Anzeige
Erschienen in:
02.06.2018 | Case Report
Intrathecal baclofen in mucopolysaccharidosis type II (Hunter syndrome): case report
Erschienen in: Child's Nervous System | Ausgabe 11/2018
Einloggen, um Zugang zu erhaltenAbstract
Purpose
Intrathecal baclofen administration is commonly used in the treatment of children’s spasticity. In general, candidates for baclofen pump are patients with spastic form of cerebral palsy. Intrathecal baclofen in the treatment of spasticity due to a metabolic disorder is rarely reported.
Methods
Authors report on an 11-year-old boy with mucopolysaccharidosis type II (Hunter syndrome) with progressive stiffness and contractures followed by profound loss of joint movement range and tiptoe walking pattern. Patient was indicated for baclofen test with subsequent pump insertion and continuous intrathecal baclofen administration.
Results
Postoperatively, patient was gradually set to current baclofen dose of 250 μg/day. At mentioned dose, we observed not only increased active and passive range of movements and facilitation in fine motor skills, but also better walking pattern.
Conclusions
Despite intrathecal baclofen administration in patients with spasticity related to mucopolysaccharidosis type II is not widely reported, we consider it as feasible treatment. To emphasize, enzyme replacement therapy is the primary treatment, and improvement is attributed to both enzyme substitution and intrathecal baclofen therapy.