What part of the body does ALS affect first?

Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS) is a fatal type of motor neuron disease. It causes progressive degeneration of nerve cells in the spinal cord and brain. It’s often called Lou Gehrig disease after a famous baseball player who died from the disease. ALS is one of the most devastating types of disorders that affect nerve and muscle function.

ALS doesn’t affect the senses (such as seeing or hearing). It also usually doesn’t affect mental functioning. It isn’t contagious. Currently, there is no cure for this disease.

ALS most often affects people between ages 40 and 70. But it can occur at a younger age. It affects people of all races and ethnic groups.

There are 2 main types of ALS:

• Sporadic. This is the most common form of ALS in the U.S., making up 9 in 10 to 19 out of 20 cases. These cases occur randomly, without any known cause. And there is no family history of ALS.
• Familial. This is an inherited form of ALS that affects a small amount of people. But many recent genetic discoveries suggest hereditary forms of ALS are more common than previously thought.

What causes ALS?

Experts don’t know the cause of ALS. More recently, genetic causes are being seen as increasingly important. Research is also looking into possible environmental causes of ALS.

What are the symptoms of ALS?

With ALS, you may first have weakness in a limb that occurs over a few days or, more often, a few weeks. Then a few weeks or months later, weakness develops in another limb. For other people, the first sign of a problem may be slurred speech or trouble swallowing.

As ALS progresses, more and more symptoms are noticed. These are the most common symptoms of ALS:

• Twitching and cramping of muscles, especially those in the hands and feet
• Loss of motor control in the hands and arms
• Trouble using arms and legs
• Tripping and falling
• Dropping things
• Lasting severe tiredness (fatigue)
• Uncontrollable periods of laughing or crying
• Slurred or thick speech and trouble projecting the voice

As the disease progresses, symptoms may include:

• Trouble breathing
• Trouble swallowing
• Paralysis

Some of these symptoms may be caused by other health problems. Always see your healthcare provider for a diagnosis.

How is ALS diagnosed?

There is no specific test to diagnose ALS. Your healthcare provider will consider your health history and symptoms. You will also have tests to rule out other conditions, such as:

• Lab tests. These include blood and urine studies.
• Electrodiagnostic tests, such as electromyography (EMG) and nerve conduction study (NCS). These studies evaluate and diagnose disorders of the nerves, muscles, and motor neurons. Your healthcare provider inserts electrodes into the muscle or places them on the skin overlying a muscle, a nerve, or muscle group to record electrical activity and muscle responses.
• MRI. This procedure uses large magnets, radio waves, and a computer to make detailed images of organs and structures in the body.

In some cases, other tests may be needed due to atypical features of ALS, such as:

• Muscle or nerve biopsy. In this procedure, your healthcare provider removes a sample of tissue or cells from the body and examines it under a microscope.
• Spinal tap (lumbar puncture). In this test, your healthcare provider places a special needle into the lower back, into the area around the spinal cord. There they can measure the pressure in the spinal canal and brain. Your healthcare provider will remove a small amount of cerebrospinal fluid (CSF) and test it for an infection or other problems. CSF is the fluid that bathes the brain and spinal cord.

How is ALS treated?

For most people with ALS, the main treatment may include managing symptoms. This may include physical, occupational, speech, respiratory, and nutritional therapies. Some medicines and heat or whirlpool therapy may help ease muscle cramping. Exercise in moderation may help maintain muscle strength and function.

There is no cure and no proven treatment for ALS. But the FDA has approved the medicines riluzole and endavarone.

These medicines may improve survival or slow physical decline in people with ALS.

Coping with ALS and managing your symptoms is challenging for you, your caregivers, and your healthcare team. But it’s important to know that there are many community resources available for support and assistance.

Researchers are conducting studies to better understand genes that may cause the disease. They are looking at the mechanisms that can trigger motor neurons to degenerate in ALS. And they’re also looking at ways to stop the progress leading to cell death.

