Berlowitz DJ, Howard ME, Fiore JF, et al. Identifying who will benefit from noninvasive ventilation in amyotrophic lateral sclerosis/motor neurone disease in a clinical cohort. J Neurol Neurosurg Psychiatry 2016;87: 280–286.
Submitted by David Haustein, MD, News Science Editorial Board
Over the last 15 years, noninvasive ventilation (NIV) has seen increasing use for patients with amyotrophic lateral sclerosis (ALS) with the goal of prolonging survival and slowing respiratory decline. In this retrospective analysis from a subspecialty center in Australia, investigators sought to quantify the effect of NIV on survival and respiratory function decline across different ALS. Data collection occurred from 1991 through 2011; the providers began routinely prescribing NIV in 2002. 929 patients with ALS-bulbar onset, ALS-cervical onset, ALS-lumbar onset or flail limb were included in the study; 219 received NIV and 711 did not. Using both univariate and multivariate Cox-regression analysis (to account for percutaneous endoscopic gastrostomy placement, riluzole use, age of onset and gender), median tracheostomy-free survival increased from 15 months to 28.63 months across all phenotypes, an increase of 90%. The survival for ALS-bulbar onset patients increased from 13.57 to 32.61 months, an increase of 140%. NIV also reduced rates of decline of forced vital capacity (FVC), forced expiratory volume in one second (FEV1), maximal inspiratory and expiratory pressures (MEP and MIP, respectively).
Comment: This retrospective study involving a large cohort of patients with ALS spans the period where NIV became commonly prescribed for patients with ALS. It bolsters the evidence to support NIV prescription in patients with ALS, prolonging survival by 90% across all phenotypes and 140% for bulbar-onset patients and slowing the rate of respiratory decline.
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