Respiratory muscle involvement is a recognized, but often late, complication of amyotrophic lateral sclerosis. In this paper, we describe the clinical features and prognosis of 21 patients with respiratory onset ALS. A retrospective chart review found that 2.7% of patients with ALS presenting to a tertiary care specialty clinic have respiratory symptoms as their first clinical symptom of ALS. Only 14% of these individuals presented acutely and required emergency intubation. The mean survival of the total group from symptom onset to death or permanent ventilation was 27.0 (± 14.9) months, which was not significantly different than survival in patients with bulbar onset ALS. NIPPV significantly improved survival compared with those who did not use NIPPV. This study suggests that ALS with respiratory onset does not necessarily follow a rapidly progressive course.
- amyotrophic lateral sclerosis