Orlova Aleksandra, Malygin Yaroslav, Gofman Anna, Sotulenko Sofija, Gandalian Veronika, Kartashov Ioan, Brylev Lev, Bolevich Sergey, Nikolic Turnic Tamara, Jakovljevic Vladimir
Department of Pathological Physiology, Institute of Digital Biodesign and Modelling of Living Systems, I.M. Sechenov First Moscow State Medical University (Sechenov University), 119991 Moscow, Russia.
Faculty of Fundamental Medicine, Lomonosov Moscow State University, 119991 Moscow, Russia.
Life (Basel). 2024 Dec 16;14(12):1664. doi: 10.3390/life14121664.
Amyotrophic lateral sclerosis is a neurodegenerative disease with high rates of disability and mortality. Non-invasive ventilation (NIV) is an effective method of treating patients, increasing life expectancy, but currently, predictors available to determine the best outcome of therapy in this category of patients are unknown. This systematic review aimed to determine the impact of prognostic factors on benefits from NIV application compared with non-NIV tools of treatment (invasive ventilation and standard care) in case of survival of ALS patients.
We systematically sought relevant longitudinal cohort and case-control studies published in PubMed, CINAHL/EMBASE, Cochrane library, and Scopus.
We included seven prospective studies, published in 2010-2020, in the analysis. According to the evidence base available to date, NIV favors survival compared to non-NIV in patients with bulbar onset ALS. We obtained conflicting data on the significance of spinal onset and bulbar function. Survival depending on patient age, and also for spinal, cervical, and flail limb phenotypes during NIV therapy has not been sufficiently studied and needs further investigation.
The studies analyzed in this review allow us to state with confidence that NIV is effective in bulbar onset ALS, taking into account recommendations for duration of ventilation and the use of the full range of symptomatic therapy, including mechanically assisted coughing. The effectiveness of NIV on severe bulbar symptoms requires further research.
肌萎缩侧索硬化症是一种致残率和死亡率都很高的神经退行性疾病。无创通气（NIV）是治疗此类患者的有效方法，可延长预期寿命，但目前尚不清楚哪些预测因素能确定这类患者的最佳治疗效果。本系统评价旨在确定在肌萎缩侧索硬化症患者生存的情况下，与非NIV治疗手段（有创通气和标准治疗）相比，预后因素对应用NIV治疗获益的影响。
我们系统检索了发表在PubMed、CINAHL/EMBASE、Cochrane图书馆和Scopus上的相关纵向队列研究和病例对照研究。
我们纳入了2010年至2020年发表的七项前瞻性研究进行分析。根据目前可得的证据，与非NIV治疗相比，NIV有利于球部起病的肌萎缩侧索硬化症患者生存。关于脊髓起病和球部功能的意义，我们获得了相互矛盾的数据。关于NIV治疗期间患者年龄以及脊髓、颈部和连枷肢体表型对生存的影响尚未得到充分研究，需要进一步调查。
本综述分析的研究使我们有信心指出，考虑到通气持续时间的建议以及包括机械辅助咳嗽在内的全方位对症治疗的使用，NIV对球部起病的肌萎缩侧索硬化症有效。NIV对严重球部症状的有效性需要进一步研究。
