Abstract

ObjectiveTo 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. DesignCase report. SettingUniversity hospital. ParticipantA 49-year old man with HSP experiencing progressive walking difficulties because of lower extremity spasticity, which did not respond to oral spasmolytics. InterventionsA 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 MeasuresModified 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. ResultsDuring 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. ConclusionsPatients 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.

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