What are possible complications of ALS?

There is no cure for ALS. For most people, the disease will progress over 3 to 5 years, making voluntary movements of arms and legs impossible. In other people, the disease can rapidly worsen over a few months or have a very slow course over many years. Each person is affected differently. In time, you will need help with personal care, eating, and mobility. Movement of the diaphragm for breathing is also impaired. You may need a machine (ventilator) for breathing. Most people with ALS die from respiratory failure. People with ALS are at risk of other complications because of the loss of mobility. These include pressure injuries. They are also at risk of losing weight from loss of muscle mass, as well as malnourishment. Muscle stiffness develops from spasticity. This can make mobility worse and cause discomfort. Some people will drool because of lower facial weakness.

Living with ALS

Over time, ALS will lead to disability and death. Your ability to move and breathe independently will be affected, but your intelligence and ability to think is usually not. You and your family will work closely with your healthcare provider to manage symptoms as they develop. Using medicines may prolong your life by a few months, particularly if you have trouble swallowing. Discuss ways to make living spaces more accessible and the use of mobility devices and wheelchairs. It’s very important to talk about end-of-life decisions with your loved ones.

Support groups such as the ALS Association may help you cope with the disease. Talk with your healthcare provider about how you feel about a feeding tube and breathing machine.

Many families find that professional counseling helps them develop coping strategies. Emotional support can also help people manage the complex emotional and physical challenges of this disease. Ask your provider for resources.

When should I call my healthcare provider?

It’s important that you keep your healthcare provider informed about new symptoms so they can advise you on therapies and community resources. Most importantly, call your provider if you start to have trouble breathing.

Key points about ALS

• ALS is a fatal motor neuron disease. It causes progressive degeneration of nerve cells in the spinal cord and brain.
• ALS affects voluntary control of arms and legs and leads to trouble breathing.
• ALS doesn’t affect intelligence, thinking, seeing, or hearing.
• There is no known cure for ALS.
• Treatment of ALS focuses on managing or minimizing symptoms as much as possible.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.
• Before your visit, write down questions you want answered.
• Bring someone with you to help you ask questions and remember what your provider tells you.
• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.
• Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
• Ask if your condition can be treated in other ways.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if you do not take the medicine or have the test or procedure.
• If you have a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your provider if you have questions.

Medical Reviewer: Joseph Campellone MD
Medical Reviewer: Marianne Fraser MSN RN
Medical Reviewer: Raymond Kent Turley BSN MSN RN

© 2000-2022 The StayWell Company, LLC. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional’s instructions.

Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS) is also called Lou Gehrig’s disease. It’s a neuromuscular disorder that causes muscle weakness. ALS symptoms include difficulty talking, swallowing and moving. Eventually, breathing becomes difficult. ALS treatment includes therapies and medications to manage the symptoms and slow the progress of the disease.

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:

• Hearing.
• Sense of taste, smell and touch.
• Vision.

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 andurine 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 spinalfluid test measures different substances in your spinal fluid to help diagnose brain and spinal cord problems.
• Muscle and/or nervebiopsy 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.

• 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.

Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig’s disease or classical motor neuron disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles. In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away, and twitch. Eventually the ability of the brain to start and control voluntary movement is lost. Symptoms are usually first noticed in the arms and hands, legs, or swallowing muscles. Muscle weakness and atrophy occur on both sides of the body. Individuals with ALS lose their strength and the ability to move their arms and legs, and to hold the body upright. When muscles in the diaphragm and chest wall fail to function properly, individuals lose the ability to breathe without ventilatory support. The disease does not affect a person’s ability to see, smell, taste, hear, or recognize touch. Although the disease does not usually impair a person’s mind or personality, several recent studies suggest that some people with ALS may develop cognitive problems involving word fluency, decision-making, and memory. The cause of ALS is not known, and scientists do not yet know why ALS strikes some people and not others.

