spinal anatomy precluded administration of intrathecal baclofen (ITB). The second was an 11-year-old boy whose dystonia was associated with cerebral palsy and had not improved significantly in response to conventional ITB treatment. The girl improved dramatically with IVB; the boy experienced no greater improvement from IVB than from ITB. No adverse effects were noted in either child during 2 to 6 months of infusion. These are the first reports of the use of IVB in this clinical setting. The results warrant additional investigation.