Successful Treatment of Stiff Person Syndrome with Intrathecal Baclofen.

Abstract

Intrathecal baclofen therapy is a recognized treatment for severe spasticity. We report here a case of stiff person syndrome in Australia, treated with intrathecal baclofen followed by a rehabilitation programme with substantial clinical and functional improvements. A 59-year-old woman diagnosed with stiff person syndrome had become hoist-dependent and required full care due to severe spasticity over the past 12 years. Treatment with oral benzodiazepines and botulinum toxin injections to the affected muscles had no therapeutic response. After a test dose of 100 pg intrathecal baclofen resulted in a substantial improvement in her physical function, a decision was made to insert an intrathecal baclofen delivery device. This case report supports the use of intrathecal baclofen therapy and a formal inpatient rehabilitation programme for spasticity related to stiff person syndrome. LAY ABSTRACT Stiff person syndrome, a rare autoimmune and neurological disease that leads to significant disability, can be treated by the use of a device delivering baclofen into the spine combined with physical rehabilitation. A 59-year-old woman was diagnosed with stiff person syndrome and had become dependent, with full-time care, due to severe spasms. Conventional treatment with oral muscle relaxants and botulinum toxin injections to her muscles did not have an adequate effect. A trial dose of baclofen was administered into her spine, followed by implantation of a drug delivery device. This resulted in a substantial improvement in function.