Kennedy Krieger Institute, Baltimore, Maryland, USA.
Am J Phys Med Rehabil. 2010 Apr;89(4):323-30. doi: 10.1097/PHM.0b013e3181ca23a8.
Sturge-Weber syndrome is a rare neurocutaneous disorder that often results in functional impairment caused by motor (typically hemiparesis) and cognitive deficits. A retrospective chart review of physiatric evaluation of 30 individuals, aged 4 mos to 55 yrs (median age, 2.4 yrs), with Sturge-Weber syndrome with brain involvement was conducted for the purpose of summarizing physiatric findings and recommendations in this cohort. Presence or absence of motor, cognitive, and behavioral concerns and need for orthoses, spasticity management, and therapy services were noted. Hemiparesis was common, but the need for intervention for spasticity was rare. Cognitive and behavioral concerns were noted frequently, meriting additional evaluation. Case vignettes are presented to highlight (1) a child with repeated functional setbacks in association with increased seizure frequency who, with seizure control, demonstrated return to functional baseline and subsequent further skill development and (2) a child with Sturge-Weber syndrome who made functional gains with constraint-induced movement therapy.
斯特奇-韦伯综合征是一种罕见的神经皮肤疾病,常导致运动(通常为偏瘫)和认知缺陷引起的功能障碍。对 30 名年龄在 4 个月至 55 岁(中位年龄 2.4 岁)、伴有脑受累的斯特奇-韦伯综合征患者进行了回顾性图表评估,以总结该队列中的物理治疗发现和建议。注意有无运动、认知和行为问题以及是否需要矫形器、痉挛管理和治疗服务。偏瘫很常见,但痉挛需要干预的情况很少。经常注意到认知和行为问题,需要进一步评估。提供病例示例以突出(1)一个孩子因癫痫发作频率增加而反复出现功能减退,在控制癫痫后,其功能恢复到基线水平,并随后进一步发展技能,和(2)一个接受强制性运动疗法的斯特奇-韦伯综合征患儿取得了功能改善。