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

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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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Authors and Affiliations

  1. Department of Pediatric Surgery, Faculty of Medicine, Comenius University, National Institute of Children Diseases, Limbova 2643/1, 83340, Bratislava, Slovak Republic

    František Horn, Michal Petrík & Dana Dúbravová

  2. Department of Pediatrics, Faculty of Medicine, Comenius University, National Institute of Children Diseases, Bratislava, Slovak Republic

    Jarmila Hornová, Katrína Brennerová & Vladimír Bzduch

Authors
  1. František Horn
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  2. Michal Petrík
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  3. Dana Dúbravová
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  4. Jarmila Hornová
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  5. Katrína Brennerová
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  6. Vladimír Bzduch
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Corresponding author

Correspondence to František Horn.

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Informed consent was obtained from all individual participants included in the study.

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The authors declare that they have no conflict of interest.

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Horn, F., Petrík, M., Dúbravová, D. et al. Intrathecal baclofen in mucopolysaccharidosis type II (Hunter syndrome): case report. Childs Nerv Syst 34, 2325–2327 (2018). https://doi.org/10.1007/s00381-018-3857-z

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  • DOI: https://doi.org/10.1007/s00381-018-3857-z

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