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Ventilatory Support Outcomes in Amyotrophic Lateral Sclerosis (ALS) Patients

Author(s): Bebiana Conde, Natalia Martins and Joao Carlos Winck


Amyotrophic Lateral Sclerosis (ALS) represents the most common and severe motor neuron disease, with inevitable respiratory failure development. Ventilatory support (VS) has shown a valuable prognostic impact, even in bulbar-onset ALS. Thus, VS outcomes related to functional and phenotypic factors were analyzed in a cohort of ALS patients.

Methods and Findings

A prospective study was conducted in 81 patients with confirmed or probable ALS diagnosis, sent to a pulmonology clinic. From 81 patients enrolled, 11 dropped out, being only considered 70 patients (mean age 66.6 ± 11.3 years, 64.3% males, 52.9% ALS bulbar-onset) for analysis. During follow-up, VS was established in 50 patients (in 48 noninvasive ventilation). A good adherence was seen in 39 patients, with residual nocturnal events only observed in 10 patients. Regarding VS initiation criteria, 24 patients were eligible by functional criteria, 14 by nocturnal hypoventilation and 12 by daytime hypercapnia. After 3-6 months VS start, there was functional improvement in 17 patients. Survival after VS was 26.3 months, being higher in spinal-onset than in bulbar-onset ALS patients (p=0.012), and was even more evident in adherent spinal-onset ALS patients (p=0.022).


VS had a marked survival impact, leading to functional improvement, mainly when started by nocturnal hypoventilation criteria.

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