Department of Clinical and Experimental Medicine, Neurology - Neurophysiopathology Unit, Pisa University Medical School, Cisanello Hospital, Via Paradisa, n. 2, 56124, Pisa, Italy.
Integrated Care Department of Medical Specialties, Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy.
Neurol Sci. 2019 Oct;40(10):2065-2071. doi: 10.1007/s10072-019-03938-9. Epub 2019 May 25.
Respiratory failure represents an unavoidable step in patients with amyotrophic lateral sclerosis (ALS) and other motor neuron diseases (MND). The development of diaphragm ultrasonography (DUS) provides an alternative useful and risk-free tool to supply clinical, functional, and neurophysiological assessment of respiratory muscle weakness. Our aim was to evaluate if sonographic changes (thickness and echogenicity in the costal portion of the diaphragm, at rest and during respiratory movements) may be used in ALS patients to assess disease severity over time, to rule out any risk or discomfort due to traditional neurophysiological investigations.
Twenty ALS patients (mean age, 64.6 ± 10.5 years) were enrolled and data were compared with age-matched healthy volunteers; DUS data were correlated with respiratory function and disease severity scale. Examinations were performed using Telemed Echo-wave II or Esaote MyLabGamma devices in conventional B-Mode.
Mean resting thickness was reduced in all cases; changes in thickness during inspiration and expiration were also reduced (p < 0.0001) and lost in severe cases (n = 3). In bulbar-onset disease, respiratory scores were strictly correlated with the difference in diaphragm thickness between full inspiration-and expiration-as well as on the diaphragm thickness in expiration (p < 0.001).
DUS represents a simple, painless, and risk-free tool; moreover, it provides useful functional and structural insights to the understanding of diaphragm function and the degree of respiratory failure in ALS.
呼吸衰竭是肌萎缩侧索硬化症（ALS）和其他运动神经元疾病（MND）患者不可避免的阶段。膈肌超声检查（DUS）的发展为提供临床、功能和神经生理学评估呼吸肌无力提供了一种替代的有用且无风险的工具。我们的目的是评估膈肌超声检查（DUS）在 ALS 患者中是否可以用于评估疾病严重程度随时间的变化，以排除由于传统神经生理学检查而导致的任何风险或不适。
共纳入 20 名 ALS 患者（平均年龄 64.6±10.5 岁），并与年龄匹配的健康志愿者进行比较；DUS 数据与呼吸功能和疾病严重程度评分相关。使用 Telemed Echo-wave II 或 Esaote MyLabGamma 设备在常规 B 模式下进行检查。
所有病例的静息厚度均减小；吸气和呼气时的厚度变化也减小（p<0.0001），在严重病例中消失（n=3）。在延髓发病的病例中，呼吸评分与膈肌厚度在完全吸气-呼气之间的差异以及呼气时的膈肌厚度严格相关（p<0.001）。
DUS 是一种简单、无痛且无风险的工具；此外，它还提供了有用的功能和结构见解，有助于理解膈肌功能和 ALS 患者呼吸衰竭的程度。
