Amyotrophic Lateral Sclerosis (ALS)

Overview

What is amyotrophic lateral sclerosis (ALS)?

Amyotrophic lateral sclerosis, or ALS, is a type of motor neuron disease. It’s also known as Lou Gehrig’s disease. ALS affects motor neurons — nerves that control your voluntary muscles. Voluntary muscles are the ones you use for actions like chewing, talking and moving your arms and legs. ALS is progressive, meaning that the symptoms get worse over time. If you have ALS, your muscles begin to atrophy or waste away. Your muscles get weaker over time, making it difficult to walk, talk, swallow and eventually breathe. Most people with ALS die of respiratory failure when their lungs can’t get enough oxygen to the blood.

Although there’s no cure for ALS, the treatments are constantly improving. The right combination of treatments can slow the progression of the disease and help people with ALS live a good quality of life.

Why is ALS called Lou Gehrig’s disease?

Lou Gehrig was a famous baseball player in the 1920s and 1930s. ALS ended his career. He died of the disease a few years after getting diagnosed.

How does ALS affect your body?

As your motor neurons (nerve cells) continue to decline, they can’t send signals to your muscles. The two types of motor neurons are:

  • Upper motor neurons, the motor nerve cells in your brain and spinal cord. Their job is to send signals to lower motor neurons.
  • Lower motor neurons, the motor nerve cells in your brain stem (lower part of your brain) and spinal cord. They receive instructions from the upper motor neurons. They then send messages to your muscles telling them to move.

Most often, ALS affects both upper and lower motor neurons.

You may have different symptoms if mostly upper or mostly lower motor neurons are affected. Upper motor neuron disorders cause muscle stiffening. Lower motor neuron diseases cause a loss of muscle tone. Both upper and lower motor neuron problems cause weakness.

How does ALS affect your breathing?

ALS can make breathing difficult or ineffective. That’s because it affects the muscles that move your chest and lungs. These muscles include the diaphragm, which separates your chest from your belly. Other breathing muscles affected include those between your ribs.

Pulmonary (breathing) problems you can get with ALS include:

  • Shortness of breath, even during rest.
  • Weak cough.
  • Difficulty clearing your throat and lungs.
  • Extra saliva.
  • Inability to lie flat in bed.
  • Repeated chest infections and pneumonia.
  • Respiratory failure.

Does ALS affect other abilities like thinking?

ALS doesn’t affect your:

It also usually doesn’t affect your sexual, bowel or bladder functions. Memory problems develop in about half of patients diagnosed with ALS and are often mild. About 10% of patients develop dementia.

What are the types of motor neuron diseases?

Healthcare providers use a system to classify types of motor neuron diseases:

  • Classical ALS, the most common type, affects more than two-thirds of people with ALS. Both the upper and lower motor neurons decline.
  • Progressive bulbar palsy (PBP) affects about 25% of people with ALS. It starts with difficulty speaking, chewing and swallowing caused by a breakdown of the upper and lower motor neurons to the mouth and throat. This condition will sometimes worsen to affect the rest of your body, in which case the condition is called bulbar-onset ALS.
  • Progressive muscular atrophy (PMA) affects only the lower motor neurons.
  • Primary lateral sclerosis is the rarest form of ALS. Only the upper motor neurons decline.

How do sporadic ALS and familial ALS differ?

Healthcare providers also divide ALS into two categories, based on where the disease comes from:

  • Sporadic ALS: About 90% to 95% of ALS cases are sporadic. This means the disease happens randomly. It’s not tied to any clear risk factor or family history of the disease.
  • Familial ALS: Also called genetic ALS, it affects about 5% to 10% of people with ALS. It happens when you inherit the disease from one or both of your parents. The disease runs in families and gets passed down through genetic mutations or changes.

Who gets ALS?

Worldwide, around one to two people per 100,000 get diagnosed with ALS every year.

Risk factors for ALS include:

  • Age: You’re most likely to develop symptoms between ages 55 and 75.
  • Race and ethnicity: White people and non-Hispanics are most likely to get ALS.
  • Sex: Earlier in life, men are at higher risk than women, though researchers aren’t entirely sure why. But as people get older, the risk is about the same.

Military veterans may also be at higher risk. Researchers are not sure why this is. It may be because they got exposed to lead, pesticides and other toxins during their service.

How common is ALS?

