Multiple system atrophy (MSA)

Overview

Multiple system atrophy (MSA) is a rare, degenerative neurological disorder affecting your body's involuntary (autonomic) functions, including blood pressure, and motor control.

MSA was formerly called Shy-Drager syndrome, olivopontocerebellar atrophy or striatonigral degeneration. MSA shares many symptoms with Parkinson's disease, such as slow movement, rigid muscles and poor balance.

Treatment includes medications and lifestyle changes to help manage symptoms, but there is no cure. The condition progresses gradually and eventually leads to death.

Symptoms

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

There are two types of MSA: parkinsonian and cerebellar. The type depends on the symptoms you have when you're diagnosed.

Parkinsonian type

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

  • Stiff muscles
  • Difficulty bending your arms and legs
  • Slow movement (bradykinesia)
  • Tremors can occur at rest or when moving your arms or legs
  • Soft voice
  • Problems with posture and balance

Cerebellar type

The main signs and symptoms are problems with muscle coordination (ataxia), but others may include:

  • Impaired movement and coordination, such as unsteady gait and loss of balance
  • Slurred, slow or low-volume speech (dysarthria)
  • Visual disturbances, such as blurred or double vision and difficulty focusing your eyes
  • Difficulty swallowing (dysphagia) or chewing
  • Changes in speech, such as slurred speech

General signs and symptoms

In addition, the primary sign of multiple system atrophy is autonomic failure, which may cause problems with body functions you can't control. This may include:

Postural (orthostatic) hypotension

Postural hypotension is a form of low blood pressure that makes you feel dizzy or lightheaded, or even faint, when you stand up from sitting or lying down. Not everyone with MSA has postural hypotension.

You can also develop dangerously high blood pressure levels while lying down (supine hypertension).

Urinary and bowel dysfunction

  • Constipation
  • Loss of bladder or bowel control (incontinence)

Changes in sweat production

  • Producing less sweat
  • Heat intolerance due to reduced sweating
  • Impaired body temperature control, often causing cold hands or feet

Sleep disorders

  • Agitated sleep due to "acting out" dreams
  • Abnormal breathing at night or a harsh breathing sound (stridor)

Sexual dysfunction

  • Inability to achieve or maintain an erection (impotence)
  • Loss of libido

Cardiovascular problems

  • Color changes in hands and feet caused by pooling of blood

Psychiatric problems

  • Difficulty controlling emotions, such as laughing or crying inappropriately

When to see a doctor

If you develop any of the signs and symptoms associated with multiple system atrophy, see your doctor for an evaluation and diagnosis. If you've already been diagnosed with the condition, contact your doctor if new symptoms occur or if existing symptoms worsen.

Causes

There's no known cause for multiple system atrophy (MSA). Some researchers are studying a possible inherited component or involvement of an environmental toxin in the disease process, but there's no substantial evidence to support these theories.

MSA causes deterioration and shrinkage (atrophy) of portions of your brain (cerebellum, basal ganglia and brainstem) that affect internal body functions and motor control.

Under a microscope, the damaged brain tissue of people with MSA shows nerve cells (neurons) that contain an unusual amount of a protein called alpha-synuclein. Some research suggests that there may be too much buildup of this protein in multiple system atrophy.

Complications

The progression of MSA varies, but the condition does not go into remission. As the disorder progresses, daily activities become more difficult.

Possible complications include:

  • Breathing problems during sleep
  • Injuries from falls caused by poor balance or fainting
  • Progressive immobility that can lead to secondary problems such as a breakdown of your skin
  • Loss of ability to care for yourself in day-to-day activities
  • Vocal cord paralysis, which makes speech and breathing difficult
  • Increased difficulty 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 respiratory problems, infections or blood clots in the lungs (pulmonary embolus).

Diagnosis

Diagnosing multiple system atrophy (MSA) can be challenging. Certain signs and symptoms of MSA — such as muscle rigidity and unsteady gait — also occur with other disorders, such as Parkinson's disease. This can make diagnosis more difficult. The physical exam, along with various autonomic tests and imaging studies, can help your doctor determine whether the diagnosis is probable MSA or possible MSA. Because of difficulty with making the diagnosis, some people are never properly diagnosed.

If your doctor thinks that you may have multiple system atrophy, they will take your medical history, perform a physical exam and possibly order blood tests. Brain imaging scans, such as an MRI, can show signs that may suggest MSA and also help determine if there are other causes that may be contributing to your symptoms.

You may receive a referral to a neurologist or other specialist for specific evaluations that can help in making the diagnosis.

Tilt table test

This test can help determine if you have a problem with blood pressure control. In this procedure, you're placed on a motorized table and strapped in place. Then the table is tilted upward so that your body is positioned at a 70-degree angle.

During the test, your blood pressure and heart rate are monitored. The findings can document both the extent of blood pressure irregularities and whether they occur with a change in physical position.

