What is Primary Lateral Sclerosis

Primary lateral sclerosis is a rare brain disease that causes a gradual loss of the nerve cells (neurons) that control voluntary muscles. Voluntary muscles are the muscles that you can control, such as the muscles in your arms, hands, legs, and face.

In this disease, the muscles gradually become weak and stiff. Movement in the affected parts of the body becomes more difficult. The disease mainly affects the arms and legs. Later, the disease begins to affect muscles in the face.

The effects of primary lateral sclerosis develop slowly over a number of years. The severity of disability can vary from person to person.

There is no cure for this disease, but treatment can help to control symptoms and improve function and movement of the affected parts of the body.

What are the causes?

The cause of this condition is not known. A gene that is passed down through families (inherited) may be the cause in some cases.

What increases the risk?

The following factors may make you more likely to develop this condition:

• Being 40–60 years of age.
• Having a family history of the disease.

What are the signs or symptoms?

Symptoms of this condition usually begin in the legs and then spread to other areas. Signs and symptoms may include:

• Weakness and stiffness in the legs.
• Lack of balance.
• Clumsiness.
• Sudden, involuntary muscle tightening (spasms) in the hands, feet, or legs.
• Slow and stiff movements.
• Dragging of the feet. This can lead to an inability to walk.
• Problems with speech.
• Problems with swallowing.
• Breathing problems.

How is this diagnosed?

Your health care provider may suspect primary lateral sclerosis based on your symptoms. He or she may do a physical exam. It can take a long time to diagnose this disease because it progresses slowly and has symptoms that can be caused by other conditions. Various tests may be done to help confirm the diagnosis and to rule out other disorders. Tests may include:

• Imaging studies of the brain and spinal cord, such as an MRI or a CT scan.
• Nerve conduction studies. These record muscle activity and check how well your nerves send signals to muscles.
• Genetic testing. You may have this test to check for an inherited cause that may require genetic counseling.
• Blood and urine tests to check for other conditions.

How is this treated?

There is no cure for this condition, but treatment can control your symptoms, slow the progression of the disease, and help you maintain your ability to move. Treatment may include:

• Medicines to reduce muscle spasms.
• Physical therapy to help maintain strength and flexibility of muscles and joints.
• Occupational therapy to improve your ability to perform tasks that require use of the affected muscles.
• Medicines to relieve anxiety or depression.
• Speech therapy, if the disease has affected the muscles that control speech.
• Assistive devices to help you move around, such as a cane, walker, braces, power wheelchair, or ramps in your home.

Follow these instructions at home:

Safety

• Make your home safe and easy for you to get around, and take steps to prevent falls. If possible:
  • Remodel a bathroom to make it accessible for a wheelchair.
  • Add a chair or bench to the shower.
  • Install grab bars for your tub, shower, and toilet.
  • Lower beds to make them easier to get in and out.
  • Widen doors and install a wheelchair ramp.
• Use assistive devices to help you move around as told by your health care provider.

General instructions

• Take over-the-counter and prescription medicines only as told by your health care provider.
• Avoid drinking alcohol.
• Do not use any products that contain nicotine or tobacco, such as cigarettes and e-cigarettes. If you need help quitting, ask your health care provider.
• Exercise daily, if you are able. Work with your physical therapist to come up with an exercise program that is safe and effective for you.
• Eat a well-balanced diet to make sure you are getting the nutrition you need.
• Work with your occupational therapist to learn techniques for doing tasks that are difficult because of your condition.
• Keep all follow-up visits as told by your health care provider. This is important.

Contact a health care provider if you:

• Have a fever.
• Have new or worsening symptoms.
• Are struggling with any part of home care.
• Are struggling with anxiety, depression, or lack of support at home.

Get help right away if you:

• Cannot swallow food or liquids.
• Choke on food or liquids.
• Have a fever.
• Have chest pain.
• Have trouble breathing.

Summary

• Primary lateral sclerosis is a rare brain disease that causes a gradual loss of the nerve cells (neurons) that control voluntary muscles.
• In this disease, the muscles gradually become weak and stiff. Movement of the affected parts of the body becomes more difficult.
• The disease mainly affects the arms and legs. Later, the disease begins to affect muscles in the face.
• There is no cure for this condition, but treatment can control your symptoms, slow the progression of the disease, and help you maintain your ability to move.