Multiple system atrophy

Overview

Multiple system atrophy, also called MSA, causes people to lose coordination and balance or become slow and stiff. It also causes changes in speech and loss of control of other bodily functions.

MSA is a rare condition. It sometimes shares symptoms with Parkinson's disease, including slow movement, rigid muscles and poor balance.

Treatment includes medicines and lifestyle changes to help manage symptoms, but there is no cure. The condition gets worse over time and eventually leads to death.

In the past, this condition has been called Shy-Drager syndrome, olivopontocerebellar atrophy or striatonigral degeneration.

Symptoms

Multiple system atrophy (MSA) symptoms affect many parts of the body. Symptoms start in adulthood, usually in the 50s or 60s.

There are two types of MSA: parkinsonian and cerebellar. The type depends on the symptoms a person has when diagnosed.

Parkinsonian type

This is the most common type of MSA. Symptoms are similar to those of Parkinson's disease, such as:

Stiff muscles.
Trouble bending the arms and legs.
Slow movement, known as bradykinesia.
Tremors at rest or when moving the arms or legs.
Slurred, slow or soft speech, known as dysarthria.
Trouble with posture and balance.

Cerebellar type

The main symptoms of the cerebellar type involve poor muscle coordination, known as ataxia. Symptoms may include:

Trouble with movement and coordination. This includes loss of balance and not being able to walk steadily.
Slurred, slow or soft speech, known as dysarthria.
Changes in vision. This may include blurred or double vision and not being able to focus the eyes.
Trouble chewing or swallowing, known as dysphagia.

General symptoms

For both types of multiple system atrophy, the autonomic nervous system doesn't work properly. The autonomic nervous system controls involuntary functions in the body, such as blood pressure. When this system doesn't work properly, it can cause the following symptoms.

Postural hypotension, also known as orthostatic hypotension

Postural hypotension is a form of low blood pressure. People who have this type of low blood pressure feel dizzy or lightheaded when they stand up after sitting or lying down. They may even faint. Not everyone with MSA has postural hypotension.

People with MSA also can develop dangerously high blood pressure levels while lying down. This is called supine hypertension.

Urinary and bowel symptoms

These symptoms include:

Constipation.
Loss of bladder or bowel control, known as incontinence.

Changes in sweat production

People with multiple system atrophy may:

Produce less sweat.
Have heat intolerance because they sweat less.
Have poor body temperature control, often causing cold hands or feet.

Conditions that affect sleep

Sleep symptoms may include:

Agitated sleep due to "acting out" dreams. This is known as rapid eye movement (REM) sleep behavior disorder.
Breathing that stops and starts during sleep, known as sleep apnea.
A high-pitched whistling sound while breathing, called stridor.

Sexual symptoms

These symptoms may include:

Trouble getting or keeping an erection, known as erectile dysfunction.
Trouble with lubrication during sex and having an orgasm.
Loss of interest in sex.

Cardiovascular symptoms

MSA may cause:

Color changes in the hands and feet.

Psychiatric symptoms

People with multiple system atrophy also may experience:

Trouble controlling emotions, such as laughing or crying when not expected.

When to see a doctor

If you develop any of the symptoms of multiple system atrophy, see your healthcare professional. If you have already been diagnosed with MSA, contact your healthcare professional if your symptoms become worse or if new symptoms occur.

Causes

There is no known cause for multiple system atrophy (MSA). Some researchers are studying the possible role of genetics or environmental causes such as a toxin in MSA. But there's no substantial evidence to support these theories.

MSA causes parts of the brain to shrink. This is known as atrophy. The areas of the brain that shrink due to MSA include the cerebellum, basal ganglia and brainstem. The atrophy of these parts of the brain affect internal body functions and movement.

Under a microscope, the brain tissue of people with MSA shows a buildup of a protein called alpha-synuclein. Some research suggests that the buildup of this protein leads to multiple system atrophy.

Risk factors

A risk factor for multiple system atrophy (MSA) is having rapid eye movement (REM) sleep behavior disorder. People with this disorder act out their dreams. Most people who have MSA have a history of REM sleep behavior disorder.

