• Amyotrophic Lateral Sclerosis

    (ALS; Lou Gehrig's Disease; Motor Neuron Disease)

    Definition

    Amyotrophic lateral sclerosis (ALS) is a nervous system disorder that steadily worsens over time. It affects nerves in the brain and spine that are responsible for muscle movement. The nerves gradually die which can lead to almost total paralysis, including being unable to breathe. ALS is fatal, often due to respiratory failure.
    The Nervous System
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    Causes

    The cause of ALS is unknown. Genes may play a role.
    The symptoms of ALS are caused by the death of the nerves. If the nerves can not send signals to the muscle they are not able to work. Overtime the muscles become weak and shrink from disuse.

    Risk Factors

    Factors that may increase your chance of ALS include:
    • Having a family member with ALS
    • Having certain genetic mutations
    Military veterans have twice the risk of ALS as the general population. It is not clear why.

    Symptoms

    The first signs of ALS are subtle and can be different from person to person. They may include:
    • Changes in how you speak
    • Frequent tripping while walking or general clumsiness
    • Trouble with fine motor tasks like grasping a pen
    The speed of the progression can be very different between people. As the disease progresses it can lead to:
    • Progressive weakness in arms and legs
    • Wrist or foot drop
    • Difficulty holding things
    • Muscle twitching—fasciculations
    • Unpredictable and changing emotions—pseudobulbar affect
    • Slurred speech—dysarthria
    • Hoarseness and coughing
    • Trouble chewing and swallowing, resulting in frequent choking and gagging
    • Weight loss due to trouble eating
    • Trouble breathing
    • Excess salivation, drooling

    Diagnosis

    You will be asked about your symptoms and medical history. A physical exam will be done. There are no tests that can specifically diagnose ALS. Instead, tests will be done to rule out other medical conditions.
    Imaging tests to look for changes in the brain or spine may include:
    Other tests may include:
    • Blood tests—to look for infections or metabolic change
    • Lumbar puncture—to look for changes in the fluid that surrounds the brain and spinal cord
    • Biopsy to evaluate tissue under a microscope
    Your muscles and nerves may also be checked. This can be done with electromyogram (EMG)/nerve conduction velocities (NCV).
    Your thinking and processing skills may also be checked.

    Treatment

    There is currently no cure for ALS. A combination of treatments may help to reduce or manage symptoms. Treatment options include:

    Medications

    The drug riluzole and edaravone have been approved for ALS. The drugs may slightly improve functioning, but it does not stop the disease from progressing.
    Medications may include:
    • Muscle relaxants to help muscles that are in spasm
    • Nonsteroidal anti-inflammatory drugs (NSAIDs) and other pain medications
    • Medication to reduce heavy drooling
    • Antidepressants and anti-anxiety drugs
    • Medication to treat inappropriate laughter or crying

    Other Types of Treatments

    Supportive care may be needed as ALS progresses, including:
    • Physical therapy—To reduce pain associated with muscle cramping and spasticity.
    • Respiratory care—In some cases, extra oxygen may be needed or a machine may be needed to help with breathing. Surgery will be eventually needed to make an artificial airway that bypasses the throat.
    • Nutritional care—Swallowing will eventually be lost. Nutrition will need to be delivered through a feeding tube.
    • Speech therapy—Speech therapy may help improve communication. This will not only include working with speech when possible but also finding other ways to communicate when speech is no longer possible.
    • Seeking mental health counseling and joining a support group

    Prevention

    There are no current guidelines to prevent ALS because the cause is unknown.

    RESOURCES

    ALS Association http://www.alsa.org

    National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov

    CANADIAN RESOURCES

    ALS Canada http://www.als.ca

    Amyotrophic Lateral Sclerosis Society of British Columbia http://www.alsbc.ca

    References

    Aggarwal, SP, Zinman L, Simpson E, et al. Clinical trial testing lithium in ALS terminates early for futility. Lancet Neurol. 2010;9(5):481-488.

    Amyotrophic lateral sclerosis. EBSCO DynaMed Plus website. Available at: http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS. Updated July 25, 2016. Accessed September 30, 2016.

    Amyotrophic lateral sclerosis (ALS) fact sheet. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail%5Famyotrophiclateralsclerosis.htm. Updated February 1, 2016. Accessed February 12, 2016.

    NeuRx Diaphragm Pacing System. Food and Drug Administration website. Available at: http://www.fda.gov/MedicalDevices/ProductsandMedicalProcedures/DeviceApprovalsandClearances/Recently-ApprovedDevices/ucm278684.htm. Updated September 6, 2013. Accessed February 12, 2016.

    Sathasivam S. Managing patients with amyotrophic lateral sclerosis. Eur J Intern Med. 2009;20(4):355-358.

    Walling AD. Amyotrophic lateral sclerosis: Lou Gehrig's disease. Am Fam Physician. 1999;59(6):1489-1496.

    4/17/2008 DynaMed Plus Systematic Literature Surveillance http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS: Fornai F, Longone P, Cafaro L, et al. Lithium delays progression of amyotrophic lateral sclerosis. Proc Natl Acad Sci USA. 2008;105(6):2052-2057.

    1/14/2011 DynaMed Plus Systematic Literature Surveillance http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS: Mateen FJ, Carone M, Sorenson EJ. Patients who survive 5 years or more with ALS in Olmsted County, 1925-2004. J Neurol Neurosurg Psychiatry. 2010;81(10):1144-1146.

    9/3/2014 DynaMed Plus Systematic Literature Surveillance. Available at: http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS: Wippold FJ, Cornelius RS, Broderick DF, et al. American College of Radiology (ACR) Appropriateness Criteria for dementia and movement disorders. Available at: http://www.acr.org/~/media/ACR/Documents/AppCriteria/Diagnostic/DementiaAndMovementDisorders.pdf. Updated 2014. Accessed February 12, 2016.

    9/26/2017 DynaMed Plus Systematic Literature Surveillance. Available at: http://www.dynamed.com/topics/dmp~AN~T116744/Amyotrophic-lateral-sclerosis-ALS: US Food and Drug Administration. FDA approves drug to treat ALS. Available at: https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm557102.htm. Published May 5, 2017. Accessed September 26, 2017.

    Revision Information

    • Reviewer: EBSCO Medical Review Board Rimas Lukas, MD
    • Review Date: 03/2017
    • Update Date: 09/26/2017
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