Novel use of intrathecal baclofen drug delivery system for periodic focal dystonia in a teenager.

Introduction:  Focal dystonia, often affecting part of a limb, is a manifestation of complex regional pain syndrome (CRPS). This can be difficult to diagnose and treat. Furthermore, there may be significant latency between the onset of dystonia after the diagnosis of CRPS. We present the case of a 15-year-old girl with periodic focal dystonia who has been successfully treated with an intrathecal baclofen pump.

Materials And Methods:  The patient had sustained a minor ankle fracture four years prior to presentation. Despite radiologic evidence of adequate bony union, the patient continued to complain of spasms and pain in her left foot leading to dystonic posturing of the foot. Multiple therapies including subcutaneous morphine infusion, ankle splinting, physiotherapy, and local botulinum injections had not provided adequate relief. Intrathecal baclofen on three separate occasions resulted in successful temporary resolution of the dystonia. A placebo double-blinded injection of intrathecal saline at a separate setting however did not resolve the dystonia.

Results:  We then proceeded with permanent delivery of baclofen by implantation of an intrathecal drug delivery pump, which resulted in resolution of the dystonia. The patient also was able to receive bolus doses of intrathecal baclofen. The patient is now able to partake in sporting and dancing activities. A detailed history of the patient, along with the difficulties in diagnosis and management, is presented.

Conclusion:  Intrathecal baclofen therapy can be effective in the management of focal dystonia after rigorous preoperative testing and counseling of adolescents with CRPS.