What is multiple system atrophy (MSA)?
Multiple system atrophy (MSA) is a rare neurodegenerative disease marked by a combination of symptoms affecting movement, blood pressure, and other body functions; hence the label "multiple system" atrophy. It was formerly known as Shy Drager syndrome. According to the American Autonomic Society, MSA is a sporadic, progressive, adult-onset disorder characterized by autonomic dysfunction, parkinsonism and ataxia (a failure of muscular coordination) in any combination.
Symptoms of MSA include:
- Orthostatic hypotension, or a significant fall in blood pressure when standing, causing dizziness, lightheadedness, fainting, or blurred vision
- Urinary difficulties or constipation
- Motor control symptoms, including tremor, rigidity, and loss of muscle coordination, loss of balance
- Male impotence (inability to achieve or maintain an erection)
- Speech or swallowing difficulties
Who gets MSA?
MSA affects both men and women primarily in their 50s.
What causes multiple system atrophy (MSA)?
MSA is associated with deterioration and shrinkage (atrophy) of portions of the brain (cerebellum, basal ganglia and brainstem) that regulate internal body functions, digestion and motor control.
There is no known cause for brain changes in MSA.
How is multiple system atrophy (MSA) diagnosed?
Diagnosis of MSA can be challenging because there is no test that can make or confirm the diagnosis in a living patient. Certain signs and symptoms of MSA also occur with other disorders, such as Parkinson's disease, making the diagnosis more difficult.
If your doctor suspects MSA, he or she will obtain a medical history and perform a physical examination. You may receive a referral to a neurologist or other specialist for specific evaluations that can help in making the diagnosis.
Tests that may be helpful in making a diagnosis include:
- Tilt table test - In this procedure, your blood pressure is monitored while you are on a special table that will tilt you to an almost upright position. This allows the physician to record blood pressure irregularities, and information about whether they occur with a change in physical position.
- Blood tests
- A sweat test to evaluate perspiration
- Tests to assess your bladder and bowel function
- Electrocardiogram to track the electrical signals of your heart
- Brain-imaging tests, particularly a magnetic resonance imaging (MRI) scan, to determine if another condition might be triggering symptoms
- Pharmacological challenge tests (administering certain medications and observing the patient’s body’s reaction to them, in controlled clinical settings)
For patients with sleep irregularities, particularly if they involve interrupted breathing or snoring, physicians may recommend an evaluation in a sleep laboratory to determine if there is an underlying and treatable sleep disorder, such as sleep apnea.
What is the treatment for multiple system atrophy (MSA)?
There is no known cure for MSA, so management of the disease focuses on treating the more disabling symptoms listed above.
Frequently Asked Questions about MSA
How common is MSA?
Between 25,000 and 100,000 Americans are believed to have MSA. The incidence (new cases per 100,000 person years) for ages 50-99 years is 3-5.
What is the progression of MSA?
Disease progression in MSA is quicker than in Parkinsonism. About 80% of patients are disabled within 5 years of onset of the motor symptoms, and only 20% survive past 12 years. The mean survival after diagnosis is roughly 6 years. The rate of progression differs in every case and speed of decline may vary widely in individual patients.
What is the prognosis of MSA / Shy-Drager Syndrome?
The probable outcome is poor. There is a progressive loss of physical functions until general debilitation develops. Early death is likely. Most people who are diagnosed with MSA die within seven to 10 years after symptoms begin.
Pneumonia is the most common cause of death, although irregularities in heartbeat or breathing may be responsible for death in some patients. Breathing problems such as aspiration, stridor (high-pitched breathing sounds due to airway obstruction), or cardiopulmonary arrest can occur.
See https://ghr.nlm.nih.gov/condition/multiple-system-atrophy for additional information.