Health Library

Primary lateral sclerosis (PLS) is a rare brain disease that slowly damages the nerve cells that help you move your muscles. These are the muscles you can control, like the ones in your arms, hands, legs, and face.

PLS causes your muscles to get weak and stiff over time. It mainly affects the arms and legs. Later, the disease can affect your face.

PLS slowly gets worse over many years. How much it affects someone can be different for each person.

What are the causes?

The cause of PLS isn't known. In some people, it may be a gene that's passed down in families (inherited).

What increases the risk?

You may be more likely to get PLS if:

1.   You are between 40 and 60 years old.

2.   Someone in your family has the disease.

What are the signs or symptoms?

Symptoms usually start in the legs and then spread to arms, hands, and body. Early symptoms may include:

1. Weak and stiff legs.
2. Trouble keeping your balance.
3. Feeling clumsy.
4. Sudden muscle tightening (spasms) in the hands, feet, or legs.
5. Slowed movements.

Other symptoms may include:

1. Dragging your feet. This can lead to not being able to walk.
2. Problems talking clearly.
3. Problems with swallowing.
4. Trouble breathing.

How is this diagnosed?

PLS is diagnosed based on your symptoms and a physical exam. It can take a long time to diagnose PLS because it develops slowly and looks like other diseases.

You may have many tests to confirm the diagnosis and to rule out other conditions. Tests may include:

1. An MRI or a CT scan of the brain and spine.
2. An electromyogram (EMG) to check how your nerves and muscles work.
3. Genetic testing to check if PLS is inherited.
4. Blood and urine tests to check for other conditions.

How is this treated?

There's no cure, but treatments can help manage symptoms and improve quality of life.

You may need:

1. Medicines to help with muscle spasms.
2. Physical therapy to keep your strength and flexibility in your muscles and joints.
3. Occupational therapy to help you do daily tasks, like dressing and bathing.
4. Speech therapy to help you with talking.
5. Assistive devices to help you move around, such as a cane, walker, braces, or wheelchair.

Follow these instructions at home:

Safety

Make your home safe and easy to move around in. To prevent falls:

1. Remove throw rugs and other things you can trip on.
2. Lower your bed to make it easier to get in and out.
3. Put a chair or bench in the shower.
4. Add grab bars in the tub, shower, and near the toilet.
5. Make the bathroom accessible for a wheelchair.
6. Widen doorways and add a wheelchair ramp.
7. Use assistive devices to help you move around as told.

Have a safety plan for emergencies, such as a safety alert system to get help quickly.

Activity

Exercise every day if you can. A physical therapist can make an exercise program that includes:

1. Stretching and range-of-motion exercises.
2. Aerobic exercises, such as swimming or walking. These exercises can help strengthen muscles.

Work with your occupational therapist to learn easier ways of doing daily tasks.

General instructions

1. Take your medicines only as told.
2. Avoid drinking alcohol.
3. Do not smoke, vape, or use nicotine or tobacco.
4. Eat healthy foods to make sure you're getting the nutrition you need.
5. Make sure you have help and support at home. Ask your provider what you may need as the disease gets worse.
6. If you feel anxious or depressed, work with a mental health provider.
7. Keep all follow-up visits to check your condition and see if treatment is helping.

Contact a health care provider if:

1. You have new symptoms.
2. Your symptoms get worse.
3. You're having trouble with your care at home.
4. You feel anxious or depressed.
5. You can't swallow food or drinks.
6. You choke on food or drinks.

Get help right away if:

You have trouble breathing.

These symptoms may be an emergency. Do not wait to see if the symptoms will go away.

Do not drive yourself to the hospital.

This information is not intended to replace advice given to you by your health care provider. Make sure you discuss any questions you have with your health care provider.