Multiple system atrophy

  • Definition
    • Multiple system atrophy (MSA) is a rare condition that causes symptoms similar to Parkinson disease. However, people with MSA have more widespread damage to the part of the nervous system that controls important functions such as heart rate, blood pressure, and sweating.

  • Alternative Names
    • Shy-Drager syndrome; Neurologic orthostatic hypotension; Shy-McGee-Drager syndrome; Parkinson plus syndrome; MSA-P; MSA-C

  • Causes
    • The cause is unknown. MSA develops gradually and is most often diagnosed in men older than 60.

  • Symptoms
    • MSA damages the nervous system. Symptoms may include:

      • Face changes, such as a masklike appearance to the face and staring
      • Difficulty chewing or swallowing (occasionally), not able to close the mouth
      • Disrupted sleep patterns (especially during rapid eye movement (REM) sleep late at night)
      • Dizziness or fainting when standing up or after standing still
      • Frequent falls
      • Erection problems
      • Loss of control over bowels or bladder
      • Problems with activity that requires small movements (loss of fine motor skills), such as writing that is small and hard to read
      • Loss of sweating in any part of the body
      • Mild decline in mental function
      • Movement difficulties, such as loss of balance, shuffling when walking
      • Muscle aches and pains (myalgia), and stiffness
      • Nausea and problems with digestion
      • Posture problems, such as unstable, stooped, or slumped over
      • Slow movements
      • Tremors
      • Vision changes, decreased or blurred vision
      • Voice and speech changes

      Other symptoms that may occur with this disease:

  • Exams and Tests
    • Your health care provider will examine you, and check your eyes, nerves, and muscles.

      Your blood pressure will be taken while you are lying down and standing up.

      There are no specific tests to confirm this disease. A doctor who specializes in the nervous system (neurologist) can make the diagnosis based on:

      • History of symptoms
      • Physical examination results
      • Ruling out other causes of symptoms

      Testing to help confirm the diagnosis may include:

  • Treatment
    • There is no cure for MSA. There is no known way to prevent the disease from getting worse. The goal of treatment is to control symptoms.

      Anticholinergic medicines, such as levodopa or carbidopa, may be used to reduce early or mild tremors.

      But, for many people with MSA these medicines do not work well.

      Medicines may be used to treat low blood pressure.

      A pacemaker that is programmed to stimulate the heart to beat at a rapid rate (faster than 100 beats per minute) may increase blood pressure for some people.

      Constipation can be treated with a high-fiber diet and laxatives. Medicines are available to treat erection problems.

  • Outlook (Prognosis)
    • Outcome for MSA is poor. Loss of mental and physical functions slowly get worse. Early death is likely. People typically live 7 to 9 years after diagnosis.

  • When to Contact a Medical Professional
    • Call your doctor if you develop symptoms of this disorder.

      Call your doctor if you have been diagnosed with MSA and your symptoms return or get worse. Also call if new symptoms appear, including possible side effects of medicines, such as:

      • Changes in alertness/behavior/mood
      • Delusional behavior
      • Dizziness
      • Hallucinations
      • Involuntary movements
      • Loss of mental functioning
      • Nausea or vomiting
      • Severe confusion or disorientation

      If you have a family member with MSA and their condition declines to the point that you are unable to care for the person at home, seek advice from your family member's doctor.

  • References
    • Jankovic J. In: Daroff RB, Fenichel GM, Jankovic J, Mazziotta JC, eds. Bradley's Neurology in Clinical Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 71.

      Ubhi K, Low, P, Masliah E. Multiple system atrophy: a clinical and neuropathological perspective. Trends Neurosci. 2011:34;581-590. PMID: 21962754