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Child's Nervous System

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01-11-2018 | Case Report

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

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

Published in: Child's Nervous System | Issue 11/2018

Abstract

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.

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Title: Intrathecal baclofen in mucopolysaccharidosis type II (Hunter syndrome): case report
Authors: František Horn
Michal Petrík
Dana Dúbravová
Jarmila Hornová
Katrína Brennerová
Vladimír Bzduch
Publication date: 01-11-2018
Publisher: Springer Berlin Heidelberg
Published in: Child's Nervous System / Issue 11/2018
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-018-3857-z

At a glance: The STEP trials

Springer Medicine

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

Abstract

Purpose

Methods

Results

Conclusions

At a glance: The STEP trials

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusions

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