Intrathecal baclofen for spasticity management in Rett syndrome.

Abstract

Intrathecal baclofen infusions have proven to be effective for management of spasticity during the last two decades. Efficacy of intrathecal baclofen for spasticity of spinal origin has been well established and has shown promise in treatment of spasticity that is not spinal in origin. Rett syndrome is a neurodevelopmental disorder primarily affecting girls and women. Manifested in the advanced stages of this syndrome is increased spasticity leading to functional decline. Presented is a case report of a 32-yr-old white woman with Rett syndrome, diagnosed before the age of 2 yr, and significant spasticity that was successfully managed with intrathecal baclofen. After placement of an intrathecal baclofen pump, the dose was increased slowly during 1 yr to 800 microg/day with good clinical response. There was observed a significant decrease in upper and lower limb Ashworth scores, from an average of 3-4 to 2-3, during this 1-yr period. The decrease in spasticity in this patient eventually led to improved range of motion, positioning, skin care, hygiene, and quality of life. Intrathecal baclofen is an effective option in managing severe spasticity from Rett syndrome.