Multiple System Atrophy (MSA)

What is Multiple System Atrophy (MSA)

Multiple system atrophy is a rare disease of the brain and spinal cord (central nervous system) that causes a loss of nerve cells. Atrophy means loss of function. This condition used to be called Shy–Drager syndrome.

Multiple system atrophy affects one or both of the following areas of the central nervous system:

• Autonomic nervous system, which controls functions such as blood pressure, heart rate, and bladder function.
• Motor system, which controls balance and muscle movements.

Symptoms tend to get worse over time. There is no cure, but treatment may help control some of the symptoms.

What are the causes?

The cause of MSA is not known.

What are the signs or symptoms?

Symptoms of this condition depend on which part of your nervous system is affected. You may have symptoms that mainly affect either your autonomic or motor system, or you might have a combination of symptoms that affect both.

Symptoms of autonomic nervous system disease include:

• Low blood pressure when standing (orthostatic hypotension). This can cause dizziness or fainting.
• Bladder control problems (incontinence).
• Difficulty passing stool (constipation).
• Difficulty getting an erection (erectile dysfunction).
• Dry mouth and skin.
• Irregular heartbeat.

Symptoms of motor system disease include:

• Rigid muscles.
• Slowed movement.
• Poor balance.
• Trembling or tremor.
• Clumsiness.
• Trouble speaking.
• Double vision.
• Trouble chewing and swallowing.
• Trouble breathing.

How is this diagnosed?

At first, MSA can seem like other nervous system disorders. Because of this, it may take a long time to get a diagnosis of MSA. There is no single test that can be used to diagnose this condition. You may be referred to a specialist in nervous system diseases (neurologist). A neurologist may diagnose MSA by doing a physical and neurological exam and by evaluating your symptoms that get worse over time.

Imaging studies of the brain may also be done, including:

• MRI.
• PET scan.
• DaTscan. This is a test that uses a radioactive substance to see specific cells in the brain.

How is this treated?

No treatments can cure or slow the progression of MSA. The goal of treatment is to manage symptoms. Treatment may include:

• Medicine to improve symptoms that affect your motor system.
• Medicine to support blood pressure control.
• Medicine to relieve constipation and control incontinence.
• A feeding tube if swallowing becomes difficult.
• Physical therapy to keep muscles healthy for as long as possible.
• A walker, wheelchair, or other assistive devices if walking becomes difficult.

Follow these instructions at home:

Eating and drinking

• Increase the amount of fiber in your diet to help prevent constipation.
• Increase the amount of salt in your diet as directed by your health care provider. This can help prevent orthostatic hypotension.
• Drink more fluid if your health care provider recommends. This will help with both constipation and low blood pressure.
• Do not drink alcohol.

Lifestyle

• Sleep with the head of your bed slightly raised. This may reduce orthostatic hypotension in the morning.
• Talk with your health care provider about using compression stockings. This might help with your blood pressure.
• Stay as active as you can. Ask your health care provider to recommend a safe level of exercise for you.
• 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.

General instructions

• Work closely with your health care providers. MSA is a progressive disease that will get worse over time.
• Take over-the-counter and prescription medicines only as told by your health care provider.
• Take steps to reduce the risk of falls at home.
• Make sure that you have a good support system at home. Ask about home care assistance if needed.
• Ask your health care provider about receiving palliative care to help manage symptoms and support your family members and loved ones.
• Keep all follow-up visits as told by your health care provider. This is important.

Contact a health care provider if:

• Your symptoms change or get worse.
• You need more support at home.

Get help right away if you:

• Have an injury from a fainting spell.
• Choke when you try to swallow food or fluids.
• Have trouble breathing.
• Have chest pain.

These symptoms may represent a serious problem that is an emergency. Do not wait to see if the symptoms will go away. Get medical help right away. Call your local emergency services (911 in the U.S.). Do not drive yourself to the hospital.

Summary

• Multiple system atrophy is a rare disease of the brain and spinal cord (central nervous system). It causes a loss of nerve cells.
• Symptoms of this condition depend on which part of your nervous system is involved. You may have symptoms that mainly affect either your autonomic or motor system, or you might have a combination of symptoms that affect both.
• Treatment depends on your symptoms. It may include medicines to support blood pressure, medicines for urinary incontinence or constipation, and physical therapy to help keep muscles strong.
• Follow your health care provider’s instructions about taking medicines, eating specific foods, preventing falls at home, and when to get medical help.