Improved gait performance in a patient with hereditary spastic paraplegia after a continuous intrathecal baclofen test infusion and subsequent pump implantation: a case report

Objective: To show the benefits of a continuous intrathecal baclofen (ITB) test infusion in a patient with hereditary spastic paraplegia (HSP), with an improved gait performance after ITB pump implantation.

Design: Case report.

Setting: University hospital.

Participant: A 49-year old man with HSP experiencing progressive walking difficulties because of lower extremity spasticity, which did not respond to oral spasmolytics.

Interventions: A prolonged, continuous ITB test infusion was started at a low dose and increased gradually, to provide a stable dose of ITB over a prolonged period. The gradual dose increase provided the patient enough time to experience the effects of ITB, because he feared that ITB therapy might cause functional loss.

Main outcome measures: Modified Ashworth Scale, electromyography, muscle strength, timed Up and Go tests, and the Patient Global Impression of Change. Gait performance before and after ITB pump implantation was assessed in a motion laboratory.

Results: During the test infusion, the ITB dose was gradually increased to a continuous dose of 108μg/d. This dose caused the spasticity to decrease, with maintenance of muscle strength. After pump implantation, gait performance was improved, resulting in increased knee flexion during the loading response and a doubled walking speed as compared with baseline.

Conclusions: Patients with HSP who have mild spasticity that does not respond to oral spasmolytics should receive a continuous ITB test infusion, to provide them with enough time to experience the delicate balance between spasmolysis and muscle strength. ITB administration is a suitable therapy to improve gait performance in patients with HSP.