LONG-TERM VENTILATION AT HOME IN ADULTS WITH NEUROLOGICAL DISEASES

Respiratory handicap due to neurological diseases is often underestima ted. Given clinical signs are either mild or absent, systematic measur ement of the vital capacity is the best mean to detect in practice the restrictive syndrome. The onset of home mechanical ventilatory suppor t should be decided at steady state, apart from episodes of acute resp iratory failure. Two types of indications should be distinguished. Nec essary ventilation aims at supplying over day and night the respirator y insufficiency incurred by the paralysis of respiratory muscles. Alth ough the criteria for the use of such a supply differ according to the neurological disease, a daytime hypercapnia above 45 mmHg is widely a ccepted 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 therapeut ic strategy in Duchenne de Boulogne muscular dystrophy showed that, gi ven that tracheostomy will become necessary in this evolutive disease, proposal of an early tracheostomy is not nonsensical. By contrast, pr eventive ventilation aims at preventing from the aggravation of the re strictive syndrome in those patients with no criterion for necessary v entilation. It has been proved ineffective in Duchenne muscular dystro phy through a controlled clinical trial.