Causes

Risk Factors

Symptoms

Amyotrophic Lateral Sclerosis

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

by Rosalyn Carson-DeWitt, MD

Definition

Amyotrophic lateral sclerosis (ALS) is a nervous system disorder that 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

Causes TOP

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't send signals to the muscle, then they are unable to work. Overtime, the muscles become weak and shrink from lack of use.

Risk Factors TOP

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 TOP

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
Unpredictable and changing emotions
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 TOP

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 TOP

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 TOP

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.... Updated November 22, 2017. Accessed February 21, 2018.

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. Updated January 8, 2018. Accessed February 21, 2018.

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...: 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...: 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...: Wippold FJ, Cornelius RS, Broderick DF, et al. American College of Radiology (ACR) Appropriateness Criteria for dementia and movement disorders. Available at:
...(Click grey area to select URL)
Updated 2014. Accessed February 12, 2016.

9/26/2017 DynaMed Plus Systematic Literature Surveillance. Available at: http://www.dynamed...: 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.

Last reviewed February 2018 by EBSCO Medical Review Board Rimas Lukas, MD
Last Updated: 9/26/2017

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