title: Continuous intrathecal baclofen infusion for symptomatic generalized dystonia.
abstract: Five patients with generalized dystonia who were refractory to oral medications were treated by continuous intrathecal baclofen infusion. Dystonia was related to cerebral palsy in three patients and to Hallervorden-Spatz disease in two. Responsiveness to intrathecally administered baclofen was evaluated after bolus injections in one patient and during continuous infusions via an external micropump in four. Patients who responded to trial injections were subsequently implanted with a programmable pump for continuous infusion of baclofen. Dystonia in the three patients were cerebral palsy was substantially improved by continuous intrathecal baclofen infusion in doses of 500 to 800 micrograms/d. Benefit has persisted for > 19 months of continuous infusion. Dystonia in the two patients with Hallervorden-Spatz disease was not improved, although the screening trial was limited by side effects in one patient and by meningitis in the other. We conclude that continuous intrathecal baclofen infusion is beneficial therapy for some patients with generalized dystonia and that additional investigations are indicated.
authors.affiliation: Department of Neurosurgery, Children's Hospital of Pittsburgh, Pennsylvania, USA.
authors.forename: A L
authors.initials: AL
authors.lastname: Albright
authors.forename: M J
authors.initials: MJ
authors.lastname: Barry
authors.forename: P
authors.initials: P
authors.lastname: Fasick
authors.forename: W
authors.initials: W
authors.lastname: Barron
authors.forename: B
authors.initials: B
authors.lastname: Shultz
mesh_terms: D000293:Adolescent; D000328:Adult; D001418:Baclofen / Q000008:administration & dosage* / Q000009:adverse effects; D002547:Cerebral Palsy / Q000175:diagnosis / Q000188:drug therapy*; D002648:Child; D004305:Dose-Response Relationship, Drug; D004421:Dystonia / Q000175:diagnosis / Q000188:drug therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015918:Infusion Pumps, Implantable; D007278:Injections, Spinal; D008297:Male; D009125:Muscle Relaxants, Central / Q000008:administration & dosage* / Q000009:adverse effects; D009460:Neurologic Examination / Q000187:drug effects; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000175:diagnosis / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D009125:Muscle Relaxants, Central; D001418:Baclofen