Pediatria pre prax 2/2018
Doplňujúca intratekálna liečba spasticity baklofenom u detí s metabolickým ochorením
Supplemental intrathecal treatment with baclofen for spasticity in children with metabolic diseases The intrathecal application of baclofen with programmable pump is well established method in treatment of childhood spasticity. Baclofen pump is widely used in spastic form of cerebral palsy. The authors of the article report on patient with type II mucopolysaccharidosis, with progressive stiffness and contractures. The patient was indicated for a baclofen test followed by implantation of the pump. We noticed not only an increase in active and passive movement and facilitating fine motor skills, but also an improved gait pattern. Intrathecal treatment of baclofen in children with type II mucopolysaccharidosis as well as other metabolic diseases is a rarely published method. In the well-indicated patients with this disease it is possible to increase the range of movement, improve the gait and increase the quality of life of children and their families. Enzyme replacement therapy remains the primary treatment for children with mucopolysaccharidosis II type. In our case, the improvement of the children condition is attributed to the combination of a long-term substitution enzyme therapy and the treatment with intrathecal baclofen.
Keywords: baclofen, pump, intrathecal, spasticity, Hunter syndrome, mucopolysaccharidosis, surgical treatment
