Shy-Drager syndrome

Causes and Risks:
Shy-Drager syndrome is a rare degenerative condition. It is a condition that appears similar to Parkinson's disease 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.

Prevention:
There is no known prevention for this rare disorder.

Symptoms:
  • dizziness or fainting when arising or after standing still
  • impotence
  • loss of sweating of any part of the body
  • loss of control of bowels or bladder
  • vision changes, decreased or blurred vision
  • muscle rigidity
    • stiffness
    • difficulty bending arms or legs

  • posture may be unstable, stooped, or slumped over
  • movement difficulties

  • slow movements
    • difficulty beginning to walk, difficulty initiating any voluntary movement
    • small steps followed by the need to run to maintain balance
    • freezing of movement when the movement is stopped, unable to resume movement

  • muscle aches and pains ( myalgia )
  • shaking, tremors (may present in varying degrees, may not be present)
    • may occur at rest or at any time
    • may become severe enough to interfere with activities
    • may be worse when tired, excited, or stressed
    • finger-thumb rubbing (pill rolling tremor ) may be present

  • changes in facial expression
    • reduced ability to show facial expressions
    • "mask" appearance to face
    • staring
    • may be unable to close mouth

  • voice and/or speech changes

  • difficulty chewing or swallowing (occasionally)
  • loss of fine motor skills
    • difficulty writing, may be small and illegible
    • difficulty eating
    • difficulty with any activity that requires small movements

  • frequent falls
  • mild decline in intellectual function (may occur)

Additional symptoms that may be associated with this disease:



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.

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.

Amantadine and/or 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.

Prognosis:
The probable outcome is poor. There is a progressive loss of mental and physical functions until general debilitation develops. Early death is likely.

Complications:

  • progressive loss of ability to walk or care for self
  • difficulty performing daily activities
  • injuries from falls/ fainting
  • side effects of medications



Call Your Healthcare 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.