Condition: Amyotrophic Lateral Sclerosis (ALS)

Also called: Lou Gehrig's Disease

What is amyotrophic lateral sclerosis (ALS)?

Amyotrophic lateral sclerosis (ALS), often called Lou Gehrig's disease, is a disease of the nervous system. It attacks nerve cells called motor neurons in the brain and spinal cord. These neurons send messages from your brain and spinal cord to your voluntary muscles. These are the muscles you can control, such as those in your arms and legs.

ALS is a progressive disease, meaning it gets worse over time. As the motor neurons die, they stop sending messages to the muscles. As a result, the muscles become weak, twitch, and waste away.

Who is more likely to develop amyotrophic lateral sclerosis (ALS)?

ALS can happen at any age, but its symptoms most often begin between ages 55 and 75. There are certain factors that may raise your risk of developing ALS:

• Being male
• Being White and not Hispanic
• Having a family member with ALS

What causes amyotrophic lateral sclerosis (ALS)?

ALS can be sporadic or familial:

• Most of the time, ALS is sporadic, meaning that there is no family history of the disease. Researchers don't know what causes this type, but it may be a combination of genetics and environmental factors.
• Familial ALS is much less common. It is inherited (passed down through families).

What are the symptoms of amyotrophic lateral sclerosis (ALS)?

The early symptoms of ALS are often mild. They may begin in one arm or leg or in the muscles used for speaking and swallowing. You may notice:

• Muscle twitches or cramps
• Stiff or weak muscles in the neck, arm, or leg
• Trouble walking, running, or writing
• Slurred speech
• Trouble chewing or swallowing

Over time, weakness spreads to other parts of the body. You gradually lose the ability to move, speak, eat, and breathe on your own. Most people with ALS can still reason, remember, and understand, but some have changes in language, thinking, or behavior. Some may develop a form of dementia known as frontotemporal dementia (FTD-ALS).

How is amyotrophic lateral sclerosis (ALS) diagnosed?

There is no single test for ALS. Since the symptoms are like those of other diseases, ALS can be hard to diagnose, especially in its early stages.

Your health care provider will do a physical exam and a neurological exam and ask about your health history. You may also have tests to rule out other conditions, such as:

• An electromyography (EMG) and nerve conduction studies (NCS) to check how your nerves and muscles are working
• An MRI scan of your brain and spinal cord
• A spinal tap (lumbar puncture)
• Blood and urine tests

How is amyotrophic lateral sclerosis (ALS) treated?

There's no treatment to reverse the damage from ALS. But medicines may slow the loss of function, help relieve symptoms, and sometimes help you live longer.

A team of providers can help you stay active and independent for as long as possible, with treatments such as:

• Physical, occupational, and speech therapy
• Devices to help you communicate
• A feeding tube, if you can't eat safely
• Breathing support, such as a mask used during sleep, and later, a breathing machine

Most people with ALS die from respiratory failure, often within three to five years after symptoms begin. But some people live 10 years or longer.

Support groups and mental health counseling may help you and your family cope with the illness and its impact on your life.

Can amyotrophic lateral sclerosis (ALS) be prevented?

There is no known way to prevent ALS. If ALS runs in your family, talk with your provider about whether genetic counseling and testing may be right for you.

NIH: National Institute of Neurological Disorders and Stroke

Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive disease that affects motor neurons, which are specialized nerve cells that control muscle movement. These nerve cells are found in the spinal cord and the brain. In ALS, motor neurons die (atrophy) over time, leading to muscle weakness, a loss of muscle mass, and an inability to control movement.

There are many different types of ALS; these types are distinguished by their signs and symptoms and their genetic cause or lack of clear genetic association. Most people with ALS have a form of the condition that is described as sporadic, which means it occurs in people with no apparent history of the disorder in their family. People with sporadic ALS usually first develop features of the condition in their late fifties or early sixties. A small proportion of people with ALS, estimated at 5 to 10 percent, have a family history of ALS or a related condition called frontotemporal dementia (FTD), which is a progressive brain disorder that affects personality, behavior, and language. The signs and symptoms of familial ALS typically first appear in one's late forties or early fifties. Rarely, people with familial ALS develop symptoms in childhood or their teenage years. These individuals have a rare form of the disorder known as juvenile ALS.

The first signs and symptoms of ALS may be so subtle that they are overlooked. The earliest symptoms include muscle twitching, cramping, stiffness, or weakness. Affected individuals may develop slurred speech (dysarthria) and, later, difficulty chewing or swallowing (dysphagia). Many people with ALS experience malnutrition because of reduced food intake due to dysphagia and an increase in their body's energy demands (metabolism) due to prolonged illness. Muscles become weaker as the disease progresses, and arms and legs begin to look thinner as muscle tissue atrophies. Individuals with ALS eventually lose muscle strength and the ability to walk. Affected individuals eventually become wheelchair-dependent and increasingly require help with personal care and other activities of daily living. Over time, muscle weakness causes affected individuals to lose the use of their hands and arms. Breathing becomes difficult because the muscles of the respiratory system weaken. Most people with ALS die from respiratory failure within 2 to 10 years after the signs and symptoms of ALS first appear; however, disease progression varies widely among affected individuals.

Approximately 20 percent of individuals with ALS also develop FTD. Changes in personality and behavior may make it difficult for affected individuals to interact with others in a socially appropriate manner. Communication skills worsen as the disease progresses. It is unclear how the development of ALS and FTD are related. Individuals who develop both conditions are diagnosed as having ALS-FTD.

A rare form of ALS that often runs in families is known as ALS-parkinsonism-dementia complex (ALS-PDC). This disorder is characterized by the signs and symptoms of ALS, in addition to a pattern of movement abnormalities known as parkinsonism, and a progressive loss of intellectual function (dementia). Signs of parkinsonism include unusually slow movements (bradykinesia), stiffness, and tremors. Affected members of the same family can have different combinations of signs and symptoms.