EFFECT OF NONINVASIVE POSITIVE-PRESSURE VENTILATION ON SURVIVAL IN AMYOTROPHIC-LATERAL-SCLEROSIS

Background: Noninvasive positive-pressure ventilation may benefit pati ents with amyotrophic lateral sclerosis and respiratory insufficiency. Objective: To determine 1) whether patients tolerant of noninvasive p ositive-pressure ventilation have better survival than intolerant pati ents and 2) whether bulbar symptoms account for intolerance of noninva sive positive-pressure ventilation. Design: Observational cohort study . Setting: Tertiary care referral center. Patients: 39 patients with a myotrophic lateral sclerosis who were treated with noninvasive positiv e-pressure ventilation. Intervention: Noninvasive positive-pressure ve ntilation was started for patients with new orthopnea, new hypercapnia , or both. Patients were divided into two groups: those tolerant of an d those intolerant of noninvasive positive-pressure ventilation. Resul ts: The risk for death from onset of respiratory insufficiency was hig her for intolerant patients than for tolerant patients. (relative risk , 3.1 [95% CI, 1.8 to 9.6]). Moderate or severe bulbar symptoms were m ore prevalent among intolerant patients than among tolerant patients ( 67% compared with 33%; P = 0.04). Conclusions: Among patients with amy otrophic lateral sclerosis, those who are tolerant of noninvasive posi tive-pressure ventilation have better survival than do those who are i ntolerant. Bulbar symptoms partially account for intolerance of noninv asive positive-pressure ventilation.