Presented at the 2013 AAPM Annual Meeting « Back

Intrathecal Baclofen Therapy for Refractory Stiff-Person Syndrome: A Case Report

Paul Sloan, MD, paulsloan1956@yahoo.com¹, J. Camp Newton, MD², (1) University of Kentucky, Lexington, Kentucky, (2) Columbia Orthopedic Group, Columbia, Missouri

Introduction: Stiff-person syndrome (SPS) is a rare disorder of the CNS resulting in stiffness and spasms of axial and proximal extremity muscles, and muscle pain, but without other neurological signs. The mainstay of symptom relief is oral GABA agonists such as benzodiazepines and baclofen. In extreme and refractory cases, intrathecal baclofen has been suggested as a treatment option. Aim: We report a patient with severe SPS, refractory to oral standard therapies, who was effectively treated with implanted intrathecal baclofen pump. Methods and Results: A 48-year-old male with severe SPS presented for treatment. In spite of oral therapies, he was not able to work as a carpenter. Physical exam revealed jaw tightness and upper and lower extremity increased muscle tone making passive movements difficult. An IT trial of baclofen was initiated at the T1 level, starting dose of 2 mcg/hr. Over the course of several hospital days, the IT baclofen dose was increased to 6 mcg/hr with much improvement in symptoms. The patient received a permanent IT pump for continued baclofen therapy and was able to return to work. Conclusions: 1) Intrathecal baclofen is a safe and effective therapy for the management of spasticity associated with SPS. 2) IT trialing of baclofen, as opposed to single injection technique, is advocated to determine the effective baclofen dose and ensure successful therapy post-trialing. References: 1) Murinson BB. The Neurologist 2004; 10:131-137. 2) Silbert PL. Neurology 1995; 45:1893-1897. 3) Harned ME. Pain Physician 2011; 14:483-489.

Funding: None

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