What is Amyotrophic Lateral Sclerosis (ALS)?

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Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND) in the U.K. or Lou Gehrig’s Disease in the U.S., is defined as a rare, progressive neurological disorder that causes nerve damage and muscle weakness. ALS affects and destroys nerve cells, called motor neurons, which are located within the spinal cord and brain and which control muscle movement. The destruction of these nerves causes progressive muscle weakness and eventually leads to paralysis.^[1]

The cause of ALS is not known. Early symptoms include tingling or weakness in arms or legs, muscle cramping, and sometimes changes to the voice. There is no treatment to cure ALS, though there are some medications that slow down the destruction of the nerve cells. Management of this condition aims to relieve the symptoms of ALS/MND and support the person and their family.

Around 20,000 people in the United States have ALS and 6,000 people are diagnosed with the condition each year. The course of ALS/motor neuron disease varies from person to person, but in most people, the condition will progress within three to five years (see FAQs). However, there are famous examples of people living for decades with ALS, such as the physicist Stephen Hawking.[2][3][4] Although the progression of muscle weakness is different from person to person, most people with ALS will eventually die as a result of the condition.

Symptoms of amyotrophic lateral sclerosis (ALS)

Early symptoms of amyotrophic lateral sclerosis can be different for each person. The progression of ALS may also vary from person to person. However, the common factor is progressive weakness and wasting of muscles, and eventual paralysis.

Early symptoms of ALS

Initial signs of amyotrophic lateral sclerosis will vary depending on the person. Common early symptoms of ALS include:^[5][6]

• Muscle weakness and tingling in the arms, legs, or neck
• Muscle twitches in the arms, legs, shoulder or tongue
• Muscle cramps
• Stiff muscles
• Slurred speech or voice becoming more nasal
• Difficulty swallowing or chewing
• Bouts of uncontrollable emotion, such as crying or laughter, caused by pseudobulbar affect (PBA). (See FAQs)

Early symptoms of ALS/motor neuron disease might be noticed as increased clumsiness, such as tripping over, dropping items or having difficulty with fine motor skills such as writing or fastening buttons.

Progression of ALS

How amyotrophic lateral sclerosis progresses will vary from person to person. Most people will experience these symptoms as different muscles become weaker:^[5][6]

• Being unable to walk
• Being unable to stand
• Losing the use of hands and arms
• Having difficulty swallowing or chewing, leading to coughing or choking when eating or drinking
• Having difficulty speaking
• Having difficulty breathing
• Weight loss

Most people with ALS retain their memories, reasoning, and decision-making capabilities. This retained awareness can lead to depression as the physical aspect of ALS progresses. In a small number of cases, a person with ALS may develop a form of dementia.^[7]

When breathing muscles become affected, the person will need support from a ventilator to continue breathing. People with ALS are also at risk of developing aspiration pneumonia, caused by inhaling food or stomach contents.^[8][9]

Good to know: ALS does not affect the senses, such as sight, hearing, and touch. It also generally does not affect the bladder.

Causes of amyotrophic lateral sclerosis (ALS)

Amyotrophic lateral sclerosis occurs when certain types of nerve cells, called neurons, degenerate and die. Motor neurons control muscle actions that can be voluntarily controlled, such as walking, speaking, and breathing. Normally, upper motor neurons in the brain transmit messages to lower motor neurons in the spinal cord, and from there to muscles. Without input from nerves, muscles do not move, and so become weak and wasted. When a person has ALS, both upper and lower motor neurons start to die.^[5][10] It is the involvement of motor neurons that leads to one name for this condition being motor neuron disease.

What causes ALS is not known. Men are slightly more likely to develop ALS than women, and most people develop the condition between the ages of 55 and 75.

Some studies suggest ALS may be up to twice as common in military veterans. This is thought to be because of exposure to environmental toxins such as pesticides or lead. The U.S. Department of Veterans Affairs recognizes ALS as a service-related condition.^[5][11]

Familial ALS

5 to 10 percent of amyotrophic lateral sclerosis cases are genetic. Only one parent needs to carry a gene for ALS/MND. There are thought to be at least 12 genetic mutations that can cause familial ALS.^[5]

Diagnosis of amyotrophic lateral sclerosis (ALS)

Diagnosis of amyotrophic lateral sclerosis is made by evaluating the symptoms and undergoing a physical examination by a neurologist. There is no single diagnostic test for ALS. However, several tests are needed to confirm the diagnosis and rule out other possible causes of symptoms, such as HIV, polio, or West Nile virus.^[5]

