Article Text

other Versions

Download PDFPDF
Prognosis of amyotrophic lateral sclerosis with respiratory onset
  1. Christen L Shoesmith (cshoesmi{at}
  1. University of Western Ontario, Canada
    1. Karen Findlater (karen.findlater{at}
    1. University of Western Ontario, Canada
      1. Ann Rowe (annm.rowe{at}
      1. University of Western Ontario, Canada
        1. Michael J. Strong (mstrong{at}
        1. University of Western Ontario, Cambodia


          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
          • prognosis
          • respiratory

          Statistics from

          Request Permissions

          If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.