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Amyotrophic Lateral Sclerosis

Definition

Amyotrophic lateral sclerosis (ALS) is a progressive nervous system disorder. It gradually destroys the neurons responsible for muscle movement. Specifically, it affects the upper and lower motor neurons. Over time, ALS leads to almost total paralysis of muscle movement, including breathing. Eventually, the disorder leads to respiratory failure.

The Nervous System
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Causes

The cause of ALS is unknown. Genes may play a role.

Risk Factors

Factors that may increase your chance of ALS include:

  • Having a family member with ALS
  • Being in the military or having other occupations with risk of exposure
  • Having certain genetic mutations

Symptoms

Symptoms of ALS include:

  • Progressive weakness in arms and legs
  • Wrist or foot drop
  • Difficulty holding things
  • Frequent tripping while walking
  • 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 definitively diagnose ALS. Tests may be used to rule out other medical conditions.

Imaging tests may include:

Other tests may include:

  • Blood tests
  • Lumbar puncture to evaluate cerebrospinal fluid that surrounds the brain and spinal cord
  • Biopsy to evaluate tissue under a microscope

Your muscles and nerves may be evaluated. This can be done with electromyogram (EMG)/nerve conduction velocities (NCV).

Your cognitive skills may be assessed. This can be done with neuropsychological testing.

Treatment

There is currently no cure for ALS.

Treatment may help to reduce or manage symptoms. A combination of treatments may work best. This may include:

  • Taking medications
  • Working with therapists and joining a support group
  • Participating in social activities

Treatment options include:

Medications

The drug riluzole has been approved for ALS. The drug may slightly improve functioning, but it does not stop the disease from progressing.

Medications may include:

  • Muscle relaxants reduce spasticity
  • Nonsteroidal anti-inflammatory drugs (NSAIDs) and other pain medications
  • Atropine, scopolamine, botulinum toxin, antihistamine to reduce heavy drooling
  • Antidepressants and anti-anxiety drugs
  • A combination of dextromethorphan and quinidine 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, you may need to receive a mixture of air and oxygen from a machine. A device may also be used that helps your breathing muscles contract. If you cannot move enough air in and out of your lungs, you may need surgery to have a tube inserted into your airway.
  • Nutritional care—Your doctor may make changes to your diet. In some cases, getting nutrition through tube feeding is needed.
  • Speech therapy—Speech therapy may be used to optimize communication. Therapy may include exploring alternate methods of communication.

Prevention

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

Revision Information

  • Reviewer: Rimas Lukas, MD
  • Review Date: 02/2016 -
  • Update Date: 02/12/2016 -
  • ALS Association

    http://www.alsa.org

  • National Institute of Neurological Disorders and Stroke

    http://www.ninds.nih.gov

  • ALS Canada

    http://www.als.ca

  • Amyotrophic Lateral Sclerosis Society of British Columbia

    http://www.alsbc.ca

  • 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.