A degenerative disorder characterized by progressive damage to the autonomic nervous system (the portion of the nervous system that controls involuntary functions such as blood pressure, heart rate, digestion and sexual function), muscle tremor and rigidity, slow movement, and other widespread neurologic losses.

Alternative Names

Shy-Drager Syndrome; Neurologic orthostatic hypotension; Shy-McGee-Drager syndrome; Parkinson's plus syndrome

Causes, incidence, and risk factors

Multiple system atrophy (MSA) is a rare degenerative condition. It is a condition that appears similar to Parkinson's disease in that patients may be slow moving, tremulous and have a shuffling gait but with more widespread neurologic damage and damage to the autonomic nervous system (the portion of the nervous system that controls involuntary functions). The cause is unknown. There is progressive damage (degeneration) of the nervous system, with damage to all parts of the nervous system. The disorder develops gradually. It is most often diagnosed in men over 60 years old.

Signs and tests

An eye examination may reveal atrophy of the iris and paralysis of eye muscles. Postural hypotension (drop in blood pressure associated with change in position) is evident. A neuromuscular examination shows abnormal reflexes and may show severe muscle wasting (atrophy). Parkinsonian movements ( tremor , rigidity, and slow movements similar to Parkinson's disease ) are common. Diagnosis is largely clinical as there are no specific tests during life to confirm the disease. A neurologist makes the diagnosis based on the history of symptoms and the findings on physical examination as well as ruling out other causes. Testing may include:

Treatment

There is no cure for the disorder and no known means to slow progression. The 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 the Levodopa work better. The response to medications may be disappointing. Many affected individuals respond poorly to treatment with anticholinergics or Levodopa. Sympathomimetics, vasoconstrictors, beta-blockers, MAO inhibitors, vasopressin , 9-fluohydrocortisone, or other medications may be used to treat low blood pressure (postural hypotension). A heart pacemaker programmed to stimulate the heart to beat at a rapid rate (faster than 100) may increase the blood pressure for some people.

Expectations (prognosis)

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.

Complications

Calling your health care provider

Call your health care provider if symptoms of Shy-Drager syndrome recur, worsen, or progress. Call your health care provider if new symptoms appear, including possible side effects of medications: involuntary movements, nausea/vomiting , dizziness , changes in alertness/behavior/mood, severe confusion or disorientation, delusional behavior, hallucinations , loss of mental functioning, or any other new symptoms that develop (see also the specific medication). Also, consult with your health care provider if the condition of a family member with this disorder deteriorates and you are unable to care for the person at home.

Prevention

There is no known prevention for this rare disorder.