Management and prognosis of respiratory insufficiency in neuromuscular disease

Abstract

Acute respiratory insufficiencies secondary to a neuromuscular disease represents about 1.5% of admissions to ICU. Two different conditions can be encountered:  an acute respiratory insufficiency secondary to a reversible disease (Guillain-Barré syndrome, myasthenia gravis, …) or an acute respiratory insufficiency secondary to a degenerative disease (amyotrophic lateral sclerosis, Steinert’s disease, …).

The management of these two conditions is very specific and should focus on finding the optimal moment for orotracheal intubation and mechanical ventilation, and on determining the best weaning strategy. Nevertheless, the available data are scarce and mainly made of case-series, retrospective studies are seldom prospective ones. Current guidelines are thus mainly expert’s advices rather then well established facts.

We will successively discuss general notion, the right time for intubation and mechanical ventilation, the place of tracheostomy, the weaning process and the prognosis.