School of Medical Sciences, Edith Cowan University, Perth, Australia.
Eur J Neurol. 2013 Sep;20(9):1325-9. doi: 10.1111/ene.12053. Epub 2012 Dec 7.
Despite advances in the understanding of Huntington's disease (HD), treatment remains symptomatic. Multidisciplinary rehabilitation, however, appears to impact disease progression. Here we show the feasibility, safety and efficacy of a 9-month multidisciplinary rehabilitation programme in a small cohort of patients with early-to-middle-stage HD.
Twenty patients with HD were assigned to two groups, equally matched for cognitive and motor scores. One group received the intervention, whilst the other served as control. The Unified-Huntington's-Disease-Rating-Scale-Total-Motor-Score was the primary outcome measure. Neurocognitive/psychological tests, body composition, postural stability, strength and quality of life assessments were secondary outcome measures.
The intervention reduced motor and postural stability deterioration, with minor improvements in depression, cognition and quality of life. Significant gains were observed for fat-free mass and strength.
This pilot study suggests that a prolonged multidisciplinary rehabilitation programme in early-to-middle-stage HD is feasible, well-tolerated and associated with therapeutic benefit. Further explorative, larger studies are warranted.
尽管亨廷顿病(HD)的发病机制得到了深入的研究,但目前仍缺乏有效的治疗手段。然而,多学科康复治疗似乎可以影响疾病的进展。本研究旨在探讨小样本量的早、中期 HD 患者接受为期 9 个月的多学科康复治疗的可行性、安全性和疗效。
20 名 HD 患者被分为两组,根据认知和运动评分进行均衡匹配。一组接受干预,另一组作为对照组。统一亨廷顿病评定量表(UHDRS)运动总分是主要的观察指标。神经认知/心理学测试、身体成分、姿势稳定性、力量和生活质量评估是次要观察指标。
干预组的运动和姿势稳定性恶化得到了改善,抑郁、认知和生活质量有轻微改善。脂肪量和力量有显著增加。
本研究表明,在早、中期 HD 中进行长期的多学科康复治疗是可行的、耐受良好的,且具有治疗效果。需要进一步开展更大规模的探索性研究。