Shy-drager Syndrome

Alternate Names
Shy-McGee-Drager syndrome
neurological orthostatic hypotension

Definition of Shy-drager Syndrome

A person with Shy-Drager syndrome has an abnormally functioning autonomic nervous system. The autonomic nervous system controls many involuntary functions of the body, including blood pressure and heart rate.

What is going on in the body?

Shy-Drager syndrome is somewhat similar to Parkinson's disease, but causes more extensive damage in the body. There is widespread degeneration of many parts of the autonomic nervous system, which controls involuntary activities of the body such as blood pressure.

What are the signs and symptoms of the disease?

Symptoms of Shy-Drager syndrome can include:

• dizziness and fainting
• low blood pressure, especially upon standing (postural hypotension)
• erectile dysfunction
• tremors, that is, small involuntary muscle movements
• muscle rigidity or muscle aches
• urinary incontinence, that is, loss of bladder control
• gait changes, that is, changes in how the person walks
• changes in facial expression
• difficulty swallowing and chewing
• confusion, dementia, or depression

What are the causes and risks of the disease?

The exact cause of Shy-Drager syndrome is not known. It is more common in people older than 60 years and occurs more frequently in males.

What can be done to prevent the disease?

There is no known way to prevent Shy-Drager syndrome.

How is the disease diagnosed?

The first step in the diagnosis of Shy-Drager syndrome is a complete medical history and full neurologic exam. The healthcare professional may then order tests, such as:

• an electroencephalogram (EEG) to study the electrical pathways in the brain
• cranial CT scan or MRI scan to check for abnormalities in the brain structures
• blood and urine tests to measure levels of chemicals involved in nerve transmission

The healthcare professional will also check the person's vital signs, such as pulse and blood pressure. These findings are often abnormal in a person with Shy-Drager syndrome. Orthostatic hypotension, which is an inability to maintain an adequate blood pressure while changing body positions, will be present in someone with Shy-Drager syndrome. Orthostatic hypotension can cause dizziness, fainting, and injury.

What are the long-term effects of the disease?

A person with Shy-Drager syndrome may experience a progressive loss of ability in walking and other motor functions.

What are the risks to others?

Shy-Drager syndrome is not transmitted from one person to another. It does not appear to be an inherited disorder.

What are the treatments for the disease?

Because there is no known cure, treatments are used to control the symptoms of Shy-Drager syndrome. Medication can be given to raise blood pressure in an individual with low blood pressure. Anticholinergic medications may be given to help reduce the tremors. A pacemaker can be inserted to control the heart rate as needed.

What are the side effects of the treatments?

Side effects vary depending on the medications used, but may include dizziness, dry mouth, and allergic reactions.

What happens after treatment for the disease?

As Shy-Drager syndrome progresses, the person may experience a decreased ability to walk, increasing falls, and side effects from medications.

How is the disease monitored?

A person with Shy-Drager syndrome will need ongoing monitoring by the healthcare professional, who can watch for any changes in neurological status and treat symptoms as they occur. Any new or worsening symptoms should be reported to the healthcare professional.