Chu M L, Sala D A, Weiner H L
The Center for Children, Hospital for Joint Diseases and New York University Hospitals Center, New York, NY 10003, USA.
Pediatr Neurol. 2001 Feb;24(2):156-8. doi: 10.1016/s0887-8994(00)00250-2.
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.
X连锁肾上腺脑白质营养不良是一种进行性神经退行性疾病，涉及大脑白质破坏和肾上腺皮质激素缺乏。临床症状最早出现在4至8岁之间，包括痉挛、视力丧失、吞咽困难和癫痫发作。在本报告中，鞘内注射巴氯芬持续输注用于治疗一名8岁X连锁肾上腺脑白质营养不良患者的严重痉挛。该患者生活质量的改善，包括疼痛消除和护理便利性增加，表明鞘内注射巴氯芬应被视为儿童和成人X连锁肾上腺脑白质营养不良及其他神经退行性疾病相关痉挛治疗策略的一部分。