Treatment

No cure has yet been found for ALS. However, the drug riluzole—the only prescribed drug approved by the Food and Drug Administration (FDA) to treat ALS—prolongs life by 2-3 months but does not relieve symptoms. The FDA has also approved the NeuRx Diaphragm Pacing System, which uses implanted electrodes and a battery pack to cause the diaphragm (breathing muscle) to contract, to help certain individuals who have ALS and breathing problems an average benefit of 16 months before onset of severe respiratory failure. Other treatments are designed to relieve symptoms and improve the quality of life for people with ALS. Drugs are available to help individuals with spasticity, pain, panic attacks, and depression. Physical therapy, occupational therapy, and rehabilitation may help to prevent joint immobility and slow muscle weakness and atrophy. Individuals with ALS may eventually consider forms of mechanical ventilation (respirators).

Prognosis

Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Individuals have increasing problems with moving, swallowing, and speaking or forming words. Eventually people with ALS will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. In later stages of the disease, individuals have difficulty breathing as the muscles of the respiratory system weaken. Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. Most people with ALS die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10 percent of those individuals with ALS survive for 10 or more years.

Research

The National Institute of Neurological Disorders and Stroke (NINDS) conducts research in its laboratories at the National Institutes of Health (NIH) and also supports additional research through grants to major medical institutions across the country. The goals of this research are to find the cause or causes of ALS, understand the mechanisms involved in the progression of the disease, and develop effective treatments.National ALS RegistryBoth the NINDS and the Centers of Disease Control and Prevention (CDC) are committed to studies of disease patterns or risk factors among persons with ALS in order to better understand the causes of ALS, the mechanisms involved in the progression of the disease, and to develop effective treatments. In keeping with this goal, the CDC has launched the National ALS Registry, a program to collect, manage and analyze data about persons with ALS. The Registry includes data from national databases as well as de-identified information provided by persons with ALS. Persons living with ALS who choose to participate can add their information to the Registry by clicking the button below.

View research on this topic.
View articles about this condition.
View studies being conducted about this condition.
View NINDS publications on this topic.

Organizations

ALS Association
Nonprofit voluntary health organization dedicated to the fight against amyotrophic lateral sclerosis. Funds global research and sponsors advocacy programs, a network of chapters, and certified centers and clinics located nationwide.

1275 K Street, N.W.
Suite 250
Washington, DC 20005
advocacy@alsa-national.org
http://www.alsa.org
Tel: Washington
Fax: 202-289-6801

Les Turner ALS Foundation
Supports medical research, patient services, and promotes awareness and education to find the cause(s), effective treatments, and ultimately, a cure for amyotrophic lateral sclerosis.

5550 W. Touhy Avenue
Suite 302
Skokie, IL 60077-3254
info@lesturnerals.org
http://www.lesturnerals.org
Tel: Skokie
Fax: 847-679-9109

Muscular Dystrophy Association
Voluntary health agency that fosters neuromuscular disease research and provides patient care funded almost entirely by individual private contributors. MDA addresses the muscular dystrophies, spinal muscular atrophy, ALS, Charcot-Marie-Tooth disease, myasthenia gravis, Friedreich’s ataxia, metabolic diseases of muscle, and inflammatory diseases of muscle, for a total of more than 40 neuromuscular diseases.

National Office — 222 S. Riverside Plaza
Suite 1500
Chicago, IL 60606
mda@mdausa.org
http://www.mda.org
Tel: Chicago
Fax: 520-529-5300

Project ALS
Not-for-profit organization that raises awareness and significant funds toward effective treatments and a cure for ALS, also known as Lou Gehrig’s disease.

3960 Broadway
Suite 420
New York, NY 10032
info@projectals.org
http://www.projectals.org
Tel: New York
Fax: 212-420-7387

Content Provided By

NINDS Disorders is an index of neurological conditions provided by the National Institute of Neurological Disorders and Stroke. This valuable tool offers detailed descriptions, facts on treatment and prognosis, and patient organization contact information for over 500 identified neurological disorders.