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Amyotrophic Lateral Sclerosis

(ALS; Lou Gehrig Disease; Motor Neuron Disease)

Definition

Amyotrophic lateral sclerosis (ALS) is a disease of the nervous system. It leads to the damage of nerve cells in the brain and spine. These nerves control muscle movement. People with ALS have muscles that weaken and shrink as the disease worsens.

The Nervous System
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Causes

The exact cause is not known. Genes and the environment may play a role.

Risk Factors

ALS is more common in men and in people who are 50 to 75 years of age. Other things that may raise the risk are:

  • Having a family member with ALS
  • Having certain genetic mutations

Symptoms

The first symptoms of ALS may be:

  • Changes in speech
  • Tripping while walking
  • Problems with fine motions like grasping a pen

Problems worsen over time and result in:

  • Hoarseness and coughing
  • Problems holding things
  • Slurred speech
  • Muscle twitching
  • Weakness in the arms and legs
  • Problems raising the hand or foot
  • Problems chewing and swallowing
  • Drooling
  • Weight loss due to trouble eating
  • Problems breathing
  • Mood swings

Diagnosis

You will be asked about your symptoms and your personal and family health history. A physical exam will be done. There are no tests to diagnose ALS.

Tests will be done to rule out other health issues that cause similar problems. These may be:

  • Blood and urine tests
  • Images of the brain and spine using:
  • Lumbar puncture—to look for changes in the fluid that surrounds the brain and spinal cord
  • Biopsy to closely examine muscle and nerve tissue in a lab
  • Electromyogram—to look at problems with the connection between the nerves and muscles

The care team will consider a person's symptoms and test results before making a diagnosis. A second opinion with a nerve specialist may help to confirm the diagnosis.

Treatment

There is no cure. The goal is to manage symptoms. Options are:

Medications

Some medicines may help to slow the disease. Others may be used to:

  • Ease pain
  • Relax muscles that are in spasm
  • Reduce heavy drooling
  • Manage depression and anxiety
  • Treat inappropriate laughter or crying

Supportive Care

A team of specialists will work with you to create a care plan. The team will focus on quality of life for you and your family.

ALS will affect muscles throughout the body. Chewing, swallowing, speech, and breathing can all be affected. Care will help to support the body when these changes occur. Options are:

  • Physical therapy to ease muscle cramping and spasms
  • Help with breathing, such as oxygen, breathing machines, or creating an airway in the base of the neck
  • Improving nutrition through supplements or a feeding tube
  • Speech therapy to improve communication
  • Mental health counseling and support groups to help people with ALS and their family members cope with the stress of this health problem

Prevention

There are no known guidelines to prevent this health problem.

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:

Amyotrophic lateral sclerosis. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/amyotrophic-lateral-sclerosis-als. Accessed July 19, 2021.

Amyotrophic lateral sclerosis (ALS) fact sheet. National Institute of Neurological Disorders and Stroke website. Available at: https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet. Accessed July 16, 2021.

Brent JR, Ajroud-Driss S. Amyotrophic lateral sclerosis: Integrated care provides the best quality of life. Pract Neurol (Fort Wash Pa) . Jul-Aug 2019;18:46-49.

Brent JR, Franz CK, et al. ALS: management problems. Neurol Clin. 2020;38(3):565-575.

What is ALS? ALS Association website. Available at: http://www.alsa.org/about-als/what-is-als.html. Accessed July 16, 2021.

9/26/2017 EBSCO DynaMed Systematic Literature Surveillance. Available at: https://www.dynamed.com/condition/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. Accessed September 26, 2017.

Last reviewed July 2021 by EBSCO Medical Review Board Rimas Lukas, MD  Last Updated: 7/16/2021