Another risk factor is having a condition caused by the autonomic nervous system not working properly. Symptoms such as urinary incontinence could be an early sign of MSA. The autonomic nervous system controls involuntary functions.

Complications

Complications of multiple system atrophy (MSA) vary from person to person. But for everyone with the disease, MSA symptoms get worse over time. The symptoms can make daily activities harder as time goes on.

Possible complications include:

Worsening breathing symptoms during sleep.
Injuries from falls caused by poor balance or fainting.
The breakdown of the skin in people who have trouble moving or can't move.
Not being able to care for yourself in day-to-day activities.
Vocal cord paralysis, which affects speech and breathing.
Increased trouble swallowing.

People typically live about 7 to 10 years after multiple system atrophy symptoms first appear. However, the survival rate with MSA varies widely. Death is often due to trouble breathing, infections or blood clots in the lungs.

Diagnosis

Diagnosing multiple system atrophy (MSA) can be challenging. Symptoms such as stiffness and trouble walking can happen in other diseases, including Parkinson's disease. This can make MSA hard to diagnose.

Your healthcare professional gives you a physical exam, reviews your medical history and tests your autonomic functions such as blood pressure. You also may need blood tests and imaging tests, such as an MRI. These tests can help diagnose MSA or point to another causes of your symptoms.

If your healthcare professional thinks you have multiple system atrophy, test results help determine whether the diagnosis is clinically established MSA or clinically probable MSA. Because it's hard to make a diagnosis, some people are never properly diagnosed.

You may be referred to a neurologist or another specialist for further evaluation. A specialist can help diagnose the disease.

Tilt table test

This test evaluates your blood pressure control. In this procedure, you're placed on a motorized table. Straps hold you in place. Then the table is tilted upward so that your body is positioned at a 70-degree angle.

During the test, your healthcare professional watches for changes in your blood pressure and heart rate. The test can show if your blood pressure doesn't respond in a typical way when changing position.

Tests of autonomic functions

Other tests can look at blood pressure control and other involuntary functions, including:

Blood pressure measurement, lying down and standing, without the use of a tilt table.
A sweat test to evaluate areas of the body that sweat.
Tests that look at bladder and bowel function.
Electrocardiogram to track the electrical signals of your heart.

You might need a sleep study if you stop breathing during sleep or if you snore or have other sleep symptoms. The test can help diagnose a sleep condition that can be treated, such as sleep apnea.

Treatment

Treatment for multiple system atrophy (MSA) involves managing your symptoms. There's no cure for MSA. Managing the disease can make you as comfortable as possible and help you maintain your body functions.

To treat specific symptoms, your healthcare team may recommend:

Medicines to raise blood pressure. These medicines can treat low blood pressure that happens when standing after sitting or lying down, known as postural hypotension. There are several medicines your healthcare professional may recommend.

The corticosteroid fludrocortisone can increase blood pressure by helping your body retain more salt and water.

Midodrine (Orvaten) can raise your blood pressure quickly. This medicine needs to be taken carefully because it can increase blood pressure while lying down. Don't lie flat for four hours after taking this medicine.

The medicine pyridostigmine (Mestinon, Regonol) can raise your blood pressure while standing without causing a large increase when you're lying down.

Another medicine called droxidopa (Northera) also treats postural hypotension. The most common side effects of droxidopa include headaches, dizziness and nausea.
Medicines to reduce Parkinson's disease-like symptoms. Medicines that treat Parkinson's disease, such as combined levodopa and carbidopa (Sinemet, Duopa, others), can help some people with MSA. The medicine can treat stiffness, trouble with balance and slow movements.

Many people with multiple system atrophy do not respond to Parkinson's medicines. The medicines also may become less effective after a few years.
Medicines to treat erection problems. If you have trouble getting or keeping an erection, you can be treated with medicines such as sildenafil (Revatio, Viagra). This medicine helps manage erectile dysfunction but it can lower blood pressure.
Steps to manage swallowing and breathing symptoms. If you have trouble swallowing, try eating softer foods. If swallowing or breathing symptoms become worse, you might need surgery to insert a feeding or breathing tube. A gastrostomy tube delivers food directly into your stomach.

