Multiple system atrophy

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What is multiple system atrophy?

Multiple system atrophy (MSA), formerly called Shy-Drager syndrome, is a rare condition of the brain and nervous system. MSA affects the body’s ability to control automatic processes (body functions that you don’t have to think about) such as breathing, digestion, heart rate, movement, and blood pressure.

Multiple system atrophy causes specific regions of the brain to deteriorate: 

  • Basal ganglia: These structures link different areas of the brain to control bodily functions, such as movement, learning, memory, and emotional processing. 
  • Brainstem: This lower region of the brain controls breathing, heart rate, blood pressure, and other important processes. 
  • Cerebellum: This region in the back of the brain coordinates movement and balance. 

Penn Medicine has one of the largest movement disorders clinics in the nation. For more than four decades, we’ve been helping people with MSA and other movement disorders manage their symptoms and live more comfortably. Our experienced team can help give you an accurate diagnosis and develop a treatment plan aimed at addressing your specific needs.

MSA symptoms

MSA affects many different body systems, causing a variety of symptoms that worsen over time. MSA is one of the atypical parkinsonism disorders, meaning that early symptoms are often very difficult to distinguish from those of Parkinson’s disease.

There are two forms of MSA: the more common parkinsonian type (MSA-P) and the cerebellar type (MSA-C). Some people can have symptoms of both types.

Multiple system atrophy progression

MSA progression varies from person to person. Some people only show mild symptoms while others may experience more severe symptoms during early stages. Problems with the automatic (autonomic) processes often occur before problems with movement. 

The speed of progression during the earlier stages of MSA may relate to the rate of buildup of an abnormally folded protein called alpha-synuclein. This protein accumulates within the nerve cells in affected brain regions, causing irreversible damage. 

How is multiple system atrophy diagnosed

There is no single test for multiple system atrophy, but a combination of tests can help rule out other conditions and confirm the diagnosis. Because levels of the neurotransmitter norepinephrine may be lower than normal in people with MSA, your doctor may check the levels in your blood plasma and look for products of norepinephrine breakdown in your urine. Genetic testing can also let your doctor know if you have genes associated with MSA.

Your doctor may check to see if you have significant drops in your blood pressure when you change position, which can be done during a clinical exam or with a tilt table test. You might also need sweat testing to assess how well your body regulates internal temperature and bladder or bowel function tests to check autonomic function. Other tests can also help determine if you have MSA.

Electrocardiogram
An electrocardiogram (ECG) is a test that records the electrical activity of the heart.
Biopsy
A biopsy is the removal of a small piece of tissue for lab examination.
MRI
An MRI provides high-resolution images of the brain, spine, and many other structures in the body.

MSA treatment

There are no treatments to cure or stop the progression of MSA, but lifestyle changes and therapies may help manage your symptoms and improve your quality of life. Your doctor may recommend wearing compression socks, adding extra salt and fluid to your diet, raising the head of your bed, and moving slowly and deliberately when changing positions to help minimize dizziness and fainting. Medications can help manage MSA symptoms, including movement problems, orthostatic hypotension, urinary urgency and bladder control, and sleep disturbances.

Physical and occupational therapy can help improve balance, mobility, safety, and independence. Speech therapy can identify exercises and strategies to address problems with speech and swallowing. Your doctor might also recommend injections to temporarily weaken or paralyze specific muscles, helping to reduce isolated tremors or fixed postures.

Botulinum toxin (Botox) injections
This treatment temporarily relaxes muscles or blocks nerve signals to relieve a wide range of conditions.

Related Specialties

Movement disorders care
One of the largest movement disorders centers in the U.S. dedicated to improving patient mobility.

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