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Multiple System Atrophy

by Phillip Low, MD

Multiple system atrophy is a progressive, fatal disorder that makes muscles stiff (rigid) and causes problems with movement, loss of coordination, and malfunction of internal body processes (such as blood pressure and bladder control).

  • The parts of the brain that control movements and many internal body processes degenerate.

  • Some symptoms resemble those of Parkinson disease, but internal body processes also malfunction.

  • Doctors base the diagnosis on symptoms.

  • Simple measures and drugs can help lessen symptoms, but the disorder is progressive and ultimately fatal.

Multiple system atrophy usually begins when people are in their 50s. It affects about twice as many men as women. It results from degeneration of several parts of the brain and spinal cord:

  • The basal ganglia (collections of nerve cells at the base of the cerebrum, deep within the brain), which help control voluntary muscle movements by balancing the actions of muscle groups that move the same muscles in opposite ways (for example, a group that bends an arm and a group that straightens the arm)

  • The cerebellum, which coordinates voluntary movements (particularly complex movements done simultaneously) and helps maintain balance

  • Areas that control the autonomic nervous system, which regulates involuntary body processes, such as how blood pressure changes in response to changes in posture

The cause of the degeneration is unknown.

Multiple system atrophy includes three disorders previously thought to be separate disorders:

  • Olivopontocerebellar atrophy, which is characterized by loss of coordination and difficulty maintaining balance

  • Striatonigral degeneration, which is very similar to Parkinson disease except that tremor often does not develop and levodopa often does not relieve symptoms

  • Shy-Drager syndrome, which is characterized by parkinsonism, problems with urination, dramatic changes in blood pressure when people change positions, and malfunction of some other internal body processes

Symptoms

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

Parkinsonism —symptoms that resemble those of Parkinson disease—may occur early. These symptoms result from degeneration in the basal ganglia. Muscles are stiff (rigid), and movements become slow, shaky, and difficult to initiate. When walking, people may shuffle and not swing their arms. People feel unsteady and off balance, making them more likely to fall. Posture may be stooped. Limbs may tremble jerkily, usually when they are held in one position. But people with multiple system atrophy are less likely to have tremors during rest than people with Parkinson disease. Articulating words is difficult, and the voice may become high-pitched and quaver.

Loss of coordination may also occur early. It results from degeneration in the cerebellum. People 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.

Malfunction of internal body processes , controlled by the autonomic nervous system, may also occur early. Blood pressure may decrease dramatically when a person stands up, causing dizziness, light-headedness, or fainting—a condition called orthostatic hypotension. Blood pressure may increase when a person lies down. People may need to urinate urgently or frequently or may pass urine involuntarily (urinary incontinence). They may have difficulty emptying the bladder (urinary retention). Constipation is common. Vision becomes poor. Men may have difficulty initiating and maintaining an erection (erectile dysfunction).

Other symptoms of autonomic malfunction may occur early or late. Less sweat, tears, and saliva are produced. As a result, people may become intolerant of heat and have dry eyes and mouth. People may have difficulty swallowing and breathing. Breathing may be noisy and high-pitched. During sleep, breathing may stop repeatedly or become inadequate (sleep apnea). People may have vivid, frightening dreams that they sometimes act out while they are asleep. People may lose control of bowel movements (fecal incontinence).

Many people are confined to a wheelchair or are otherwise severely disabled within 5 years after symptoms begin. The disorder results in death 9 to 10 years after symptoms begin.

Diagnosis

The diagnosis is based on symptoms. However, symptoms may resemble those of other disorders, making the disorder difficult to diagnose.

The only sure way to diagnose multiple system atrophy is to examine brain tissue after death. Nonetheless, some tests help with the diagnosis. For example, if the combination of levodopa plus carbidopa relieves parkinsonism, the cause is probably Parkinson disease. This combination has little or no lasting effect on similar symptoms due to multiple system atrophy. Magnetic resonance imaging (MRI) of the brain may help rule out other neurologic disorders.

Tests to evaluate the autonomic nervous system may be done. For example, blood pressure may be measured while the person is sitting and after the person stands to check for orthostatic hypotension. The presence of orthostatic hypotension supports the diagnosis of multiple system atrophy.

Treatment

No treatment can cure multiple system atrophy. However, a combination of simple measures and drugs may help relieve symptoms.

  • Parkinsonism: Continuing to do as many daily activities as possible helps maintain muscle strength and flexibility. Stretching and exercising regularly may also help. Drugs used to treat Parkinson disease, such as levodopa plus carbidopa, taken by mouth, may be tried, but this combination usually has little effect or is effective for only a few years.

  • Orthostatic hypotension: Measures are taken to stabilize the sudden changes in blood pressure. Consuming more salt and water may increase the volume of blood and thus help increase blood pressure. Standing up slowly may help prevent blood pressure from decreasing too much or too fast when a person stands, as may wearing an abdominal binder or compression stockings. These garments help maintain blood pressure by promoting blood flow from the legs to the heart and thus prevent too much blood from staying (pooling) in the legs. Raising the head of the bed by about 4 inches (10 centimeters) can help prevent blood pressure from increasing too much when the person lies down. Fludrocortisone may be taken by mouth. It helps the body retain salt and water and thus may increase blood pressure as needed when a person stands. Other drugs, such as midodrine, taken by mouth, may also help.

  • Decreased production of body fluids: If sweating is reduced or absent, people should avoid warm environments to avoid overheating the body. Good dental care and regular check-ups are essential for people with dry mouth. Artificial tears (eye drops containing substances that resemble real tears) applied every few hours may relieve dry eyes.

  • Urinary retention: If needed, people can learn to insert a catheter (a thin rubber tube) into the bladder themselves. They insert it several times a day. It is inserted through the urethra, allowing urine in the bladder to drain out. People remove the catheter after the bladder is empty. This measure helps prevent the bladder from stretching and urinary tract infections from developing. Washing the hands, cleansing the area around the urethra, and using a sterile or clean catheter also help prevent infections. Inserting a catheter becomes more difficult as coordination deteriorates.

  • Urinary incontinence: Oxybutynin 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.

  • Constipation: A high-fiber diet and stool softeners are recommended. If constipation persists, enemas may be necessary.

  • Erectile dysfunction: Usually, treatment consists of drugs such as sildenafil, tadalafil, vardenafil, or avanafil, taken by mouth.

As the disorder progresses, people may need a breathing tube, a feeding tube (usually surgically inserted), or both. Physical, occupational, and speech therapists can teach people ways to compensate when walking, doing daily activities, and speaking become difficult. Social workers can help people find support groups and, when symptoms become disabling, home health care or hospice services.

End-of-life issues

Because the disorder is progressive and ultimately fatal, people should prepare advance directives soon after the disorder is diagnosed. These directives should indicate what kind of medical care people want at the end of life (see Advance directives).


Drugs Mentioned In This Article

  • Generic Name
    Select Brand Names
  • LODOSYN
  • CIALIS
  • STENDRA
  • DITROPAN XL
  • ORVATEN
  • VIAGRA
  • DETROL
  • No US brand name
  • LEVITRA