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    Amyotrophic Lateral Sclerosis


    Last Updated Jun 17, 2023
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    By Joe Finkler, Allie Cui


    • 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?


    Evidence is derived from well conducted RCTs and systematic reviews that have evaluated safety and efficacy.


    Related Information

    Reference List

    1. 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

    2. 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/


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