Amyotrophic lateral sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS) – also known as Lou Gehrig’s disease – is a chronic disease that causes weakness in voluntary muscles throughout the body. Voluntary muscles are the ones you have conscious control over.

What is amyotrophic lateral sclerosis (ALS)?

Amyotrophic lateral sclerosis is a neuromuscular disease that causes muscle weakness that gradually gets worse. It causes weakness because it gradually destroys the motor neurons that send messages from the brain to voluntary muscles. As the muscles stop receiving messages sent from motor neurons, they waste away (atrophy) and no longer work.

The senses of sight, touch, hearing, taste and smell aren’t affected by ALS because they get messages from the brain and spinal cord through sensory neurons, not motor neurons. ALS affects only motor neurons.

Movement of the eyes isn’t usually affected by ALS, so both the sense of sight and the ability to look around are usually preserved. Muscles of the bladder are also usually unaffected by amyotrophic lateral sclerosis (ALS) disease.

ALS symptoms

Risk factors for amyotrophic lateral sclerosis (ALS)

Amyotrophic lateral sclerosis is the most common disease that affects motor neurons. About 90% of people who have amyotrophic lateral sclerosis (ALS) have no family history of the disease. About 10% develop what’s called familial ALS, where they’ve inherited a gene known to be related to the disease. Changes in more than a dozen genes are related to familial ALS.

Men are more likely to develop ALS than women, and it’s more common in Whites and non-Hispanics. ALS symptoms are most likely to start among people aged 55-75.

For unknown reasons, military veterans are more likely to acquire ALS.

The cause of ALS is unknown. Researchers are looking for the cause and cure as well as more effective ALS treatments.

Diagnosis of ALS

Getting an early ALS diagnosis is vital because treatments are more effective in the early stages of the disease. However, there is no single diagnostic test.

Your doctor will perform a physical exam and test your reflexes, muscle strength and other responses. They’ll talk to you about your potential ALS symptoms and your medical history. They’ll also order muscle and imaging tests to help with diagnosis. Some of the tests they’ll order include:

• Blood and urine tests.
• Spinal tap (lumbar puncture) to gather cerebrospinal fluid (CSF) for testing.
• Nerve or muscle biopsy to rule out other diseases.
• Electromyography (EMG): Evaluates how well nerves and muscles are functioning. This test can include:
  • Nerve conduction study (NCS): Measures the electrical activity of nerves and muscles by assessing the nerve's ability to send a signal along the nerve or to the muscle.
  • Needle exam: Recording technique that detects electrical activity in muscle fibers using a needle electrode.
• Magnetic resonance imaging (MRI): Uses a magnetic field and radio waves to produce detailed images of the brain and spinal cord.

To diagnose amyotrophic lateral sclerosis, your doctor will need to rule out other diseases with similar signs to ALS symptoms.

ALS treatments

There’s no cure for amyotrophic lateral sclerosis and no treatment to reverse damage to motor neurons. Treatments are aimed at managing ALS symptoms, extending survival and maintaining your quality of life.

Medications & amyotrophic lateral sclerosis

Some ALS medications are aimed at stopping or reducing the amount of damage being done to motor neurons. They work in various ways, such as:

• Decreasing levels of glutamate, which is used to transfer nerve messages
• Slowing functional decline
• Blocking stress signals in cells to help prevent nerve cell death

Other ALS medications are used to manage symptoms like muscle cramps, excessive saliva, pain, depression and sleep disturbances.

New treatments are being researched. Talk to your doctor about the possibility of participating in ongoing clinical trials.

Rehabilitation therapy & ALS

Rehabilitation and physical therapy are important parts of ALS treatment. Therapists work to help people manage balance problems and maintain their range of motion. Physical therapists recommend low impact exercises to help maintain muscle function and strength.

Occupational therapists provide strategies to help with activities of daily living and can suggest assistive devices to help people maintain independence.

Speech therapists help people maintain clear speech as long as possible. They also provide guidance about speech generating devices when they’re needed.

Assistive devices & ALS

Many people who have ALS use assistive devices such as powered wheelchairs, wheelchair ramps, breathing aids, and speech generating computer apps or devices.

Some of these aids can take some time to put in place. It’s recommended to get equipment before you think you’ll need it to avoid a delay when such devices become necessary. Having these aids ahead of time lets you learn to use them when you’re able to clearly communicate about your needs.