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Intrathecal baclofen for spasticity management in Rett syndrome.
Vivek Kadyan1, Albert C Clairmont, Rebecca J George
1Department of Physical Medicine and Rehabilitation, The Ohio State University Medical Center, Columbus, USA.
American Journal of Physical Medicine & Rehabilitation
|June 24, 2003
Summary
Intrathecal baclofen infusions effectively manage severe spasticity in Rett syndrome patients. This treatment improved limb function, mobility, and overall quality of life in a case study.
Area of Science:
- Neurology
- Neuroscience
- Pharmacology
Background:
- Intrathecal baclofen infusions are established for managing spasticity, particularly of spinal origin.
- Rett syndrome, a neurodevelopmental disorder, can cause significant spasticity and functional decline in later stages.
- The efficacy of intrathecal baclofen for non-spinal spasticity is promising.
Observation:
- A case report details a 32-year-old woman with Rett syndrome and severe spasticity.
- She received an intrathecal baclofen pump, with dosage gradually increased to 800 microg/day over one year.
- Significant clinical improvement was observed during the treatment period.
Findings:
- The patient experienced a notable reduction in upper and lower limb spasticity, with Ashworth scores decreasing from an average of 3-4 to 2-3.
- This reduction in spasticity led to enhanced range of motion and improved positioning.
- Improvements in skin care, hygiene, and overall quality of life were also documented.
Implications:
- Intrathecal baclofen is a viable and effective treatment option for severe spasticity associated with Rett syndrome.
- This approach can significantly improve functional outcomes and patient well-being.
- Further research may explore broader applications of intrathecal baclofen in complex neurodevelopmental disorders.