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.

Complications of Intrathecal Baclofen Pumps in Children: Experience from a Tertiary Care Center

“…This procedure is also helpful in the management of medically refractory secondary dystonias [7,8]. The cost-effectiveness of this procedure in the treatment of spastic cerebral palsy has been proven in 2007 by the elegant study by De Lissovoy et al [9] .…”

Evidence of the effects of intrathecal baclofen for spastic and dystonic cerebral palsy

“…Credibility is further limited by low ratings of the internal validity of the nine outcomes: seven Level V results (primarily Annotation 643 31 Case report V 6 h 1 0 yes Albright 32 Case report V 10 mo 5 0 yes Albright 29 Case series Phase 1. anecdotal) and two Level IV. Only three outcomes were measured and only two of these were statistically evaluated.…”

Bilateral Subthalamic Nucleus Stimulation in the Treatment of Neurodegeneration with Brain Iron Accumulation Type 1

“…Medications, including dopaminergic agents for dystonia and tremor and diazepam for choreoathetoid movement, only provide little or transient benefit. Results of intrathecal baclofen infusion were frustrating [1] . There have been sporadic reports of stereotactic ablation surgery for NBIA1 [2][3][4][5] .…”