The Centers for Disease Control and Prevention (CDC) estimates that around 12,000 to 15,000 people in the United States have ALS. Every year, about 5,000 people receive an ALS diagnosis.

Symptoms and Causes

What causes ALS?

Researchers do not know what causes ALS. They believe it’s a combination of factors:

  • Genetics: Mutations, or changes, in certain genes may lead to motor neuron breakdown.
  • Environment: Getting exposed to certain toxic substances, viruses or physical trauma may cause ALS.

What are the symptoms of ALS?

At first, you may notice muscle weakness or stiffness:

  • Limb onset ALS is when the symptoms start in your legs or arms. For example, you may have hand weakness. Buttoning a shirt or writing might be difficult. Or you may notice leg symptoms. You may have trouble walking or frequently trip.
  • Bulbar onset is when the symptoms start with your speech or swallowing.

No matter where the symptoms begin, they soon spread to other parts of your body. As ALS progresses, symptoms include:

  • Muscle cramps and twitching, especially in the hands and feet.
  • Difficulty using your arms and legs.
  • Thick speech and difficulty projecting your voice.
  • Weakness and fatigue.
  • Weight loss.

When ALS gets more severe, symptoms can include:

  • Shortness of breath.
  • Difficulty breathing, chewing and swallowing.
  • Inability to stand or walk independently.
  • Weight loss, since people with ALS burn calories at a faster rate.
  • Depression and anxiety, as people become aware of what’s happening to them.

What are fasciculations?

Fasciculations are muscle twitches, a common ALS symptom. While they’re not painful, they can be bothersome and disrupt your sleep. Fasciculations can also be seen in other conditions and don't always mean that you have ALS.

Diagnosis and Tests

How is ALS diagnosed?

Your healthcare provider will do a physical exam and ask you about your medical history. You’ll also need several tests to confirm the diagnosis:

  • Blood and urine tests.
  • A neurological examination to test your reflexes and other responses.
  • Electromyogram (EMG) to measure the electrical activity of your nerves and muscles.
  • A nerve conduction study to test your nerves’ ability to send a signal.
  • Magnetic Resonance Imaging (MRI) to look at your brain or spine for areas of damage.

Other tests can’t diagnose ALS but can help rule out different diseases that may cause similar symptoms:

  • A spinal fluid test measures different substances in your spinal fluid to help diagnose brain and spinal cord problems.
  • Muscle and/or nerve biopsy takes a tissue sample of your muscles or nerves for examination.

If I was diagnosed with ALS, should I get a second opinion?

Most healthcare providers recommend a second opinion after an initial ALS diagnosis. Several conditions have similar symptoms to ALS. And many of those are treatable. It’s important to get an accurate diagnosis, so you get the treatment you need.

Management and Treatment

Who should be on an ALS care team?

People with ALS get the best care from a team of specialists from many fields of medicine. This team works together to help you manage symptoms, avoid complications and live as best you can with ALS. This team may include:

  • Doctors.
  • Speech, occupational and physical therapists.
  • Pharmacists.
  • Nutritionists.
  • Respiratory therapists.
  • Psychologists.
  • Social workers.
  • Home care and hospice nurses.

How is ALS treated?

There is no cure for ALS. But treatment can slow the progression of the disease. The right treatment plan can help you stay comfortable and independent for as long as possible.

Treatments include:

  • Medications to relieve muscle cramps, extra saliva and other symptoms.
  • Physical therapy to help you stay mobile. It can ease discomfort from stiff muscles, cramps and fluid retention.
  • Nutritional counseling ensures you eat a healthy, balanced diet. A nutritionist can also recommend other food options when swallowing becomes difficult.
  • Speech therapy provides strategies for safer swallowing and communication training help you maintain verbal communication for as long as possible. You may also learn nonverbal communication techniques.
  • Assistive devices, including splints, braces, grab bars and reach devices, help you stay independent. You use these devices to get dressed, eat, use the toilet and bathe.
  • Special equipment such as wheelchairs and electric beds to help you function independently.

How can medications treat ALS?

The U.S. Food and Drug Administration has approved two drugs to treat ALS:

  • Riluzole may help reduce damage to the motor neurons. It may extend survival by a few months, but it can’t reverse damage that's already occurred.
  • Edaravone can slow the decline in someone’s functioning.

Other medications can help manage ALS symptoms. Drugs can help with muscle cramps and stiffness and extra saliva. There are also drugs to help with pain, depression, sleep problems and constipation.

