Amyotrophic lateral sclerosis (ALS) is a progressive nervous system disorder. It gradually destroys the nerves responsible for muscle movement. Over time, ALS leads to almost total paralysis of muscle movement, including breathing. Eventually, the disorder leads to respiratory failure.
The Nervous System
Copyright © Nucleus Medical Media, Inc.
The cause of ALS is unknown. Genes may play a role.
Factors that may increase your chance of ALS include: Having a family member with ALSBeing in the military or having other occupations with risk of exposureHaving certain genetic mutations
Symptoms of ALS include: Progressive weakness in arms and legsWrist or foot dropDifficulty holding thingsFrequent tripping while walkingMuscle twitchingUnpredictable and changing emotionsSlurred speechHoarseness and coughingTrouble chewing and swallowing, resulting in frequent choking and gaggingWeight loss due to trouble eatingTrouble breathingExcess salivation, drooling
You will be asked about your symptoms and medical history. A physical exam will be done. There are no tests that can diagnose ALS. Tests may be used to rule out other medical conditions.
Imaging tests may include: MRI scanCT scan
Other tests may include: Blood testsLumbar puncture to evaluate cerebrospinal fluid that surrounds the brain and spinal cordBiopsy to evaluate tissue under a microscope
Your muscles and nerves may be evaluated. This can be done with
(EMG)/nerve conduction velocities (NCV).
Your cognitive skills may be assessed. This can be done with neuropsychological testing.
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 groupParticipating in social activities
Treatment options include:
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 spasticityNonsteroidal anti-inflammatory drugs (NSAIDs) and other pain medicationsAtropine,
botulinum toxin, antihistamine—To reduce heavy droolingAntidepressants and anti-anxiety drugsA combination of
dextromethorphan and quinidine—to treat inappropriate laughter or crying
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
to have a tube inserted into your airway.
Nutritional care—Your doctor may make changes to your diet. In some cases, getting nutrition through
Speech therapy—Speech therapy may be used to optimize communication. Therapy may include exploring alternate methods of communication.
There are no current guidelines to prevent ALS because the cause is unknown.
Aggarwal, SP, Zinman L, et al. Clinical trial testing lithium in ALS terminates early for futility.
Amyotrophic lateral sclerosis. EBSCO DynaMed website. Available at:
http://www.ebscohost.com/dynamed. Updated February 4, 2014. Accessed February 12, 2014.
Amyotrophic lateral sclerosis fact sheet.
National Institute of Neurological Disorders and Stroke website. Available at:
http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_amyotrophiclateralsclerosis.htm. Updated February 5, 2014. Accessed February 12, 2014.
FDA approves NeuRx Diaphragm Pacing System. ALS Association website. Available at: http://www.alsa.org/news/archive/fda-approves-neurx-diaphragm.html. Updated September 29, 2011. Accessed February 12, 2014.
NeuRx Diaphragm Pacing System. US Food & Drug Administration website. Available at: http://www.fda.gov/MedicalDevices/ProductsandMedicalProcedures/DeviceApprovalsandClearances/Recently-ApprovedDevices/ucm278684.htm. Updated September 6, 2013. Accessed February 12, 2014.
Sathasivam S. Managing patients with amyotrophic lateral sclerosis.
Eur J Intern Med.
Walling AD. Amyotrophic lateral sclerosis: Lou Gehrig's disease.
Am Fam Physician. 1999;59:1489-1496.
4/17/2008 DynaMed Systematic Literature Surveillance
http://www.ebscohost.com/dynamed: Fornai F, Longone P, et al. Lithium delays progression of amyotrophic lateral sclerosis.
Proc Natl Acad Sci USA.
1/14/2011 DynaMed's Systematic Literature Surveillance
http://www.ebscohost.com/dynamed: Mateen FJ, Carone M, et al.
Patients who survive 5 years or more with ALS in Olmsted County, 1925-2004.
J Neurol Neurosurg Psychiatry.
9/3/2014 DynaMed's Systematic Literature Surveillance. Available at:
http://www.ebscohost.com/dynamed: Wippold FJ, Cornelius RS, 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 September 3, 2014.
Last reviewed January 2015 by Rimas Lukas, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
Copyright © EBSCO Publishing. All rights reserved.