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

Intrathecal baclofen in mucopolysaccharidosis type II (Hunter syndrome): case report 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. . . . Keywords Intrathecal baclofen Mucopolysaccharidosis Hunter syndrome Spasticity Introduction sulfatase, leading to an accumulation of heparan sulfate and dermatan sulfate, and thus, its absence results in a http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Child's Nervous System Springer Journals

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

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Publisher
Springer Journals
Copyright
Copyright © 2018 by Springer-Verlag GmbH Germany, part of Springer Nature
Subject
Medicine & Public Health; Neurosurgery; Neurosciences
ISSN
0256-7040
eISSN
1433-0350
D.O.I.
10.1007/s00381-018-3857-z
Publisher site
See Article on Publisher Site

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. . . . Keywords Intrathecal baclofen Mucopolysaccharidosis Hunter syndrome Spasticity Introduction sulfatase, leading to an accumulation of heparan sulfate and dermatan sulfate, and thus, its absence results in a

Journal

Child's Nervous SystemSpringer Journals

Published: Jun 2, 2018

References

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