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Multiple system atrophyDefinition:
Multiple system atrophy (MSA) is a rare condition that causes symptoms similar to Parkinson's disease . However, patients with MSA have more widespread damage to the autonomic nervous system. This is the part of the nervous system that controls important organ functions such as heart rate, blood pressure, digestive system muscles, and sweating.
Shy-Drager syndrome; Neurologic orthostatic hypotension; Shy-McGee-Drager syndrome; Parkinson's plus syndrome
Causes, incidence, and risk factors:
The cause is unknown. MSA develops gradually and is most often diagnosed in men older than 60.
MSA damages the nervous system, which can cause the following symptoms:
- Changes in facial expression
- "Mask" appearance to face
- May be unable to close mouth
- Reduced ability to show facial expressions
Difficulty chewing or swallowing (occasionally)
- 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
- Loss of control over bowels or bladder
- Loss of fine motor skills
- Difficulty eating
- Difficulty with any activity that requires small movements
- Writing that is small and hard to read
- Loss of sweating in any part of the body
- Mild decline in mental function (may occur)
Muscle aches and pains (myalgia)
- Difficulty bending arms or legs
- Nausea and problems with digestion
- Posture difficulties: may be unstable, stooped, or slumped over
- Slow movements
- Difficulty beginning to walk or starting any voluntary movement
- Freezing of movement when the movement is stopped, unable to start moving again
- Small steps followed by the need to run to keep balance
- May become severe enough to interfere with activities
- May be worse when tired, excited, or stressed
- May occur at rest or at any time
- May occur with any action, such as holding a cup or other eating utensils
- Finger-thumb rubbing (pill rolling tremor)
- Vision changes, decreased or blurred vision
- Voice and speech changes
Other symptoms that may occur with this disease:
Signs and tests:
The health care provider may perform the following:
Blood pressure measurement, lying and standing
- Eye examination
- Nerve and muscle (neuromuscular) examination
There are no specific tests to confirm this disease. A neurologist can make the diagnosis based on:
- History of symptoms
- Findings during a physical examination
- Ruling out other causes of symptoms
Testing to help confirm the diagnosis may include:
There is no cure for MSA, and there is no known way to prevent the disease from getting worse. The goal of treatment is to control symptoms.
Anticholinergic medications may be used to reduce early or mild tremors. Levodopa may improve movement and balance.
Carbidopa may reduce the side effects of Levodopa and make it work better. However, the response to medications may be disappointing. Many people respond poorly to treatment with anticholinergics or Levodopa.
Medications that may be used to treat low blood pressure include:
- Fludrocortisone (Florinef)
- MAO inhibitors
- Vasoconstrictors (midodrine)
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. Impotence may be treated with drugs that enhance erections.
The outcome is poor. Loss of mental and physical functions slowly get worse. Early death is likely.
- Progressive loss of ability to walk or care for self
- Difficulty performing daily activities
- Injuries from falls/fainting
- Side effects of medications
Calling your health care provider:
Call your health care provider if you develop symptoms of this disorder.
Call your health care provider 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 medications:
- Changes in alertness/behavior/mood
- Delusional behavior
- Involuntary movements
- Loss of mental functioning
- Severe confusion or disorientation
Contact your health care provider if you have a family member with this disorder and his or her condition deteriorates to the point that you are unable to care for the person at home.
Lang A. Parkinsonism. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 433.
|Review Date: 10/30/2008|
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Daniel B. Hoch, PhD, MD, Assistant Professor of Neurology, Harvard Medical School Department of Neurology, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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