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Multiple System Atrophy

By Phillip Low, MD, Professor of Neurology;Consultant, Department of Neurology, College of Medicine, Mayo Clinic;Mayo Clinic




  • Doctors base the diagnosis on a person's response to levodopa (used to treat Parkinson disease) and results of magnetic resonance imaging and tests of autonomic function.



There are two types of multiple system atrophy (MSA):

  • MSA-C is characterized by loss of coordination and difficulty maintaining balance

  • MSA-P is very similar to Parkinson disease except that tremor often does not develop and levodopa often does not relieve symptoms

Both types involve autonomic nervous system dysfunction. Although multiple system atrophy begins as one of these types, symptoms of the other type eventually develop. After about 5 years, symptoms tend to be similar regardless of which disorder developed first.






The cause of the degeneration is unknown, but degeneration in multiple system atrophy probably results when alpha-synuclein changes shape and accumulates in support cells in the brain. Alpha-synuclein is a protein in the brain that helps nerve cells communicate, but whose normal function is not yet fully understood. Abnormal alpha-synuclein can also accumulate in people with pure autonomic failure, Parkinson disease, or Lewy body dementia. REM (rapid eye movement) sleep behavior disorder often occurs in people with disorders that involve accumulation of alpha-synuclein, including multiple system atrophy.

Locating the Cerebellum and Basal Ganglia

The cerebellum is located below the cerebrum just above the brain stem. The cerebellum coordinates the body’s movements. With information it receives from the cerebral cortex and the basal ganglia about the position of the limbs, the cerebellum helps the limbs move smoothly and accurately. (The cerebral cortex is the convoluted layer of tissue that forms the outer surface of the cerebrum. It contains most of the nerve cells in the nervous system.)

The basal ganglia are collections of nerve cells located deep within the brain. They include the following structures:


  • Putamen

  • Globus pallidus (located within the putamen)

  • Subthalamic nucleus

  • Substantia nigra



Multiple system atrophy is a progressive disorder. Early symptoms of multiple system atrophy vary, depending on which part and how much of the brain is affected first. The disorder causes three groups of symptoms.


Loss of coordination may occur. It results from degeneration in the cerebellum. People lose their balance. Later, they may be unable to control movements of their arms and legs. Consequently, they have difficulty walking and take wide, irregular steps. When reaching for an item, they may reach beyond it. When sitting, they may feel unstable. People may have difficulty focusing their eyes on and following objects. Tasks that require rapidly alternating movements, such as turning a door knob or screwing in a light bulb, also become difficult.


Other symptoms of autonomic malfunction may include the following:


  • People may have difficulty swallowing and breathing.

  • Breathing may be noisy and high-pitched.






  • A doctor's evaluation

  • Magnetic resonance imaging

  • Tests to evaluate the autonomic nervous system

The diagnosis of multiple system atrophy is based on a doctor's evaluation and results of certain tests. For example, doctors may suspect multiple system atrophy if parkinsonian symptoms are rapidly worsening and levodopa (used to treat Parkinson disease) has little or no effect on symptoms.

Magnetic resonance imaging (MRI) is usually done to check for certain changes in the brain.

Tests to evaluate the autonomic nervous system are done. They include the thermoregulatory sweat test and measurement of blood pressure while the person is sitting and after the person stands to check for orthostatic hypotension.

If MRI shows changes that suggest multiple system atrophy and test results are abnormal, multiple system atrophy is likely.


  • Symptom relief






  • Urinary incontinence: Oxybutynin, mirabegron, tamsulosin, or tolterodine, taken by mouth, may be used to relax the muscles of an overactive bladder. If incontinence persists, using a catheter inserted into the bladder may help. People may learn to insert it themselves.





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