emDocs - December 13, 2017 - By Gipson J - Edited by: Koyfman A and Long B
"Take Home Points
- ALS is a progressive, degenerative disease which leads to paralysis and respiratory failure. It inevitably results in death, usually within 3-5 years from the onset of symptoms.
- ALS has a variety of phenotypes, and it is important for the emergency physician to recognize the various ways the disease can present and to refer patients with signs/symptoms of the disease to a neurologist for further testing.
- Bilevel positive pressure ventilation has been shown to reduce symptoms, prolong life, and improve quality of life in patients with ALS.
- Patients with ALS should have relatively normal lung function and generally do not require supplemental oxygen unless a secondary process is present. Supplemental oxygen should be administered concurrently with NIV when it is needed.
- ALS treatment strategies should focus on symptomatic treatment and should serve to enhance quality of life while maintaining patient autonomy."