Tests to assess autonomic functions

Doctors may order other tests to assess your body's involuntary functions, including:

  • Blood pressure measurement, lying down and standing
  • A sweat test to evaluate areas of the body that sweat
  • Tests to assess your bladder and bowel function
  • Electrocardiogram to track the electrical signals of your heart

If you have sleep irregularities, especially interrupted breathing or snoring, your doctor may recommend an evaluation in a sleep laboratory. This can help diagnose an underlying and treatable sleep disorder, such as sleep apnea.

Treatment

There's no cure for multiple system atrophy. Managing the disease involves treating signs and symptoms to make you as comfortable as possible and to maintain your body functions.

To treat specific signs and symptoms, your doctor may recommend:

  • Medications to raise blood pressure. The corticosteroid fludrocortisone and other medications can increase your blood pressure by helping your body retain more salt and water.

    The drug pyridostigmine (Mestinon) can raise your standing blood pressure without increasing your blood pressure while you're lying down.

    Midodrine (Orvaten) can raise your blood pressure quickly; however, it needs to be taken carefully as it can elevate pressure while lying down. You should not lie flat for four hours after taking the medication.

    The FDA has approved droxidopa (Northera) for treating orthostatic hypotension. The most common side effects of droxidopa include headache, dizziness and nausea.

  • Medications to reduce Parkinson's disease-like signs and symptoms. Certain medications used to treat Parkinson's disease, such as combined levodopa and carbidopa (Sinemet, Rytary, others), can be used to reduce Parkinson's disease-like signs and symptoms, such as stiffness, balance problems and slowness of movement. These medications can also improve overall well-being.

    However, not everyone with multiple system atrophy responds to Parkinson's drugs. They may also become less effective after a few years.

  • Impotence drugs. Impotence can be treated with a variety of drugs, such as sildenafil (Revatio, Viagra), designed to manage erectile dysfunction, but these can lower blood pressure.
  • Steps to manage swallowing and breathing difficulties. If you have difficulty swallowing, try eating softer foods. If swallowing or breathing becomes increasingly problematic, you may consider a surgically inserted feeding or breathing tube. In advanced MSA, you may consider a tube (gastrostomy tube) that delivers food directly into your stomach.
  • Bladder care. If you're experiencing bladder control problems, medications can help in the earlier stages. Eventually, when the disease becomes advanced, you may need to have a soft tube (catheter) inserted to allow you to drain your bladder.
  • Therapy. A physical therapist can help you maintain as much of your motor and muscle capacity as possible as the disorder progresses.

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

Lifestyle and home remedies

Doctors often advise using certain self-care measures to help minimize MSA symptoms, such as:

  • Taking 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 caffeinated fluids to raise your blood pressure.
  • Elevating the head of your bed. Raising the head of your bed by about 4 to 6 inches will minimize increases in blood pressure when you sleep. Get up slowly from a reclining position.
  • Making dietary changes. Add more fiber to your diet in order to ease constipation. You may also benefit from over-the-counter laxatives or stool softeners. Eat small, low-carbohydrate meals.
  • Avoiding getting too hot. Stay in air-conditioned rooms on very hot days. Avoid excessive amounts of heat in the bathroom when bathing.
  • Wearing elastic support stockings up to your waist. This can help keep your blood pressure from dropping.

Preparing for an appointment

You may have your first appointment to discuss your symptoms with your family doctor, but you may be referred to a doctor who specializes in the diagnosis and treatment of conditions that affect the brain and nervous system (neurologist).

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

What you can do

  • Write down any symptoms you're experiencing, 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 doctor.
  • Make a list of your key medical information, including other conditions with which you've been diagnosed, and the names of any prescription and over-the-counter medications you're taking.
  • Write down key personal information, including any changes in your sex life, such as reduced interest in sex or difficulty getting an erection.
  • Find a family member or friend who can come with you to the appointment, if possible, to help you remember what the doctor says.
  • Write down the questions to ask your doctor.

For multiple system atrophy, some basic questions to ask your doctor 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 progress?
  • Will treatment slow the progression of my illness or simply relieve symptoms?
  • Are there self-care steps that could help ease my symptoms?
  • How will you monitor my health over time?
  • Do I need to adjust the medications I'm taking for other health conditions?

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

What to expect from your doctor

Your doctor is likely to ask you a number of questions, including:

  • What are your symptoms?
  • When did you begin having symptoms?
  • Do you feel lightheaded or dizzy when you stand up?
  • Have you ever fainted?
  • Do your symptoms include any emotional changes, such as swinging between laughter and tears?
  • 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 problems with bladder control?
  • Have you been constipated?
  • Have you had any sexual problems, such as reduced libido or impotence?
  • Do you have difficulty chewing or swallowing?
  • Do you have difficulty breathing?
  • Do you have any movement problems, such as slowness or poor coordination?
  • Do you have any family history of Parkinson's disease or other 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 such as a parent, sibling, or grandparent have been diagnosed with neurological disorders such as Parkinson's disease or cerebellar ataxia. Multiple system atrophy (MSA) is not known to be an inherited condition, so a family history of a condition with similar symptoms may help your doctor rule out MSA.


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