There are four criteria required for a diagnosis of amyotrophic lateral sclerosis:^[12]

Degeneration of lower motor neurons, in the spinal cord and brainstem
Degeneration of upper motor neurons, in the brain
Symptoms start in one body part and spread to other parts of the body
Lack of other explanations for symptoms

Tests that are used to diagnose ALS/MND include:^{[5][6][12][13][14]}

• Electromyogram (EMG), which uses needles to measure electrical signals between nerves and muscles
• Nerve conduction study (NCS), which uses electrode patches to measure electrical signals along nerves
• Magnetic resonance imaging (MRI) test, which produces images of the brain and spinal cord
• Lumbar puncture (spinal tap), which uses a needle inserted between two vertebrae to withdraw spinal cord fluid for testing
• Muscle biopsy, which is rarely used and involves removing a small sample of muscle under local anaesthesia for testing
• Blood tests, to exclude other potential causes for symptoms

Treatment for amyotrophic lateral sclerosis (ALS)

There is no cure for amyotrophic lateral sclerosis. Treatment involves managing the progression of ALS and providing as good a quality of life as possible. Recent years have seen advances in the ability to manage ALS symptoms.

Medication

Riluzole is believed to work by reducing, but not reversing, damage to motor neurons. It can delay the need for a tracheostomy (see the section on Breathing support). Riluzole is a tablet that should be taken twice a day. Side effects associated with riluzole include:^[5][15]

• Tiredness
• Dizziness
• Upset stomach and stomach pain
• Diarrhea
• Muscle aches
• Headache

Edaravone is an antioxidant that has been shown to slow the decline of people with ALS, possibly by slowing nerve damage. It is injected intravenously and given initially for the first 14 days of a 28-day cycle, and for the first 10 days in future 28-day cycles. Side effects associated with edaravone include:^[5][16]

• Bruising
• Headache
• Itchy or scaly rash
• Difficulties breathing, wheezing and chest tightness

Edaravone is not available in all countries.

Other medications can help relieve symptoms of ALS such as muscle cramps, drooling, and the pseudobulbar effect.

Breathing support

As ALS progresses and the muscles that control breathing atrophy, there are various interventions that can be used to aid breathing:^[5][17]

Noninvasive ventilation (NIV): uses a mask or nose tube to deliver air into the nose and/or mouth. NIV may initially only be needed at night but, as ALS progresses, can be used all the time.

Assisted coughing: atrophy of the muscles that control coughing increases the risk of dangerous mucus plugs in the airways. An assisted coughing device can help clear the airways, or a breathing technique called breath stacking can help.

Mechanical ventilation: when breathing muscles atrophy further, a machine may be needed to inflate and deflate the lungs. Mechanical ventilation can be provided by a breathing tube through the mouth, or a tracheostomy. In a tracheostomy, a hole is made surgically in the windpipe and a breathing tube is placed through it, which is attached to a ventilator. Modern ventilators are portable and allow the person more independence. After a tracheotomy, although the vocal cords are left in place, the tracheostomy tube means that air does not pass over them, making normal speech difficult. However, there are valves available that modify the airflow in the tracheotomy tube and offer some speech retention.^[18][19]

Speech and physical therapy

Physical therapy can help a person with ALS maintain their mobility for longer. Exercises can include:^[5][17]

• Swimming
• Cycling on a stationary bike
• Walking
• Stretching exercises

Most people with ALS will need a wheelchair for mobility, and, particularly in the later stages, an electric wheelchair may be preferred and allow more independence.

Speech therapy can teach methods of communicating that are louder and clearer and use less energy as the speaking muscles degenerate. Speech synthesizers are an option for later stages of ALS.

Nutritional support

Preventing weight loss is an important aspect of treatment for ALS to help a person maintain strength. Nutritionists can advise on types of food that are easy to swallow and offer a healthy diet. In the later stages of ALS, a feeding tube may be necessary to provide additional nutrition and to avoid the risk of choking.^[5][17]

Amyotrophic lateral sclerosis (ALS) FAQs

Other names for amyotrophic lateral sclerosis

• Lou Gehrig’s disease
• ALS
• Charcot’s disease
• Motor neuron disease
• MND

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1. The ALS Association. “What is ALS?” Accessed July 3, 2018. ↩

