[Amyotrophic lateral sclerosis and intermittent self ventilation therapy. Indications and follow-up].

BACKGROUND

Fully informed patients were willing to start with IPPV.

PATIENTS AND METHODS

Twenty-three patients with amyothropic lateral sclerosis (ALS) were treated with noninvasive intermittent mechanical ventilation (7 women, 16 men, mean age 59 years). The major symptoms were dyspnoe, heavy sleep disruption resulting in severely reduced quality of life.

RESULTS

Their symptoms improved. The blood gases normalized during spontaneous breathing. They remained stable, despite a rapid decrease in respiratory force. Thirteen patients died, 6 during an acute event, 4 during bronchopulmonary infection and 3 stopped therapy, when they were fully ventilator dependent.

CONCLUSION

We conclude that respiratory symptoms and quality of life can be improved by home intermittent mechanical ventilation. Indication should therefore be made earlier.