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Definition :
Shy-Drager syndrome is a degenerative neurological disorder that affects your brain and other parts of your central nervous system. It causes a drop in blood pressure when standing up, bladder dysfunction and other Parkinson's-like symptoms, such as slowness of movement, muscle rigidity and poor balance.

Shy-Drager syndrome develops in adulthood, beginning at an average age of 50 years, and is gradually progressive and usually fatal. A rare condition, Shy-Drager syndrome occurs about two to three times more frequently in men.

Shy-Drager syndrome is named after two researchers who first described it in 1960. It's commonly referred to as multiple system atrophy (MSA). Treatment for Shy-Drager syndrome involves management of symptoms.

Shy-Drager syndrome is associated with deterioration and shrinkage (atrophy) of portions of your brain (cerebellum, basal ganglia and brainstem) that regulate internal body functions, digestion and motor control.

When damaged brain tissue of people with Shy-Drager syndrome is evaluated under a microscope, nerve cells (neurons) that contain an abnormal amount of a protein called alpha-synuclein can be seen. Some research suggests that this protein may be overexpressed in Shy-Drager.

Nevertheless, there's no known cause for the disease-related changes in Shy-Drager syndrome. Some researchers are studying whether there's an inherited component or environmental toxin involved in the disease process, but there's no substantial evidence to support these theories.

When to seek medical advice :
If you develop any of the signs and symptoms associated with Shy-Drager syndrome, see your doctor for an evaluation and diagnosis. If you've already been diagnosed with the condition, contact your doctor if new symptoms occur, or if existing symptoms worsen.

Shy-Drager is a neurological condition. It's also known as "multiple system atrophy" because it encompasses a number of signs and symptoms and involves impairment of multiple parts of your body.

The condition is characterized by impairment of your autonomic nervous system, which governs the involuntary, internal processes of your body — including blood pressure, heart rate, bladder function and digestion.

The primary sign of Shy-Drager syndrome is orthostatic hypotension — an excessive decrease in your blood pressure, typically when standing up, or while standing still. This decline in blood pressure can trigger symptoms that include dizziness and lightheadedness.

Sometimes, blood pressure in people with Shy-Drager syndrome can climb to very high levels while reclining in a facedown or faceup position, due to an overall dysfunction of the body's blood pressure control mechanisms.

You also may experience :

  • Rigid muscles and difficulty bending your arms and legs
  • Impairment of movement and coordination (unsteady gait, loss of balance)
  • A reduction in the production of perspiration, tears and saliva
  • Impaired control of body temperature
  • Loss of bladder or bowel control (incontinence), often the first sign reported by women
  • Constipation
  • Slurred speech; slow or low-volume speech
  • Visual disturbances (blurred vision, difficulty focusing your eyes)
  • Impotence
  • Trouble controlling emotions
  • Irregular heartbeat
  • Cognitive impairment and confusion — mild in most cases, but possibly interfering with attention span and the speed of thinking
  • Impaired ability to chew or swallow

Diagnosis of Shy-Drager syndrome can be challenging because there's no test that can make or confirm the diagnosis. At the same time, certain signs and symptoms of Shy-Drager — such as muscle rigidity and unsteady gait — also occur with other disorders, such as Parkinson's disease, making the diagnosis more difficult. As a result, some people are never properly diagnosed, although doctors are increasingly aware of the disease and thus more likely to identify its symptoms.

If your doctor suspects Shy-Drager syndrome, he or she will obtain a medical history and perform a physical examination. You may receive a referral to a neurologist or other specialist for specific evaluations that can help in making the diagnosis.

Tilt table test
One procedure that can aid in the diagnostic process is a tilt table test to help determine if you have a problem with blood pressure control. In this procedure, you're placed on a motorized table and strapped in place. Then the table is tilted upward so that your body is nearly vertical.

Throughout this maneuver, your blood pressure and heart rate are monitored. The findings can document not only the extent of blood pressure irregularities, but also whether they occur with a change in physical position.

