BACKGROUND/OBJECTIVE Weight loss is a predictor of shorter survival in amyotrophic lateral sclerosis (ALS). We performed serial measures of body composition using Dual-energy X-ray Absorptiometry (DEXA) in ALS patients to explore its utility as a biomarker of disease progression. METHODS DEXA data were obtained from participants with ALS (enrollment, at 6- and 12- months follow ups) and Parkinson's disease (enrollment and at 4-month follow up) as a comparator group. Body mass index, total lean mass index, appendicular lean mass index, total fat mass index, and percentage body fat at enrollment were compared between the ALS and PD cohorts and age-matched normative data obtained from the National Health and Nutrition Examination Survey database. Estimated monthly changes of body composition measures in the ALS cohort were compared to those of the PD cohort and were correlated with disease progression measured by the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R). RESULTS The ALS cohort (N = 20) had lower baseline total and appendicular lean mass indices compared to the PD cohort (N = 20) and general population. Loss in total and appendicular lean masses were found to be significantly associated with follow-up time. Low baseline percentage body fat (r = 0.72, p = 0.04), loss of percentage body fat (r = 0.81, p = 0.01), and total fat mass index (r = 0.73, p = 0.04) during follow up correlated significantly with monthly decline of ALSFRS-R scores in ALS cohort who had 2 or more follow-ups (N = 8). CONCLUSION Measurement of body composition with DEXA might serve as a biomarker for rapid disease progression in ALS.

中文翻译：

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脂肪量减少与肌萎缩侧索硬化患者的疾病进展更快相关：在前瞻性研究中探索双能 X 射线吸收测定法的效用。

背景/目的 体重减轻是肌萎缩侧索硬化 (ALS) 患者生存期缩短的预测因素。我们使用双能 X 射线吸收测定法 (DEXA) 在 ALS 患者中进行了身体成分的连续测量，以探索其作为疾病进展生物标志物的效用。方法 DEXA 数据来自作为比较组的 ALS（入组，6 个月和 12 个月随访）和帕金森病（入组和 4 个月随访）参与者。比较 ALS 和 PD 队列之间的体重指数、总瘦体重指数、四肢瘦体重指数、总脂肪质量指数和体脂百分比，以及从国家健康和营养检查调查数据库中获得的年龄匹配的规范数据。将 ALS 队列中身体成分测量的估计每月变化与 PD 队列进行比较，并与修订版肌萎缩侧索硬化功能评定量表 (ALSFRS-R) 测量的疾病进展相关。结果 与 PD 队列 (N = 20) 和一般人群相比，ALS 队列 (N = 20) 的基线总和四肢瘦体重指数较低。发现总和四肢瘦肉块的减少与随访时间显着相关。低基线体脂百分比 (r = 0.72, p = 0.04)、体脂百分比减少 (r = 0.81, p = 0.01) 和总脂肪质量指数 (r = 0.73, p = 0.04) 与随访期间显着相关有 2 次或更多次随访的 ALS 队列中 ALSFRS-R 评分的月度下降（N = 8）。