Ryan Terence E, Erickson Melissa L, Verma Ajay, Chavez Juan, Rivner Michael H, Mccully Kevin K
Department of Kinesiology, University of Georgia, Athens, Georgia, USA.
Muscle Nerve. 2014 Nov;50(5):767-74. doi: 10.1002/mus.24223. Epub 2014 Sep 29.
Mitochondrial dysfunction in the motor neuron has been suspected in amyotrophic lateral sclerosis (ALS). If mitochondrial abnormalities are also found in skeletal muscle, assessing skeletal muscle could serve as an important biomarker of disease progression.
Using 31P magnetic resonance (31P-MRS) and near infrared (NIRS) spectroscopy, we compared the absolute values and reproducibility of skeletal muscle oxidative capacity in people with ALS (n = 6) and healthy adults (young, n = 7 and age-matched, n = 4).
ALS patients had slower time constants for phosphocreatine (PCr) and muscle oxygen consumption (mVO2 ) compared with young, but not age-matched controls. The coefficient of variation for the time constant was 10% (SD = 2.8%) and 17% (SD = 6.2%) for PCr and mVO2 , respectively.
People with ALS had, on average, a small but not statistically significant, impairment in skeletal muscle mitochondrial function measured by both 31P-MRS and NIRS. Both methods demonstrated good reproducibility.
肌萎缩侧索硬化症（ALS）患者的运动神经元中存在线粒体功能障碍。如果在骨骼肌中也发现线粒体异常，那么评估骨骼肌可能成为疾病进展的重要生物标志物。
我们使用31P磁共振（31P-MRS）和近红外（NIRS）光谱，比较了肌萎缩侧索硬化症患者（n = 6）与健康成年人（年轻人，n = 7；年龄匹配者，n = 4）骨骼肌氧化能力的绝对值和可重复性。
与年轻人相比，肌萎缩侧索硬化症患者的磷酸肌酸（PCr）和肌肉耗氧量（mVO2）的时间常数较慢，但与年龄匹配的对照组相比则不然。PCr和mVO2时间常数的变异系数分别为10%（标准差=2.8%）和17%（标准差=6.2%）。
平均而言，通过31P-MRS和NIRS测量，肌萎缩侧索硬化症患者的骨骼肌线粒体功能存在轻微但无统计学意义的损害。两种方法均显示出良好的可重复性。