Other tests
At times, doctors may order certain other tests to help establish a disorder or rule out the presence of other conditions. These other tests may include :

  • Blood tests
  • A "sweat test" to evaluate perspiration
  • Tests to assess your bladder and bowel function
  • Electrocardiogram, to track the electrical signals of your heart
  • Brain-imaging tests, particularly a magnetic resonance imaging (MRI) scan, to determine whether brain lesions are present that may be triggering symptoms

If you have sleep irregularities, particularly if they involve interrupted breathing or snoring, your doctor may recommend having you evaluated in a sleep laboratory to determine if you have an underlying and treatable sleep disorder, such as sleep apnea.

Complications :
The rate of progression of Shy-Drager syndrome varies from person to person, but the condition does not go into remission. As the disorder progresses, daily activities become increasingly difficult.

You may experience the following complications :

  • Breathing abnormalities during sleep
  • Injuries from falls associated with impaired walking
  • Progressive immobility that can lead to secondary problems such as a breakdown of your skin
  • Loss of ability to care for yourself in day-to-day activities (from bathing to brushing teeth)
  • Vocal cord paralysis, which makes speech and breathing difficult
  • Increased difficulty swallowing

On average, about 80 percent of people are disabled within five years after motor symptoms of Shy-Drager initially occur. People live about seven to 10 years after Shy-Drager symptoms first appear, and survival beyond 10 years is uncommon. The most frequent reason for death is breathing impairment or the development of pneumonia, or both. In a smaller number of people, irregular heart rhythms or choking is the cause of death.

There's no cure for Shy-Drager syndrome. Management of the disease involves treating signs and symptoms to make you as comfortable as possible and to maintain your body functions and capabilities.

To treat specific signs and symptoms, your doctor may recommend :

  • Medications to raise blood pressure. Medications such as the corticosteroid fludrocortisone (Florinef) can increase your blood pressure by leading to more retention of salt and water. This drug, which is a steroid, is often prescribed on a twice-a-day regimen. The drug pyridostigmine (Mestinon) can raise your standing blood pressure without increasing your blood pressure while you're lying down.

    Other medications to increase standing pressure, such as midodrine, are available and can raise your blood pressure quickly; however, midodrine has been shown to elevate pressure while lying down, which can increase the risk of stroke in some people.

  • Medications to reduce Parkinson's-like signs and symptoms. Certain anti-Parkinson's medications, such as levodopa, can be used to reduce Parkinson's-like signs and symptoms, such as stiffness, balance problems and slowness of movement, as well as improve an overall feeling of well-being.

    However, response to Parkinson's drugs varies in people with Shy-Drager, and these drugs don't work for everybody. Levodopa also is difficult for some people to tolerate because of side effects, such as allergic reactions, nausea and depression. Often, another drug — carbidopa — is prescribed simultaneously in order to reduce the dosage and side effects of levodopa and increase effectiveness.

  • Pacemaker. Your doctor may advise implanting a heart pacemaker to keep your heart beating at a rapid pace, which can increase your blood pressure.
  • Impotence drugs. Impotence can be treated with a variety of drugs designed to manage erectile dysfunction.
  • Steps to manage swallowing and breathing difficulties. If you have difficulty swallowing, try eating softer foods. If swallowing or breathing becomes increasingly problematic, you may need a feeding tube or breathing tube inserted surgically to manage the problem. Late in the disease process, you may require a tube (gastrostomy tube) that delivers food directly into your stomach.
  • Bladder care. If you're experiencing bladder control problems, medications can help in the earlier stages. Eventually, when the disease becomes advanced, you may need to have a soft tube (catheter) inserted permanently to allow you to drain your bladder.

A physical therapist can help you maintain as much of your motor and muscle capacity as possible as the disorder progresses. A speech-language pathologist can help you learn to improve or maintain your speaking ability.

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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