Details

Autor(en) / Beteiligte

• Horn, František
• Petrík, Michal
• Dúbravová, Dana
• Hornová, Jarmila
• Brennerová, Katrína
• Bzduch, Vladimír

Titel

Intrathecal baclofen in mucopolysaccharidosis type II (Hunter syndrome): case report

Ist Teil von

• Child's nervous system, 2018-11, Vol.34 (11), p.2325-2327

Ort / Verlag

Berlin/Heidelberg: Springer Berlin Heidelberg

Erscheinungsjahr

2018

Quelle

Alma/SFX Local Collection

Beschreibungen/Notizen

• 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.