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Multiple system atrophy
Alternative namesShy-Drager syndrome; Neurologic orthostatic hypotension; Shy-McGee-Drager syndrome; Parkinson's plus syndrome
Multiple system atrophy is a degenerative disorder characterized by the following:
Causes, incidence, and risk factors
Multiple system atrophy (MSA) is a rare degenerative condition. Its symptoms are similar to those of Parkinson's disease in that patients may move slowly, be tremulous, and have a shuffling gait.
However, in MSA there is more widespread neurologic damage and damage to the autonomic nervous system (the portion of the nervous system that controls involuntary functions). There is progressive degeneration of the nervous system, with damage to all parts of the nervous system, including the brain.
The cause is unknown. MSA develops gradually and is most often diagnosed in men older than 60.
Signs and tests
The health care provider may perform the following:
Parkinsonian movements ( tremor , rigidity, and slow movements similar to Parkinson's disease ) are common.
There are no specific tests to confirm this disease. A neurologist can make the diagnosis based on the history of symptoms, the findings during physical examination, and ruling out other causes of symptoms.
Testing to help confirm the diagnosis may include the following:
There is no cure for MSA, and there is no known means to slow progression. Treatment is aimed at controlling symptoms such as postural hypotension and parkinsonian movements.
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 affected individuals respond poorly to treatment with anticholinergics or Levodopa.
Medications that may be used to treat low blood pressure include the following:
A pacemaker programmed to stimulate the heart to beat at a rapid rate (faster than 100 beats per minute) may increase blood pressure for some people.
The probable outcome is poor. There is a progressive loss of mental and physical functions until general debilitation develops. Early death is likely.
Rate of progression differs in every case and speed of decline may vary widely in individual patients.
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 recur, worsen, or progress. Also call if new symptoms appear, including possible side effects of medications:
See also the specific medication for possible side effects.
Consult with 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.
Update Date: 1/12/2003Elaine T. Kiriakopoulos, M.D., M.Sc., Department of Neurology, Beth Israel Deaconess Medical Center, Harvard University, Boston, MA. Review provided by VeriMed Healthcare Network.
Last updated: Tue, 06 Jan 2009 00:20:03 GMT