Electrical impedance tomography for the improved assessment of pulmonary function in neuromuscular disease

项目概要 呼吸无力是许多神经肌肉疾病的常见表现，包括 肌萎缩侧索硬化症 (ALS)、重症肌无力、吉兰-巴利综合征和某些肌肉萎缩症 营养不良。标准肺功能测试 (PFT) 存在许多局限性。其中包括：1. 无法获得伴有延髓无力的患者的准确数据 2. 对患者的依赖 能够有效合作； 3. 无法获得持续的评估，尤其是当患者处于治疗状态时 重症监护病房； 4. 它无法深入了解肺部状况，包括是否存在肺不张。一 为监测肺功能提供潜在更有效手段的方法是通过应用 电阻抗断层扫描（EIT）。在EIT中，放置了嵌入多个表面电极的带 胸部周围，通过非常微弱的非电离电流获得低分辨率图像 在不同的电极组之间进行测量并测量产生的电压。实时呼吸视频可以 患者在休息时呼吸舒适，吸气和呼气充分。从这些 通过图像，可以得出肺功能的简单指标。该技术已经在关键领域得到应用 护理患有多种疾病的患者并且非常安全，甚至被用于早产儿来帮助监测 肺部状况和肺成熟度。在此 R21 提案中，我们寻求建立以下具体应用： 这项技术适用于有 ALS 风险或患有肺功能障碍的患者，因为他们代表了一个群体 该技术可能对谁特别有用。在具体目标 1 中，我们计划评估该技术的可靠性 在 ALS 患者以及年龄和性别匹配的健康对照中进行测试，并确定其相对敏感性 肺功能障碍。在具体目标2中，我们将确定EIT值与标准之间的关系 大约 3 个月内的横向和纵向肺功能参数 时间，无论患者是否伴有延髓无力。在本研究结束时，我们希望 证明了 EIT 在 ALS 肺功能评估中的潜在价值，为其打开了大门 广泛应用于各种神经肌肉疾病患者的急性和慢性护理。

PROJECT SUMMARY Respiratory weakness is a common manifestation of many neuromuscular diseases, including amyotrophic lateral sclerosis (ALS), myasthenia gravis, Guillain-Barre syndrome, and certain muscular dystrophies. Standard pulmonary function tests (PFTs) suffer from a number of limitations. These include: 1. inability to obtain accurate data in patients with concomitant bulbar weakness 2. Reliance on the patient being able to cooperate effectively; 3. Inability to obtain continuous assessment, especially when the patient is in the intensive care unit; 4. It provides no insight into pulmonary condition, including the presence of atelectasis. One approach that offers a potential more effective means for monitoring pulmonary function is via the application of electrical impedance tomography (EIT). In EIT, a belt embedded with multiple surface electrodes is placed around the chest and a low-resolution image is obtained by passing a very weak, non-ionizing electrical current between varying sets of electrodes and measuring the resulting voltages. Real-time videos of breathing can be obtained with the patient at rest breathing comfortably and also with full inspiration and expiration. From these images, simple metrics of pulmonary function can be derived. The technology has already found use in critical care patients with a variety of disorders and is very safe, it is even being used in premature infants to help monitor pulmonary condition and lung maturation. In this R21 proposal, we seek to establish the specific application of this technology in patients at risk for or with pulmonary dysfunction with ALS since they represent a group in whom the technology may be especially useful. In specific aim 1, we plan to assess the reliability of the technique in both ALS patients and age- and sex-matched healthy controls and to determine its relative sensitivity to pulmonary dysfunction. In specific aim 2, we will determine the relationship between EIT values and standard pulmonary function parameters both cross-sectionally and longitudinally over an approximately 3-month period of time, in patients both with and without concomitant bulbar weakness. At the end of this study we hope to have demonstrated the potential value for EIT the assessment of pulmonary function in ALS, opening the door to its wide application in the acute and chronic care of patients across a range of neuromuscular diseases.