Brito Ozana, Fregonezi Guilherme, Pondofe Karen, Vieira Rayane Grayce da Silva, Ribeiro Tatiana, Dourado Júnior Mário Emílio, Fidelix Emanuela Coriolano, Nagem Danilo, Valentim Ricardo, Sarmento Antonio, Resqueti Vanessa
PneumoCardioVascular Lab/HUOL, Onofre Lopes University Hospital, Brazilian Hospital Services Company (EBSERH), Federal University of Rio Grande do Norte, Natal 59010-090, Brazil.
Laboratory of Technological Innovation in Health, Onofre Lopes University Hospital, Brazilian Hospital Services Company (EBSERH), Federal University of Rio Grande do Norte, Natal 59010-090, Brazil.
Healthcare (Basel). 2024 Mar 11;12(6):627. doi: 10.3390/healthcare12060627.
This study aimed to monitor the clinical and functional progression of patients with amyotrophic lateral sclerosis (ALS) and adjust ventilatory support during the COVID-19 pandemic in Brazil using telemedicine. This longitudinal case series included five evaluations from January 2019 to June 2021. The first and second assessments were performed in person and consisted of pulmonary function, respiratory muscle strength, functionality (ALS Functional Rating Scale-Revised [ALSFRS-R]) and disease staging (King's College criteria). The use of non-invasive ventilation (NIV), ALSFRS-R, and disease staging were assessed in the third, fourth, and fifth assessments during the COVID-19 pandemic, using telemedicine. The rate of functional decline was calculated by the difference in the total score of ALSFRS-R between evaluations. A cutoff of 0.77 in the ALSFRS-R was used to characterize the speed of functional decline. Eleven patients (mean age of 51 years, eight males) were assessed. The total score of the ALSFRS-R ( < 0.01) and its motor domain ( < 0.01) reduced significantly during the pandemic. NIV prescription increased from 54.4% to 83.3%. Telemedicine helped with the clinical and functional follow-up of patients with ALS.
本研究旨在监测肌萎缩侧索硬化症（ALS）患者的临床和功能进展，并在巴西新冠疫情期间利用远程医疗调整通气支持。这个纵向病例系列包括2019年1月至2021年6月的五次评估。第一次和第二次评估是面对面进行的，包括肺功能、呼吸肌力量、功能（修订版ALS功能评定量表[ALSFRS-R]）和疾病分期（国王学院标准）。在新冠疫情期间的第三次、第四次和第五次评估中，利用远程医疗评估无创通气（NIV）的使用情况、ALSFRS-R和疾病分期。功能下降率通过评估之间ALSFRS-R总分的差异来计算。使用ALSFRS-R中0.77的临界值来表征功能下降的速度。对11名患者（平均年龄51岁，8名男性）进行了评估。在疫情期间，ALSFRS-R的总分（<0.01）及其运动领域（<0.01）显著降低。NIV处方从54.4%增加到83.3%。远程医疗有助于对ALS患者进行临床和功能随访。
