Amyotrophic Lateral Sclerosis
Neurological
Context
- Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder, with an unknown etiology, clinically characterized by dysfunction of upper and lower motor neurons.
- ALS is the most common motor neuron disease in adults with an incidence of 1–2.6 cases per 100,000 persons annually.
Diagnostic Process
The symptoms and signs of ALS typically include:
- Muscle weakness which often begins in the hands, arms, legs or feet
- Muscle atrophy and wasting
- Spasticity
- Difficulty with speech and swallowing
- Respiratory Distress
- Fatigue and Weight loss
- Fasciculations
Refer to neurology for diagnosis.
- Common tests used include blood and urine studies, electromyography and nerve conduction velocity tests, and an MRI of the central nervous system.
Recommended Treatment
There is no cure for ALS — treatment is targeted to manage symptoms and improve quality of life. Refer to neurology for treatment.
- Riluzole and edaravone are the only drugs approved by Health Canada for treatment of ALS.
Criteria For Hospital Admission
Typically, advanced directives and community resources will be in place, but admission may indicated for:
- Airway or respiratory compromise especially in setting of reversible cause (infection).
- Complications such as skin breakdown from pressure sores.
Criteria For Close Observation And/or Consult
- Consultation with neurology is recommended.
Quality Of Evidence?
High
We are highly confident that the true effect lies close to that of the estimate of the effect. There is a wide range of studies included in the analyses with no major limitations, there is little variation between studies, and the summary estimate has a narrow confidence interval.
Moderate
We consider that the true effect is likely to be close to the estimate of the effect, but there is a possibility that it is substantially different. There are only a few studies and some have limitations but not major flaws, there are some variations between studies, or the confidence interval of the summary estimate is wide.
Low
When the true effect may be substantially different from the estimate of the effect. The studies have major flaws, there is important variations between studies, of the confidence interval of the summary estimate is very wide.
Justification
Evidence is derived from well conducted RCTs and systematic reviews that have evaluated safety and efficacy.
Related Information
Reference List
Talbott EO, Malek AM, Lacomis D. Chapter 13 – The epidemiology of amyotrophic lateral sclerosis. In: Aminoff MJ, Boller F, Swaab DF, editors. Handbook of Clinical Neurology [Internet]. Elsevier; 2016 [cited 2023 May 19]. p. 225–38. (Neuroepidemiology; vol. 138). Available from: https://www.sciencedirect.com/science/article/pii/B9780128029732000136
Verma A. Clinical Manifestation and Management of Amyotrophic Lateral Sclerosis. In: Araki T, editor. Amyotrophic Lateral Sclerosis [Internet]. Brisbane (AU): Exon Publications; 2021 [cited 2023 May 19]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK573427/
RESOURCE AUTHOR(S)
DISCLAIMER
The purpose of this document is to provide health care professionals with key facts and recommendations for the diagnosis and treatment of patients in the emergency department. This summary was produced by Emergency Care BC (formerly the BC Emergency Medicine Network) and uses the best available knowledge at the time of publication. However, healthcare professionals should continue to use their own judgment and take into consideration context, resources and other relevant factors. Emergency Care BC is not liable for any damages, claims, liabilities, costs or obligations arising from the use of this document including loss or damages arising from any claims made by a third party. Emergency Care BC also assumes no responsibility or liability for changes made to this document without its consent.
Last Updated Jun 17, 2023
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