Amyotrophic Lateral Sclerosis

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Introduction

What is amyotrophic lateral sclerosis?

Amyotrophic lateral sclerosis (ALS), also called Lou Gehrig’s disease, is a relentlessly progressive, incurable disease of the nerve cells that control voluntary muscle movement. ALS causes muscle weakness and leads to severe disability. In a small percentage of cases, the disease is inherited, but in most cases, the cause of the disease is not known. About five out of every 100,000 people will develop ALS, usually after age 50. At age 65 or younger males predominate – over 65 both sexes are equally affected. (Source: PubMed).

Early stages of ALS are usually marked by twitching, cramping, stiffness, weakness, or difficulty chewing. As the disease progresses, muscle control decreases, leading to muscle contractions, drooling, dropping head, increased weakness, difficulty performing daily activities, and difficulty breathing. Late stages of the disease are marked by complete paralysis; however mental capacity remains normal. ALS does not affect your ability to feel, see or sense.

The drug riluzole is used to slow the progression of the disease, and anti-epileptic drugs can help control muscle spasms. Physical therapy can help prolong your ability to perform daily activities. In addition, social workers, support groups and counseling, and home hospice care can support you and your family as you deal with the serious impacts of the disease.

Seek immediate medical care (call 911) for serious symptoms of ALS, such as difficulty breathing or choking.

Seek prompt medical care if you experience any loss of muscle function that may be related to ALS or if you have a family history of ALS, as early diagnosis is important for slowing the progression of the disease. It is also important to seek medical care if you have been diagnosed with ALS and your symptoms worsen or you experience new symptoms.

Symptoms

What are the symptoms of ALS?

Symptoms of amyotrophic lateral sclerosis (ALS) are related to problems with communication between your nerves and muscles. ALS starts out with mild symptoms of muscle weakness, stiffness, difficulty chewing, or voice changes. As the disease progresses over the course of three to five years, worse symptoms develop, including difficulty moving, drooling, head drop, severe muscle cramps and contractions, and increased weakness. The late stages of ALS are marked by paralysis and breathing difficulties. While the ability to move is impaired, the ability to reason and the senses (sight, hearing, touch, taste and smell) remain normal.

Early symptoms of ALS

You may experience ALS symptoms daily or just once in a while. In the early stages of ALS, symptoms may be subtle:

  • Changes in voice
  • Difficulty chewing or swallowing
  • Muscle stiffness or cramps
  • Muscle twitching or spasms
  • Weakness (loss of strength)

Later symptoms of ALS

As ALS progresses over the course of three to five years, symptoms become more persistent and severe. Later-stage symptoms of ALS include:

  • Balance problems, difficulty walking, and falls
  • Difficulty breathing
  • Difficulty swallowing
  • Exaggerated reflexes (hyperreflexia)
  • Gradual difficulty walking and speaking
  • Head drop
  • Impaired balance and coordination
  • Increased weakness (loss of strength)
  • Loss of muscle coordination
  • Muscle twitching or spasms
  • Paralysis
  • Unexplained weight loss

Serious symptoms that might indicate a life-threatening condition

ALS is a life-threatening condition. Seek immediate medical care (call 911) if you, or someone you are with, have any of these life-threatening symptoms including:

  • Paralysis or inability to move a body part

  • Respiratory or breathing problems, such as shortness of breath, difficulty breathing or inability to breathe, labored breathing, wheezing, or choking

Causes

What causes ALS?

Although approximately one-tenth of all amyotrophic lateral sclerosis (ALS) cases are understood to be genetic in origin (run in families), it remains unclear what causes the majority of ALS cases. Currently, researchers are investigating factors that may be related to the development of ALS, including abnormally high levels of glutamate (chemical that passes messages between nerves), and environmental factors that may trigger the development of the disease, such as infections, toxic substances, and dietary deficiencies.
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001708/

It is also possible that the body’s own immune response may play a role in the development of ALS. Some researchers believe that the immune system may attack the nerves that control muscles, leading to a loss of muscle control. Currently, not enough evidence is available to indicate clearly the cause of non-hereditary ALS.

What are the risk factors for ALS?

While the risk factors for amyotrophic lateral sclerosis (ALS) are often not known, in approximately 10% of cases there are identifiable risk factors for disease development. Not all people with risk factors will get ALS. Risk factors for ALS include:

  • Age over 50 years

  • Family history of ALS

  • Male gender below age 65

  • Mutation in the SOD1 gene, which helps protect your body’s cells from damage

Treatments

How is ALS treated?

While there is no cure for amyotrophic lateral sclerosis (ALS), drug treatment can help reduce the severity of symptoms. One drug, riluzole (Rilutek), may help slow damage to the nerves that control muscles. Other treatments and medical support, such as physical therapy, speech therapy, assisted breathing apparatuses, and home hospice care, are aimed at helping the patient and family cope with the patient's progressive lack of muscle control.

Medications for ALS

With the exception of riluzole, which may slow the progression of ALS, drugs used to treat ALS are aimed at addressing the symptoms. Drugs frequently prescribed for ALS include:

  • Antidepressants to help treat depression and anxiety, if they develop

  • Drugs to control muscle spasms, such as baclofen (Kemstro, Gablofen) or diazepam (Valium)

  • Drugs to control the production of saliva and prevent choking, such as amitriptyline (Elavil)

  • Medications to control pain

  • Riluzole (Rilutek), which slows damage to the nerves that communicate with voluntary muscles

  • Stimulants to control fatigue

Other therapy for ALS

Many people with ALS find that physical, emotional, or other therapy helps them to cope with the symptoms of ALS and may extend the time that they are able to perform normal activities. Therapy for ALS includes:

  • Feeding tubes to prevent weight loss and promote proper nutrition, especially in late stages of the disease

  • Home health care or hospice care to assist with daily activities

  • Nutritional support to prevent weight loss

  • Occupational therapy to assist patients in continuing to work and to perform daily functions

  • Physical therapy to improve the ability to use muscles

  • Speech therapy to assist patients who develop difficulties talking

  • Support groups or counseling to help patients and families deal with the emotional consequences of ALS

  • Ventilation devices to assist in breathing

Complementary treatments

Some complementary treatments may help some people to better deal with amyotrophic lateral sclerosis (ALS) and its treatments. These treatments, sometimes referred to as alternative therapies, are used in conjunction with traditional medical treatments. Complementary treatments are not meant to substitute for traditional medical care. Be sure to notify your doctor if you are consuming nutritional supplements or homeopathic (nonprescription) remedies as they may interact with the prescribed medical therapy.

Complementary treatments may include:

  • Acupuncture

  • Massage therapy

  • Nutritional dietary supplements, herbal remedies, tea beverages, and similar products

  • Yoga

What are the potential complications of ALS?

Early diagnosis of amyotrophic lateral sclerosis (ALS) is important for reducing the severity of complications and slowing the progression of the disease. Complications of ALS are serious and life threatening. You can help minimize your risk of some complications and slow disease progression by following the treatment plan you and your health care professional design specifically for you. Complications of ALS include:

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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2018 Nov 12
  1. ALS (amyotrophic lateral sclerosis) fact sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_ALS.htm.
  2. Amyotrophic lateral sclerosis. PubMed Health, a service of the NLM from the NIH. http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001708/.
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