Presented at the 2013 AAPM Annual Meeting « Back

The Use of Intrathecal Baclofen in the Treatment of Stiff-Person Syndrome

David M. DeChellis, DO, dr.ddechellis@live.com¹, Abraham (Jack) Kabazie, MD¹, akabazie@wpahs.org, Matthew Boeckman, MD¹, (1) West Penn Allegheny Health System, Pittsburgh, Pennsylvania

Introduction: A 48-year-old female with history of Stiff-Person Syndrome (SPS) presented with chronic low back and bilateral leg pains, associated with frequent muscle spasms. She also had progressively worsening function in terms of transfers and ambulation. The patient had improvements of her symptoms with oral diazepam and baclofen, however was unable to continue these medications secondary to cognitive side effects. The patient has referred to our Pain Management clinic for an intrathecal baclofen injection trial. SPS is a rare neurological disorder which often presents with progressive stiffness and spasms in the thoracolumbar and lower limb musculature. The disorder is hypothesized to be mediated by antibodies that limit the synthesis of gamma-aminobutyric acid (GABA). Detection of anti-glutamic acid decarboxylase antibodies and electrodiagnostic studies are used to confirm the disorder. Treatment of the symptoms typically involves GABA-enhancing medications, steroids, and immunoglobulins. There has been literature to suggest that intrathecal baclofen may have a role in treatment as well. Methods: An intrathecal test dose of 50 micrograms of Baclofen was injected into the patient. Results: The patient had significant subjective improvements in pain and muscle spasms of the lower back and bilateral legs at 1 hour, 2 hours, and 4 hours post injection. The patient was also observed to have improvement in her ability to transfer and ambulate. Conclusion: Intrathecal baclofen may provide benefit in terms of improvement in pain, spasms, and function in Stiff-Person Syndrome patients unable to tolerate other forms of treatment. References: 1) Murinson B, Guarnaccia J. Stiff-person syndrome with amphiphysin antibodies: Distinctive features of a rare disease. Neurology. 2008 December 9; 71(24): 1955-8. 2) Baker MR, Das M, Isaacs J et al. Treatment of stiff person syndrome with rituximab. Journal of Neurology, Neurosurgery, & Psychiatry with Practical Neurology. 2005 Jul;76(7):999-1001 3) Maramattom B. Intrathecal baclofen pump implantation in a case of stiff person syndrome. Neurology India 2010 Jan-Feb;58(1):115-7.

Funding: None