Brain & Nervous System More Neurological Diseases What Is ALS? By Mark Stibich, PhD Mark Stibich, PhD LinkedIn Twitter Mark Stibich, PhD, FIDSA, is a behavior change expert with experience helping individuals make lasting lifestyle improvements. Learn about our editorial process Updated on December 20, 2021 Medically reviewed Verywell Health articles are reviewed by board-certified physicians and healthcare professionals. These medical reviewers confirm the content is thorough and accurate, reflecting the latest evidence-based research. Content is reviewed before publication and upon substantial updates. Learn more. by Nicholas R. Metrus, MD Medically reviewed by Nicholas R. Metrus, MD LinkedIn Nicholas R. Metrus, MD, is a board-certified neurologist and neuro-oncologist. He currently serves at the Glasser Brain Tumor Center in Summit, New Jersey. Learn about our Medical Expert Board Print Table of Contents View All Table of Contents What Is ALS? Prognosis Life Expectancy Treatments Frequently Asked Questions Amyotrophic lateral sclerosis (ALS), also called "Lou Gehrig's disease," is a progressive and ultimately fatal neurodegenerative disease that affects the nerves that control movement. ALS leads to people becoming so weak that they are paralyzed, and half of the people impacted will die within two to five years. The causes are unknown, but some combination of genetics and environmental exposures may be to blame. There is no cure for ALS, but there are some treatments that may slow the loss of physical function and improve the quality of life for people impacted by it. Verywell / Melissa Ling What Is ALS? ALS is a neurodegenerative disease that affects motor neurons, which are the nerve cells that control the movement of muscles. "Upper motor neurons" originate in the brain. "Lower motor neurons" originate in the spinal cord. ALS destroys both upper and lower motor neurons, but a person often starts with symptoms of either upper or lower motor neuron disease. When upper motor neurons are impacted, the result is muscle stiffness ("spasticity"). When lower motor neurons are impacted, the result is muscle weakness, loss of muscle ("atrophy"), and twitching ("fasciculations"). ALS is medically classified by which motor neurons are impacted: Classical ALS is characterized by a deterioration of upper and lower motor neurons. This type of ALS affects more than two-thirds of those with the disease.Primary lateral sclerosis (PLS) is characterized by deterioration of the upper motor neurons. If the lower motor neurons are not impacted within two years, the disease usually remains a pure upper motor neuron disease. This is the rarest form of ALS.Progressive bulbar palsy (PBP) starts with difficulties in speaking, chewing, and swallowing due to lower motor neuron deterioration. This disorder affects about 25% of those with ALS.Progressive muscular atrophy (PMA) is characterized by deterioration of the lower motor neurons. If the upper motor neurons are unaffected within two years, the disease usually remains a purely lower motor neuron disease. Prognosis Unfortunately, the prognosis for ALS is poor. ALS is progressive, and it is terminal within five years for 80% of the people impacted by it. As ALS progresses, people are unable to move their arms and legs to walk or get out of bed, and they may lose the ability to talk, eat, and breathe on their own. Complications Complications can arise from the neurological damage of the disease process or from treatments: Choking, due to impaired swallowingShortness of breath, due to the atrophy of breathing musclesRisk of falling, due to weakness and atrophy of the musclesNutritional deficiencies, due to trouble eatingInfections, from medical devices like ventilators, feeding tubes, and urinary cathetersAnxiety and depression, related to an unpredictable and terminal illnessInappropriate emotional outbursts, related to damage to that part of the brainCognitive changes and dementia Life Expectancy According to the ALS Association, the average life expectancy of a person with ALS is three years. However, it varies greatly: 20% live five years or more.10% live 10 or more years.5% will live for more than 20 years. How ALS Causes Death Death from ALS is usually caused by respiratory failure, due to the muscles that allow for breathing becoming paralyzed. Both non-invasive and invasive ventilation can ease breathing, but ALS will continue to progress. Treatments While there is no cure for ALS or ways to stop the progression of the disease completely, there are some treatments that may improve life expectancy and quality of life. These include: Medication Rilutek (riluzole) has been in use since 1995 and has been shown in randomized double-blind clinical trials to prolong life expectancy for approximately two to three months, but it did not relieve symptoms. This medication also goes by the name of Tiglutek when in a liquid form and Exservan when used as an oral film, both of which are easier for people with problems swallowing. Radicava (edaravone) is a free-radical scavenger medication that was approved in 2017. In the phase 3 trial that led to its licensure, it lessened the decline in physical functioning by 33% in a very select group of people who were in the study. However, researchers caution that those results may not be generalizable to everyone with ALS. Nuedexta (dextromethorphan hydrobromide/quinidine sulfate) may help improve bulbar motor function (important for speech and swallowing). In particular, it may help to better speech control with fewer pauses, but more study is needed. Noninvasive Ventilation The use of noninvasive ventilation (NIV) is a cornerstone of ALS treatment. The primary mode of NIV is positive-pressure ventilation, which is basically breathing through a mask that fits over the mouth and nose. Ultimately, more invasive ventilation through a breathing tube down the throat or via a hole in the front of the neck (tracheostomy) may become necessary. Because ventilation ultimately becomes a life-support technology in ALS patients, it can interfere with the ability to die naturally. People with ALS may want to decide at the outset when to withdraw mechanical ventilation, while they have the physical and mental capacity to craft advance directives. Diaphragmatic