Cubo Esther, Rivadeneyra Jessica, Gil-Polo Cecilia, Armesto Diana, Mateos Ana, Mariscal-Pérez Natividad
Neurology Department, Hospital Universitario Burgos, Spain.
Research Unit, Hospital Universitario Burgos, Spain.
J Neurol Sci. 2015 Nov 15;358(1-2):335-8. doi: 10.1016/j.jns.2015.09.351. Epub 2015 Sep 15.
Skeletal muscle wasting is likely to play an important role in the Huntington's disease (HD) pathogenesis. Our aim was to analyze the body composition, and specifically fat-free mass (FFM), as an indirect marker of skeletal muscle in patients with HD, and its association with HD severity and energy balance.
Cross-sectional, case-control study. Body composition was analyzed using bioelectrical impedance. Information was collected as regards of the anthropometrics, disease severity [Unified Huntington Disease Rating (UHDRS) and Total functional capacity (TFC) scores], CAG repeats, protein catabolism, energy intake and energy expenditure.
Twenty two patients with HD [mean age 50.3±15.6, mean UHDRS of 27.9±23.7, median TFC of 11 (IQR: 7; 13); median body mass index 23.6 (IQR: 26.8; 22.5)], and 18 controls were included. Both groups were similar in terms of age, gender, body mass index, body composition, physical activity level, and protein catabolism. FFM was correlated with energy intake (r=0.73, p<0.001), resting energy expenditure (r=0.64, p=0.001) and physical activity (r=0.54, p=0.003), but not with CAG repeats, or HD severity.
Our results do not support the presence of significant muscle wasting in patients with early-moderate Huntington's disease. However, to prevent muscle wasting in HD, dietary strategies, in addition to physical exercise, should be further investigated.
骨骼肌萎缩可能在亨廷顿舞蹈病(HD)的发病机制中起重要作用。我们的目的是分析HD患者的身体组成,特别是去脂体重(FFM),作为骨骼肌的间接标志物,及其与HD严重程度和能量平衡的关系。
横断面病例对照研究。使用生物电阻抗分析身体组成。收集有关人体测量学、疾病严重程度[统一亨廷顿疾病评定量表(UHDRS)和总功能能力(TFC)评分]、CAG重复序列、蛋白质分解代谢、能量摄入和能量消耗的信息。
纳入22例HD患者[平均年龄50.3±15.6岁,平均UHDRS为27.9±23.7,TFC中位数为11(四分位间距:7;13);体重指数中位数为23.6(四分位间距:26.8;22.5)]和18名对照者。两组在年龄、性别、体重指数、身体组成、身体活动水平和蛋白质分解代谢方面相似。FFM与能量摄入(r=0.73,p<0.001)、静息能量消耗(r=0.64,p=0.001)和身体活动(r=0.54,p=0.003)相关,但与CAG重复序列或HD严重程度无关。
我们的结果不支持早-中度亨廷顿舞蹈病患者存在明显肌肉萎缩。然而,为预防HD患者的肌肉萎缩,除体育锻炼外,还应进一步研究饮食策略。