Department of Neurology, Tokushima University Hospital, Tokushima, Japan.
Department of Neurology, Kanazawa Medical University, Kanazawa, Japan.
Acta Neurol Belg. 2021 Feb;121(1):225-230. doi: 10.1007/s13760-020-01531-y. Epub 2020 Nov 2.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder affecting the upper and lower motor neurons causing progressive weakness. It eventually involves the diaphragm which leads to respiratory paralysis and subsequently death. Phrenic nerve (PN) conduction studies and diaphragm ultrasound has been studied and correlated with pulmonary function tests in ALS patients. However, PN ultrasonography has not been employed in ALS. This study aims to sonographically evaluate the morphologic appearance of the PN of ALS patients. Thirty-eight ALS patients and 28 normal controls referred to the neurophysiology laboratory of two institutions were retrospectively included in the study. Baseline demographic and clinical variables such as disease duration, ALS Functional Rating Scale-Revised score, and ALS region of onset were collected. Ultrasound was used to evaluate the PN cross-sectional area (CSA) of ALS and control subjects. The mean PN CSA of ALS patients were 1.08 ± 0.39 mm on the right and 1.02 ± 0.34 mm on the left. The PN CSA of ALS patients were significantly decreased compared to controls (p value < 0.00001). The PN CSA of ALS patients was not correlated to any of the demographic and clinical parameters tested. This study demonstrates that ALS patients have a smaller PN size compared to controls using ultrasonography.
肌萎缩侧索硬化症（ALS）是一种影响上下运动神经元的神经退行性疾病，导致进行性无力。它最终累及横膈膜，导致呼吸肌麻痹，随后死亡。膈神经（PN）传导研究和膈肌超声已在 ALS 患者中进行了研究，并与肺功能测试相关。然而，PN 超声检查尚未应用于 ALS。本研究旨在通过超声评估 ALS 患者 PN 的形态外观。
本研究回顾性纳入了来自两个机构神经生理学实验室的 38 名 ALS 患者和 28 名正常对照者。收集了基线人口统计学和临床变量，如疾病持续时间、肌萎缩侧索硬化功能评定量表修订版评分和 ALS 发病部位。使用超声评估 ALS 和对照组患者的 PN 横截面积（CSA）。
ALS 患者右侧 PN CSA 的平均值为 1.08±0.39mm，左侧为 1.02±0.34mm。与对照组相比，ALS 患者的 PN CSA 明显减小（p 值<0.00001）。ALS 患者的 PN CSA 与测试的任何人口统计学和临床参数均无相关性。
本研究表明，与对照组相比，ALS 患者的 PN 大小使用超声检查明显较小。
