Wang Changsong, Li Mingjuan, Jiang Hongquan, Tong Hongshuang, Feng Yue, Wang Yue, Pi Xin, Guo Lei, Nie Maomao, Feng Honglin, Li Enyou
Department of Anesthesiology, the First Affiliated Hospital of Harbin Medical University, Harbin, China.
Department of critical care medicine, the Third Affiliated Hospital of Harbin Medical University, Harbin, China.
Sci Rep. 2016 May 23;6:26120. doi: 10.1038/srep26120.
Amyotrophic lateral sclerosis (ALS) is an incurable neurological degenerative disease. It can cause irreversible neurological damage to motor neurons; typical symptoms include muscle weakness and atrophy, bulbar paralysis and pyramidal tract signs. The ALS-mimicking disease cervical spondylotic myelopathy (CSM) presents similar symptoms, but analysis of breath volatile organic compounds (VOCs) can potentially be used to distinguish ALS from CSM. In this study, breath samples were collected from 28 ALS and 13 CSM patients. Subsequently, gas chromatography/mass spectrometry (GCMS) was used to analyze breath VOCs. Principal component analysis (PCA) and orthogonal partial least-squares discriminant analysis (OPLSDA) were the statistical methods used to process the final data. We identified 4 compounds with significantly decreased levels in ALS patients compared with CSM controls: (1) carbamic acid, monoammonium salt; (2) 1-alanine ethylamide, (S)-; (3) guanidine, N,N-dimethyl-; and (4) phosphonic acid, (p-hydroxyphenyl)-. Currently, the metabolic origin of the VOCs remains unclear; however, several pathways might explain the decreasing trends observed. The results of this study demonstrate that there are specific VOC profiles associated with ALS and CSM patients that can be used to differentiate between the two. In addition, these metabolites could contribute to a better understanding of the underlying pathophysiological mechanisms of ALS.
肌萎缩侧索硬化症（ALS）是一种无法治愈的神经退行性疾病。它会对运动神经元造成不可逆的神经损伤；典型症状包括肌肉无力和萎缩、延髓麻痹和锥体束征。类似ALS的疾病——脊髓型颈椎病（CSM）也表现出相似症状，但对呼出挥发性有机化合物（VOCs）的分析可能有助于区分ALS和CSM。在本研究中，收集了28例ALS患者和13例CSM患者的呼气样本。随后，使用气相色谱/质谱联用仪（GCMS）分析呼出的VOCs。主成分分析（PCA）和正交偏最小二乘判别分析（OPLSDA）是用于处理最终数据的统计方法。我们鉴定出4种在ALS患者中水平显著低于CSM对照组的化合物：（1）氨基甲酸单铵盐；（2）（S）-1-丙氨酸乙酰胺；（3）N，N-二甲基胍；（4）（对羟基苯基）膦酸。目前，VOCs的代谢来源尚不清楚；然而，有几种途径可能解释所观察到的下降趋势。本研究结果表明，ALS和CSM患者存在特定的VOC谱，可用于区分两者。此外，这些代谢产物可能有助于更好地理解ALS潜在的病理生理机制。
