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Shy-Drager Syndrome
Introduction
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.
Signs and symptoms
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.
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.
Treatment
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. - 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.
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.
Information obtained from National Institute of Health