[Long-term ventilation at home in adults with neurological diseases]

Respiratory handicap due to neurological diseases is often underestimated. Given clinical signs are either mild or absent, systematic measurement of the vital capacity is the best mean to detect in practice the restrictive syndrome. The onset of home mechanical ventilatory support should be decided at steady state, apart from episodes of acute respiratory failure. Two types of indications should be distinguished. Necessary ventilation aims at supplying over day and night the respiratory insufficiency incurred by the paralysis of respiratory muscles. Although the criteria for the use of such a supply differ according to the neurological disease, a daytime hypercapnia above 45 mmHg is widely accepted in the literature. It is otherwise established to use first a non invasive technique, while tracheostomy is secondarily proposed in case of failure of these techniques. The application of this therapeutic strategy in Duchenne de Boulogne muscular dystrophy showed that, given that tracheostomy will become necessary in this evolutive disease, proposal of an early tracheostomy is not nonsensical. By contrast, preventive ventilation aims at preventing from the aggravation of the restrictive syndrome in those patients with no criterion for necessary ventilation. It has been proved ineffective in Duchenne muscular dystrophy through a controlled clinical trial.