Pacing The FDA has approved the NeuRx diaphragm pacing system, which uses implanted electrodes to cause the diaphragm to contract and create a breath. This device is also used for people with spinal cord injuries. If severe respiratory failure has not yet occurred, this device allows people to breathe without a ventilator for up to four hours per day. Even though the FDA has approved its use, some healthcare providers question its usefulness for ALS,with some specialists even stating it can be harmful. Percutaneous Endoscopic Gastronomy (PEG) A feeding tube is placed into the stomach and leads out of the body through the abdominal wall. Although studies have failed to show a consistent impact on survival time, it is considered to be a safe nutritional protocol in patients with a functional gastrointestinal system. Because a PEG tube bypasses the mouth and throat, the risk of choking and inhaling food and medications into the lungs is minimized. Liquid tube feedings, medications, and water can all be given by PEG tube. Managing Symptoms of ALS Improving Quality of Life Physical therapists can help a person with ALS to do physical exercise and stretching that strengthens functioning muscles, decreases spasticity, and doesn't overwork their muscles, with the goal of preserving the strength and flexibility they still have. Occupational therapists can help with assistive devices that improve functionality and increase independence, such as ramps, braces, walkers, and wheelchairs. Speech therapists can help teach adaptive strategies to speak more loudly and more clearly. As speech deteriorates, there are adaptive devices like computer-based speech synthesizers using eye tracking technology and even brain-computer interfaces that can control equipment like wheelchairs using only brain activity. Hopeful Research for ALS Many researchers and clinical trials are working on trying to find treatments for ALS. Therapies on the horizon involve stem cells that could help to protect remaining motor neurons, plus medications that may treat possible genetic variations that contribute to ALS. A Word From Verywell Advances have been made in recent years in the support and treatment for ALS. Researchers have also made advances in understanding the disease process of ALS, which will hopefully lead to new treatments. Educating yourself on treatment and care options for ALS can help you understand this difficult and complicated diagnosis. A specialized multidisciplinary treatment team for ALS may be most helpful. Talk to your healthcare provider about what you can do to help the person with ALS live more comfortably, and realize that caregivers will also need a lot of support navigating this difficult illness. Frequently Asked Questions What are ALS symptoms? ALS symptoms in early stages of the disease include:Muscle twitching in the arm, leg, shoulder, or tongueMuscle cramps, as well as tight or stiff musclesMuscle weakness affecting the arm, leg, neck, or diaphragmSlurred and nasal speechDifficulty in chewing and swallowingEarly signs of ALS often appear in one hand or arm. This starts as muscle weakness, but can eventually cause trouble with lifting things or having difficulty using the hands. What is bulbar ALS? Bulbar ALS refers to the disease causing difficulty in speech and swallowing. When these are the first symptoms to appear, it is called "bulbar onset" ALS. What is the typical age of onset of ALS? ALS most commonly appears between the ages of 40 and 70 years old. The average age of onset is 55. However, uncommonly, the disease can begin as early as 20 to 30 years old. Is there treatment for Lou Gehrig's disease? The primary goal of treatment for Lou Gehrig's disease, or ALS, is to improve life expectancy or quality of life. This can involve medication, non-invasive ventilation (assisted breathing using a mask), a feeding tube, physical therapy, and using assistive devices such as braces or a wheelchair. While there is not a cure for ALS, a drug called riluzole is believed to somewhat slow the disease's progression. Do women get ALS? Women can get ALS, but the disease is 20% more common in men. The rate of ALS incidence becomes equal at older ages. Was this page helpful? Thanks for your feedback! Sign up for our Health Tip of the Day newsletter, and receive daily tips that will help you live your healthiest life. Sign Up You're in! Thank you, {{form.email}}, for signing up. There was an error. Please try again. What are your concerns? Other Inaccurate Hard to Understand Submit 13 Sources Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy. Nowicka N, Juranek J, Juranek JK, Wojtkiewicz J. Risk factors and emerging therapies in amyotrophic lateral sclerosis. Int J Mol Sci. 2019;20(11). doi: 10.3390/ijms20112616 Emory University School of Medicine. What is ALS? Johns Hopkins Medicine. ALS- Amyotrophic lateral sclerosis. National Institute of Neurological Disorders and Stroke. Amyotrophic lateral sclerosis information page. Rusina R, Vandenberghe R, Bruffaerts R. Cognitive and behavioral manifestations in ALS: beyond motor system involvement. Diagnostics (Basel). 2021;11(4). doi: 10.3390/diagnostics11040624 ALS Association. Stages of ALS. Oskarsson B, Gendron TF, Staff NP. Amyotrophic lateral sclerosis: An update for 2018. Mayo Clinic Proceedings. 2018;93(11):1617-1628. doi: 10.1016/j.mayocp.2018.04.007 Green JR, Allison KM, Cordella C, et al. Additional evidence for a therapeutic effect of dextromethorphan/quinidine on bulbar motor function in patients with amyotrophic lateral sclerosis: A quantitative speech analysis. Br J Clin Pharmacol. 2018;84(12):2849-2856. doi: 10.1111/bcp.13745 Shoesmith C, Abrahao A, Benstead T, et al. Canadian best practice recommendations for the management of amyotrophic lateral sclerosis. CMAJ. 2020;192(46):E1453-E1468. doi: 10.1503/cmaj.191721 FDA Humanitarian Device Exemption. NeuRx diaphragm pacing system. National Institute of Neurological Disorders and Stroke. Amyotrophic lateral sclerosis (ALS) fact sheet. ALS Association. Who gets ALS? UCSF Health. ALS treatments. Additional Reading Pinto S, Carvalho MD. Breathing new life into treatment advances for respiratory failure in amyotrophic lateral sclerosis patients. Neurodegenerative Disease Management. 2014;4(1):83-102. doi:10.2217/nmt.13.74.