What is ALS?

What is ALS?

Amyotrophic lateral sclerosis (ALS) is a disease that weakens the muscles we use to move, swallow and breathe. It can, in some cases, also cause changes in behavior and thinking. The effects of ALS grow more severe over time and eventually become fatal. The symptoms and progression of the disease may vary greatly, which can make the disease difficult to diagnose, manage and treat.

The average survival for someone affected by ALS is 2 to 5 years. There is no cure, yet.

There were an estimated 32,000 people living with ALS in the US in 2017. An ALS care team can help people manage their symptoms and offer access to therapeutic treatment and clinical trials.

Who gets ALS?

ALS attacks the nerves that control movement so the muscles weaken until they no longer work. Researchers think that genetics, environment and age by play a role in the development of ALS. The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others.

  • ALS affects people from all communities.
  • Men are affected slightly more frequently than women.
  • It most commonly occurs between 40 and 70 years of age, although the disease can strike at any age.
  • More than 90% of people with ALS have no known family history of the disease. Genetic counseling and testing is available to people living with ALS and their families.
  • U.S. military veterans are twice as likely to develop ALS compared to non-veterans. It's unclear what about military service might trigger the development of ALS.
  • While technically considered a “rare disease,” researchers predict an individual’s lifetime risk of acquiring ALS is about 1 in 300 by the age of 85.
  • The Center for Disease Control and Prevention’s National ALS Registry Dashboard estimates that 5,000-6,000 people are diagnosed each year with ALS.
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    What are the symptoms of ALS?

    While no two people with ALS are alike or will progress the same way, there is a great deal of knowledge, wisdom and experience available from an ALS Care Team, people living with ALS, and caregivers. To learn more about ALS care, visit lesturnerals.org/resources.

    ALS can cause:

    • weakness in arms and legs
    • twitching and rippling sensations under the skin (known as fasciculations)
    • muscle tightness and cramping (which may cause pain)
    • problems with breathing and extreme tiredness
    • difficulties with speech, swallowing and saliva

    ALS may cause:

    • Changes in thinking, reasoning and behavior
    • Crying, laughing or yawning too much (pseudobulbar affect)
    • Frontotemporal dementia (FTD), a rare but more severe form of changes to behavior and thinking

    ALS usually does not affect:

    • Senses – sight, hearing, smell, touch and taste are typically unchanged
    • Bladder – although mobility problems can make it more difficult to get to the toilet in time and some people have a more frequent urge to urinate
    • Bowels – although lack of mobility, nutrition and breathing problems can cause constipation
    • Sexual function – but sexual expression may be more difficult as people living with ALS feel increasingly tired and experience both loss of movement and the emotional impact of dealing with the disease. People living with ALS can continue to enjoy intimacy with their loved ones, though some adaptation will be needed as the disease progresses.

    How is ALS diagnosed?

    • It may take time to receive a diagnosis of ALS, as there is no single, definitive test. Often, tests will be administered to rule out illnesses with similar symptoms. These may include a magnetic resonance imaging (MRI) of the brain or spinal cord, an electromyography (EMG) study of nerve and muscle function, and a variety of blood and urine tests.
    • After reviewing these test results and medical history, and performing a complete neurological exam, a neuromuscular specialist can usually reach a diagnosis.
    • It may take several months of observation and retesting to reach a definitive diagnosis, which should be confirmed via a second opinion from another neuromuscular specialist.

    What is the prognosis of ALS?

    • The average survival for someone affected by ALS is 2 to 5 years. ALS progresses at different rates in each individual and some people live longer.

    Is there treatment? 

    FDA Approved Treatments

    Decisions regarding medication should be made in consultation with your doctor and should be part of a comprehensive treatment approach. There is no cure for ALS, but several therapies have been approved by the FDA to slow or treat symptoms.

    Rilutek was the first FDA-approved medication for the treatment of ALS, approved in 1995. It has been proven to modestly increase lifespan.  It is available as Riluzole (pill form), Tiglutik (liquid form) and  Exservan (oral film form) by prescription.

    Nuedexta was approved by the FDA in 2011 to treat pseudobulbar affect, a symptom that can occur in ALS and other neurological conditions, which is characterized by uncontrollable laughing and crying.

    In 2017, the FDA approved Radicava, an infusion treatment from Mitsubishi Tanabe Pharma America. It is also known as edaravone. According to the FDA, the pivotal clinical trial for Radicava in Japan showed a 33 percent slowing of loss of function in participants as rated on the ALS Functional Rating Scale. In 2022, the FDA approved Radicava ORS (liquid form) which can be taken orally or via feeding tube.

    How should I seek treatment?

    Connecting with an ALS Care Team such as the Lois Insolia ALS Clinic at the Les Turner ALS Center at Northwestern Medicine helps people living with ALS and their families coordinate care in the best way possible. Care provided by clinicians in ALS multidisciplinary centers has been shown to prolong survival and improve quality of life.

    Are there Clinical Trials or Research Studies?

    The Lois Insolia ALS Clinic at the Les Turner ALS Center at Northwestern Medicine is actively involved in multi-center drug trials. Clinical research in ALS at the Les Turner ALS Center at Northwestern Medicine encompasses a broad range of work, including biomarker studies, open label therapeutic studies, natural history studies and clinical trials.

    There are a number of ongoing clinical trials in ALS that have the potential to slow down disease progression and/or manage symptoms. Choosing to participate in a study is an important personal decision. We encourage you to talk with your doctor and family or friends about deciding to join a study. To learn more about ALS clinical research visit Northeast Amyotrophic Lateral Sclerosis Consortium.

    ALS Signal: Clinical Research Dashboard  provides an overview of treatments and supplements that are being tested in the USA and across the world.

    To learn more about living with ALS visit, lesturnerals.org/resources:

    • Speech and communication support
    • Breathing support
    • Diet, nutritional and swallowing support
    • Complementary therapies

    Citations:

    Miller, Robert G., et al. "Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology." Neurology 73.15 (2009): 1218-1226.

    Weisskopf, M. G., et al. "Prospective study of military service and mortality from ALS." Neurology 64.1 (2005): 32-37.

    Martin, Sarah, Ahmad Al Khleifat, and Ammar Al-Chalabi. "What causes amyotrophic lateral sclerosis?." F1000Research 6 (2017).

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