If you have sleep apnea, you might be treated with continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP). Stridor also can be treated with CPAP.
Bladder care. If you have trouble with bladder control, medicines can help in the earlier stages. But as MSA gets worse, you may need to have a soft tube inserted to drain your bladder. The soft tube is known as a catheter.
Therapy. A physical therapist can help you maintain as much of your movement and strength as possible as the disease gets worse.

A speech-language pathologist can help you improve or maintain your speech.

Lifestyle and home remedies

Some lifestyle and home remedies can help minimize multiple system atrophy (MSA) symptoms.

Take steps to raise your blood pressure. Add a little salt to your diet and drink more fluids, especially before exercise. Salt and fluids can increase blood volume and raise your blood pressure. Drink coffee and other fluids that contain caffeine to increase your blood pressure.
Raise the head of your bed. Raising the head of your bed by about 4 to 6 inches (10 to 15 centimeters) helps control your blood pressure when you sleep. Get up slowly after lying down.
Make dietary changes. Eat more fiber to ease constipation. Laxatives or stool softeners may help. You can get them without a prescription. Also eat small, low-carbohydrate meals.
Don't get too hot. Stay in air-conditioned rooms on very hot days. Make sure the bathroom doesn't become too hot when you shower or bathe.
Wear elastic support stockings up to your waist. This can help keep your blood pressure from dropping.

Preparing for an appointment

You first may see your healthcare professional. This person may refer you to a doctor trained in nervous system conditions, called a neurologist.

Here's some information to help you prepare for your appointment.

What you can do

Write down any symptoms you're having, and for how long. Include all recent changes in your physical or emotional well-being. For example, if you or your loved ones have noticed your moods shifting more quickly, share this with your healthcare professional.
Make a list of your medical information. Include other conditions you have. Also include the names of any medicines you take with or without a prescription, along with the doses.
Write down key personal information, including any changes in your sex life. For example, let your healthcare professional know if you have less interest in sex or trouble getting an erection.
Find a family member or friend who can come with you to the appointment, if possible. A family member or friend may help you remember what your healthcare professional says.
Write down questions to ask during your appointment.

For multiple system atrophy, some basic questions include:

What is likely causing my symptoms?
Are there any other possible causes for these symptoms, such as Parkinson's disease?
How will you make a firm diagnosis?
What tests do I need?
What treatment options are available for multiple system atrophy?
What are the possible side effects of those treatment options?
How is my condition likely to change over time?
Will treatment slow the illness or simply relieve symptoms?
Can self-care steps help ease my symptoms?
How is my health monitored over time?
Do I need to adjust the medicines I'm taking for other health conditions?

Don't hesitate to ask any other questions that you have.

What to expect from your doctor

You're likely to be asked a number of questions. Questions about your symptoms may include:

What are your symptoms and when did they begin?
Do you feel lightheaded or dizzy when you stand up? Have you ever fainted?
Do your symptoms include emotional changes, such as laughing then crying?
Have you noticed any changes in your voice?
Have you been told that you snore loudly or have interrupted breathing while sleeping?
Have you had constipation or problems with bladder control?
Have you had any sexual symptoms, such as lower sex drive or trouble getting or keeping an erection?
Do you have trouble chewing or swallowing?
Do you have trouble breathing?
Do you have any movement symptoms, such as slowness or poor coordination?

General questions may include:

Do you have a family history of Parkinson's disease or another neurological disease?
Are you being treated for any other health conditions?

What you can do in the meantime

While you wait for your appointment, find out if any blood relatives have been diagnosed with a nervous system condition such as Parkinson's disease or cerebellar ataxia. Blood relatives include a parent, sibling or grandparent. Multiple system atrophy (MSA) is not known to be passed down through families. A family history of a condition with similar symptoms may help rule out MSA.