How can physical therapy treat ALS?

The goal of physical therapy for ALS is to help you remain independent and safe. Gentle aerobic exercise, such as walking or swimming, can strengthen muscles and improve heart health. These exercises can also help you feel more energetic and less depressed.

Physical therapists can create an exercise plan that helps you feel your best without overworking your muscles.

How can occupational therapy treat ALS?

Occupational therapists can teach you techniques and strategies for moving through your day. They can also help figure out what devices will help you most. For example, wheelchairs, braces or walkers can help you move without feeling exhausted.

How can speech therapy treat ALS?

Speech therapy can help you learn new ways to speak loudly and clearly. As ALS progresses, you may find it difficult to talk. Speech therapists can help you maintain your communication skills. They can also teach you ways to communicate nonverbally. Even if you still have verbal abilities, nonverbal communication can help you conserve energy.

A speech-language pathologist can also:

  • Assess your swallowing ability and teach you techniques for making adjustments.
  • Recommend the most helpful communication technologies.
  • Treat any type of speech, language and communication issue.

What is voice banking?

Some people with ALS choose to do voice banking. While you can still speak, you record your voice in a device called a speech synthesizer. If you can’t speak later on, you can use the speech synthesizer to communicate.

How can nutritional support treat ALS?

People with ALS lose weight quickly. Getting the right nutrition helps you keep your strength. Nutritionists create a meal plan that:

  • Avoids foods that are hard to swallow.
  • Provides the right amount of calories, fiber and fluid.

At some point, you may need a feeding tube to get the nutrition you need. A feeding tube also reduces the risk of choking and pneumonia. These complications come from accidentally inhaling liquid or food in your lungs.

Will I need breathing support?

As the disease progresses, you may find it hard to breathe. You may benefit from noninvasive ventilation (NIV). You get NIV through a mask that you wear over your nose and mouth.

Eventually, you may need mechanical ventilation, which means getting put on a respirator. This machine helps you breathe. It inflates and deflates your lungs. You’ll discuss this option with your care team and loved ones when it’s time to decide.

Prevention

Can ALS be prevented?

There’s no proven way to prevent ALS. If you have ALS, you can take part in clinical trials and the National ALS Registry. Doing so can help further understanding of the disease. As researchers gain more knowledge about ALS, they can learn more about causes and risk factors. This information may lead to prevention methods and better treatments.

Outlook / Prognosis

What is the outlook for people with ALS?

A person’s prognosis depends on how quickly the symptoms progress. Typically, people with ALS live for about three to five years after diagnosis. But others can live for 10 years or even more.

Living With

What are communication tips for people with ALS?

People with ALS can use these strategies to communicate effectively:

Plan ahead:

  • Ask your speech-language pathologist which exercises are right for you.
  • Set aside a rest period before a conversation or phone call. Being tired affects your speaking ability.

Choose an environment that is:

  • Quiet, since it can be tiring to try and talk over TV or other loud noises.
  • Well-lit to enhance face-to-face communication.

When you’re talking:

  • Choose a comfortable posture and position, especially if you’re having a long or stressful conversation.
  • Establish the topic before speaking.
  • Face your listener. Look at them while you talk.
  • Over articulate your speech. Prolong the vowels and exaggerate the consonants to be as clear as possible.
  • Speak slowly.
  • Use short phrases. Try saying one or two words or syllables per breath.
  • Use telegraphic speech, leaving out unnecessary words.

Consider using devices:

  • An amplifier helps if your voice is low or soft.
  • An electrolarynx or respiratory tube provides an alternative air source if you want to communicate while you’re on a respirator.

If people are having trouble understanding you, try to:

  • Carry pen and paper with you as a backup, so you can write down what you want to say.
  • Use an alphabet board if writing is difficult. Point to the first letter of words you want to say.
  • Spell words out loud or on the alphabet board if people don’t understand you.

How can people with ALS communicate nonverbally?

Nonverbal communication is also called augmentative and alternative communication or AAC. It’s a way of communicating without spoken words. People with ALS can use these techniques to communicate:

  • Expressions and gestures.
  • Residual speech or making the best use of your remaining speaking ability.

Nonverbal communication has some benefits. It can help you communicate better because it:

  • Allows you to be more relaxed.
  • Reduces frustration and stress of being unable to communicate.
  • Relieves the pressure to speak.

