Intrathecal baclofen in X-linked adrenoleukodystrophy.
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.
The Center for Children, Hospital for Joint Diseases and New York University Hospitals Center, New York, NY 10003, USA.
M L
ML
Chu
D A
DA
Sala
H L
HL
Weiner
D000326:Adrenoleukodystrophy / Q000188:drug therapy*; D001418:Baclofen / Q000008:administration & dosage*; D002648:Child; D018718:Home Infusion Therapy; D006801:Humans; D015918:Infusion Pumps, Implantable; D007278:Injections, Spinal; D008297:Male; D009125:Muscle Relaxants, Central / Q000008:administration & dosage*; D016896:Treatment Outcome
D009125:Muscle Relaxants, Central; D001418:Baclofen