ITB for Generalized Dystonia: 86 Cases

Subject: ITB for Generalized Dystonia: 86 Cases

Date: 3/8/2002

Intrathecal baclofen for generalized dystonia
AL Albright, MJ Barry, DH Shafron, SF Ferson
Developmental Med Child Neurol 2001;43:652-657

Intrathecal baclofen is an effective treatment for generalized dystonia, according to this report.

Eighty-six patients ages 3-42 (mean 13 years) with generalized dystonia were offered ITB treatment. Dystonia was secondary to cerebral palsy in 71% of patients. External infusion or bolus-dose screening was positive in approximately 90% of patients. Programmable pumps were implanted in 77 patients. Infusion began at 200 micrograms per day, and increased by 10-20% per day until the best dose was achieved. Median duration ITB therapy was 26 months. Mean dose increased over time, from 395 micrograms at 3 months, to 610 at 24 months, to 960 at 36 months.

Mean Barry-Albright dystonia scores decreased over time, from 18 at baseline to 7 at 12 months, but rose again to 10 at 24 months (all decreases versus baseline were highly significant). Quality of life and ease of care were rated by patients and caregiver as improved in approximately 85% of patients. Speech and swallowing were improved in slightly less than one third, and unchanged in two thirds. Function in upper and lower extremities was improved in one third, and unchanged in slightly less than two thirds. Approximately 5% of patients reported worsening in each category.

Seven patients, including 4 with CP, lost their response to ITB during the study, usually during the first year. The most common side effects were increased constipation (19%), decreased neck/trunk control, and drowsiness. Surgical and device complications occurred in 38% of patients, including infections and catheter breakage and disconnection. Complication rates decreased over time.

The authors conclude, "In our opinion, ITB is the treatment of choice for severe, generalized secondary dystonia after oral medications have been shown to be ineffective."