What devices can help people with ALS communicate more clearly?

Your care team can help you find the right assistive device or devices. Options include:

  • Amplification can increase your volume and decrease voice fatigue.
  • Low technology devices include notebooks and language boards. They can help you communicate without speaking.
  • High technology devices include electronic speech enhancers and communication devices. These computers come with voice synthesizers to help you speak.
  • A palatal lift is like a dental retainer. It lifts your soft palate and stops air from escaping out of your nose while you’re speaking.
  • TTY telephone relay system is a telephone with a keyboard. You type what you want to say, and a relay operator reads it to the listener. You can type the entire message. Or you can just type the words that the listener doesn’t understand.

How can friends and family communicate better with a person who has ALS?

If your friend or loved one has difficulty speaking, you can:

  • Ask questions that require a “yes” or “no” answer.
  • Ask them to repeat what they said, to speak slower or to spell out words you didn’t understand.
  • Repeat part of the sentence that you understood. That way, they only need to repeat whatever you didn’t understand.
  • Talk to them face-to-face. Look at them while they talk.

What exercises can help people with ALS?

Staying active can help with muscle strength and overall well-being. There are several approaches you can take:

  • Aerobic exercises increase your heart and breathing rates and improve heart health. They also help your muscles work better and increase endurance. Exercise in moderation. If you get fatigued, you may make your muscles weaker.
  • Stretching exercises can relieve muscle cramping. Do them every day to prevent pain and stiffness.
  • Range of motion exercises (ROM) can help your joints move fully. Do these every day to prevent pain and stiffness.

Avoid strengthening exercises, such as exercising with weights. They won’t strengthen weak muscles, and they could even make your muscles weaker.

What devices can help people with ALS move more easily?

Devices that can make it easier to move and walk include:

  • Ankle-foot orthosis (AFO), a brace to stabilize your ankle.
  • Cane.
  • Neck brace to help hold your head up.
  • Walker.
  • Wheelchair.

How can I improve my breathing?

Make sure you’re up to date on your vaccines, including flu vaccine and Pneumovax® (pneumonia vaccine). Your healthcare provider may recommend pulmonary therapy to help you breathe easier. Your therapy team will monitor your respiratory muscles to diagnose early signs of breathing trouble. The team can also provide breathing assistive devices, such as:

  • In-Exsufflator, a machine that helps clear mucus from your lungs.
  • Noninvasive ventilation.
  • Suction machine to clear mouth and throat secretions.

Other ways to improve your breathing include:

  • Avoid large meals since they can prevent your diaphragm from fully expanding.
  • Don’t lie down right after eating.
  • Eat a healthy diet.
  • When you sleep, elevate your head with pillows, so your abdominal organs aren’t squeezed next to your diaphragm.

What should I ask my healthcare provider?

If you received an ALS diagnosis, ask your doctor:

  • What treatments are available?
  • What medications can help?
  • How can I take care of myself?
  • What types of therapy will I need?
  • What steps can I take now to slow the progression of the disease?

A note from Cleveland Clinic

Amyotrophic lateral sclerosis (ALS), or Lou Gehrig’s disease, causes muscle weakness that worsens over time. ALS symptoms include difficulty with muscle control, such as during writing or chewing. Eventually, people with ALS lose the ability to walk, swallow and even breathe. But ALS treatments such as medication and therapy can help people with ALS live as long and independently as possible. Talk to a healthcare provider if you notice unusual muscle problems. Maybe you’ve started tripping more often or have trouble buttoning your shirts. A provider can help you get the diagnosis and treatment you need to feel your best.

Last reviewed by a Cleveland Clinic medical professional on 06/15/2021.

References

  • ALS Association. . Accessed 7/23/2021.Understanding ALS (https://www.als.org/understanding-als)
  • Centers for Disease Control and Prevention. ? Accessed 7/23/2021.Amyotrophic Lateral Sclerosis. What Is ALS (https://www.cdc.gov/als/WhatisALS.html)
  • Muscular Dystrophy Association (MDA). . Accessed 7/23/2021. Amyotrophic Lateral Sclerosis (ALS) (https://www.mda.org/disease/amyotrophic-lateral-sclerosis)
  • National Institute of Neurological Disorders and Stroke. . Accessed 7/23/2021.Amyotrophic Lateral Sclerosis (ALS) Fact Sheet (https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet)

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