Literature DB >> 11275468

Intrathecal baclofen in X-linked adrenoleukodystrophy.

M L Chu1, D A Sala, H L Weiner.   

Abstract

X-linked adrenoleukodystrophy is a progressive neurodegenerative disorder involving the destruction of white matter in the brain and adrenocortical hormone deficiency. Clinical symptoms first appear between 4 and 8 years of age and include spasticity, visual loss, dysphagia, and seizures. In this report, continuous infusion of intrathecal baclofen was used to treat the severe spasticity of an 8-year-old patient with X-linked adrenoleukodystrophy. The improvement in this patient's quality of life, including the elimination of pain and the increased ease of care, suggests that intrathecal baclofen should be considered as part of the treatment strategy for spasticity associated with X-linked adrenoleukodystrophy and other neurodegenerative disorders in children and adults.

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Year:  2001        PMID: 11275468     DOI: 10.1016/s0887-8994(00)00250-2

Source DB:  PubMed          Journal:  Pediatr Neurol        ISSN: 0887-8994            Impact factor:   3.372


  5 in total

Review 1.  Emergencies in motoneuron disease.

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Journal:  Intern Emerg Med       Date:  2017-03-09       Impact factor: 3.397

2.  Intrathecal baclofen therapy after liver transplant in a patient with Crigler-Najjar syndrome.

Authors:  Napala R Pratini; Jacob A Neufeld
Journal:  PM R       Date:  2013-12-21       Impact factor: 2.298

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

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Journal:  Childs Nerv Syst       Date:  2018-06-02       Impact factor: 1.475

Review 4.  ABCD1 Gene Mutations: Mechanisms and Management of Adrenomyeloneuropathy.

Authors:  Alyssa M Volmrich; Lauren M Cuénant; Irman Forghani; Sharon L Hsieh; Lauren T Shapiro
Journal:  Appl Clin Genet       Date:  2022-08-12

5.  Modeling and rescue of defective blood-brain barrier function of induced brain microvascular endothelial cells from childhood cerebral adrenoleukodystrophy patients.

Authors:  Catherine A A Lee; Hannah S Seo; Anibal G Armien; Frank S Bates; Jakub Tolar; Samira M Azarin
Journal:  Fluids Barriers CNS       Date:  2018-04-04
  5 in total

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