X-linked adrenoleukodystrophy is a progressive neurodegenerative disorder i
nvolving the destruction of white matter in the brain and adrenocortical ho
rmone deficiency. Clinical symptoms first appear between 4 and 8 years of a
ge and include spasticity, visual loss, dysphagia, and seizures. In this re
port, continuous infusion of intrathecal baclofen was used to treat the sev
ere spasticity of an 8-year-old patient with X-linked adrenoleukodystrophy.
The improvement in this patient's quality of life, including the eliminati
on of pain and the increased ease of care, suggests that intrathecal baclof
en should be considered as part of the treatment strategy for spasticity as
sociated with X-linked adrenoleukodystrophy and other neurodegenerative dis
orders in children and adults. (C) 2001 by Elsevier Science Inc. All rights
reserved.