Andersen Tiina M, Sandnes Astrid, Fondenes Ove, Nilsen Roy M, Tysnes Ole-Bjørn, Heimdal John-Helge, Clemm Hege H, Halvorsen Thomas, Vollsæter Maria, Røksund Ola D
Norwegian Centre of Excellence for Home Mechanical Ventilation, Thoracic Department, Haukeland University Hospital, Bergen, Norway.
Department of Physiotherapy, Haukeland University Hospital, Bergen, Norway.
Respir Care. 2018 May;63(5):538-549. doi: 10.4187/respcare.05924. Epub 2018 Apr 17.
Respiratory complications represent the major cause of death in amyotrophic lateral sclerosis (ALS). Noninvasive respiratory support is the mainstay therapy, but treatment becomes challenging as the disease progresses, possibly due to a malfunctioning larynx, which is the entrance to the airways. We studied laryngeal response patterns to mechanically assisted cough (mechanical insufflation-exsufflation) as ALS progresses.
This prospective longitudinal study of 13 consecutively included subjects with ALS were followed up during 2011-2016 with repeated tests of lung function, neurological status, and laryngeal responses to mechanical insufflation-exsufflation using video-recorded flexible transnasal fiberoptic laryngoscopy.
Follow-up time was median 17 (range 6-59) months. In total, 751 laryngoscopy recordings from 67 individual examinations (median 4 per subject, range 2-11 per subject) were analyzed. Adverse laryngeal events that developed with disease progression during insufflation included adduction of true vocal folds in 8 of 9 spinal-onset subjects and adduction of aryepiglottic folds in all subjects, initially at the highest positive pressure and prior to onset of other bulbar symptoms in spinal-onset subjects. As cough became less expulsive with disease progression, laryngeal adduction occurred at lower insufflation pressures. Retroflex movement of the epiglottis was observed in 7 of 13 subjects regardless of insufflation pressures and independent of bulbar involvements. Backward movement of the tongue base occurred regardless of insufflation pressures in all but 1 subject. During exsufflation, constriction of the hypopharynx was observed in all subjects regardless of the presence of bulbar symptoms, after the adverse events that occurred during insufflation.
Applying high insufflation pressures during mechanically assisted cough in ALS can become counterproductive as the disease progresses as well as prior to the onset of bulbar symptoms. The application of positive inspiratory pressures should be tailored to the individual patient, and laryngoscopy during ongoing treatment appears to be a feasible tool.
呼吸并发症是肌萎缩侧索硬化症（ALS）患者的主要死因。无创呼吸支持是主要治疗方法，但随着疾病进展，治疗变得具有挑战性，这可能是由于作为气道入口的喉部功能失调所致。我们研究了随着ALS病情进展，喉部对机械辅助咳嗽（机械吸气-呼气）的反应模式。
这项前瞻性纵向研究连续纳入了13例ALS患者，于2011年至2016年期间进行随访，重复进行肺功能、神经功能状态检查，以及使用视频记录的可弯曲经鼻纤维喉镜检查喉部对机械吸气-呼气的反应。
随访时间中位数为17（范围6 - 59）个月。总共分析了来自67次个体检查（每位受试者中位数为4次，范围为每位受试者2 - 11次）的751份喉镜检查记录。在吸气过程中，随着疾病进展出现的不良喉部事件包括9例脊髓起病患者中有8例真性声带内收，所有受试者均出现杓会厌襞内收，在脊髓起病患者中最初出现在最高正压时且早于其他延髓症状出现之前。随着疾病进展咳嗽排出能力减弱，喉部内收在较低吸气压力时出现。13例受试者中有7例观察到会厌后倾，与吸气压力无关且与延髓受累情况无关。除1例受试者外，所有受试者无论吸气压力如何均出现舌根后移。在呼气过程中，在吸气时出现不良事件后，所有受试者无论是否存在延髓症状均观察到下咽收缩。
随着ALS病情进展以及在延髓症状出现之前，在机械辅助咳嗽时施加高吸气压力可能会适得其反。正吸气压力的应用应根据个体患者进行调整，并且在持续治疗期间进行喉镜检查似乎是一种可行的工具。
