From the Research
Intrathecal baclofen (ITB) is the most effective treatment for managing severe spasticity in patients with hereditary spastic paraparesis (HSP), as it has been shown to improve spasticity and stabilize mobility functions for up to 6-8 years. Hereditary spastic paraparesis is a group of inherited neurological diseases characterized by progressive stiffness and weakness in the lower limbs, with a wide genetic heterogeneity 1. The condition typically presents with symptoms including increased muscle tone (spasticity), weakness in the legs, abnormal gait, and urinary problems. Treatment is supportive and multidisciplinary, focusing on physical therapy to maintain muscle strength and range of motion, medications like baclofen or tizanidine to reduce spasticity, and assistive devices to aid mobility.
Some key points to consider in the management of HSP include:
- The use of ITB has been shown to be effective in reducing spasticity and improving quality of life in patients with HSP, with a mean baclofen dosage of 188 ± 60 μg per day at steady state post-implantation 2.
- ITB therapy may initially improve spasticity and stabilize mobility functions for the first 6-8 years in patients with HSP, with no ITB-related severe side effects occurring 2.
- Other treatment options, such as botulinum toxin injection, may also be effective in reducing spasticity and improving gait velocity in patients with HSP, although the evidence is less robust 3.
- Genetic counseling is essential for affected families due to the genetic heterogeneity of HSP, with novel approaches to provide etiological treatment being developed for certain genetic subgroups 1.
Overall, the management of HSP requires a comprehensive and multidisciplinary approach, with a focus on reducing spasticity, improving mobility, and enhancing quality of life. The use of ITB is a key component of this approach, and should be considered in patients with severe spasticity who have not responded to other treatments 4.