2. The ALS Association. “Who Gets ALS?” June 2016. Accessed July 3, 2018. ↩ ↩

3. The New England Journal of Medicine. “Amyotrophic Lateral Sclerosis.” July 2017. Accessed July 3, 2018. ↩

4. Scientific American. “How Has Stephen Hawking Lived Past 70 with ALS?” January 2012. Accessed July 3, 2018. ↩

5. The National Institute of Neurological Disorders and Stroke. “Amyotrophic Lateral Sclerosis (ALS) Fact Sheet.” June 2013. Accessed July 3, 2018. ↩ ↩ ↩ ↩ ↩ ↩ ↩ ↩ ↩ ↩ ↩ ↩

6. The ALS Association. “Symptoms and Diagnosis.” Accessed July 3, 2018. ↩ ↩ ↩

7. Muscular Dystrophy Association. “ALS: Amyotrophic Lateral Sclerosis.” Accessed July 3, 2018. ↩

8. National Organization for Rare Disorders. “Amyotrophic Lateral Sclerosis.” Accessed July 3, 2018. ↩

9. MedlinePlus. “Aspiration pneumonia.” July 2017. Accessed July 3, 2018. ↩

10. The National Institute of Neurological Disorders and Stroke. “Motor Neuron Diseases Fact Sheet.” March 2012. Accessed July 3, 2018. ↩

11. US Department of Veterans Affairs. “VA Secretary Establishes ALS as a Presumptive Compensable Illness.” September 2008. Accessed July 3, 2018. ↩

12. Muscular Dystrophy Association. “Diagnosis.” Accessed July 3, 2018. ↩ ↩

13. Johns Hopkins Medicine. “Electromyography (EMG).” Accessed July 3, 2018. ↩

14. Johns Hopkins Medicine. “Nerve Conduction Studies.” Accessed July 3, 2018. ↩

15. MedlinePlus. “Riluzole.” December 2017. Accessed July 3, 2018. ↩

16. MedlinePlus. “Edaravone Injection.” June 2017. Accessed July 3, 2018. ↩

17. Muscular Dystrophy Association. “Medical Management.” Accessed July 3, 2018. ↩ ↩ ↩

18. National Heart, Lung and Blood Institute. “Tracheostomy.” Accessed July 3, 2018. ↩

19. Johns Hopkins Medicine. ↩

20. ALS Society of Canada, “ALS, MS and MD.” Accessed July 3, 2018. ↩ ↩

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**What is‌ Amyotrophic Lateral Sclerosis (ALS)?**



**Q: What is ALS?**



**A:** Amyotrophic Lateral Sclerosis (ALS), ⁤also ⁤known as Lou Gehrig’s disease, is a progressive ⁢neurodegenerative disease that attacks motor ⁤neurons, the nerve cells ‌responsible for ⁣controlling voluntary muscle movement.



**Q: What are the Symptoms of⁣ ALS?**



**A:** Symptoms typically ⁢include:

– Muscle weakness and atrophy

– Difficulty speaking, swallowing, and⁤ breathing

– Twitching and cramping

– Progressive‌ paralysis



**Q: ‍What Causes ALS?**



**A:** The exact cause of ALS is⁣ unknown, but it is believed to‌ involve a ⁢complex⁤ interplay of genetic, environmental, and lifestyle factors. Approximately 10% of cases​ have a familial genetic basis.



**Q:‌ How is ALS Diagnosed?**



**A:** Diagnosis is based on a combination of:

– Neurological examination

– Electromyography (EMG) to measure electrical activity in muscles

– Imaging ‌studies like MRI ⁣to rule out other conditions



**Q: Is ALS Curable?**



**A:** Currently, ALS is incurable, ​but treatments can slow⁣ disease progression​ and improve⁢ quality of life.



**Q: What are⁣ the ⁣Treatment Options for ALS?**



**A:** Treatments focus ⁣on symptom ⁤management and include:

-⁢ Medication like riluzole and edaravone

– Physical and occupational therapy

– Speech ⁤and swallowing therapy

– Respiratory support

– Palliative care



**Q: What‍ is the Prognosis for ALS?**



**A:** The‍ average life expectancy after diagnosis is 2-5 years, but⁢ some people‍ live longer. Age of ⁢onset, disease type,​ and access to proper care can influence prognosis.



**Q: How is ALS Researched?**



**A:** Research into ALS aims to:

– Identify genetic and environmental risk factors

– Understand disease ⁤mechanisms

– Develop new therapies and treatments



**Additional Keywords:**



* Motor neuron⁢ disease

* Lou Gehrig’s disease

* Progressive paralysis

* Neurological ⁢disorder

* Neurodegenerative disease