Multiple system atrophy

Definition

Multiple system atrophy (MSA) is a rare degenerative condition that causes symptoms similar to Parkinson's disease. However, patients with MSA have more widespread damage to the autonomic nervous system, the part of the nervous system that controls involuntary functions.

Alternative Names

Shy-Drager syndrome; Neurologic orthostatic hypotension; Shy-McGee-Drager syndrome; Parkinson's plus syndrome

Causes

MSA is characterized by the following:

• Progressive damage to the nervous system, leading to low blood pressure when standing, difficulty urinating, and abnormal breathing during sleep
• Muscle tremor and rigidity
• Slow movement
• Disrupted REM sleep patterns

The cause is unknown. MSA develops gradually and is most often diagnosed in men older than 60.

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 difficulties: may be unstable, stooped, or slumped over
• Movement difficulties
  • Loss of balance
  • Gait (walking pattern) changes
  • Shuffling
• Slow movements
  • Difficulty beginning to walk or 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)
• Tremors: may be present in varying degrees or 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
  • May occur with any action such as holding a cup or other eating utensils
• Changes in facial expression
  • Reduced ability to show facial expressions
  • "Mask" appearance to face
  • Staring
  • May be unable to close mouth
• Voice and speech changes
  • Slow speaking
  • Voice is low volume
  • Monotone
  • Difficulty speaking
• Difficulty chewing or swallowing (occasionally)
• Loss of fine motor skills
  • 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:

• Depression
• Confusion
• Dementia
• Seborrheic dermatitis
• Sleep-related breathing difficulties, especially sleep apnea and air passage obstruction accompanied by a harsh vibrating sound

Exams and Tests

The health care provider may perform the following:

• Eye examination
• Neuromuscular examination
• Blood pressure measurement

There are no specific tests to confirm this disease. A neurologist can make the diagnosis based on the history of symptoms, the findings during physical examination, and by ruling out other causes of symptoms.

Testing to help confirm the diagnosis may include:

• Plasma norepinephrine levels
• Urine examination for norepinephrine breakdown products (urine catecholamines)
• MRI of head to rule out other conditions

Treatment

There is no cure for MSA, and there is no known way to prevent the disease from getting worse. The goal of treatment is to control symptoms.

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 it work better. However, the response to medications may be disappointing. Many affected individuals respond poorly to treatment with anticholinergics or Levodopa.

Medications that may be used to treat low blood pressure include:

• Vasoconstrictors (midodrine)
• Beta-blockers
• MAO inhibitors
• Vasopressin
• 9-fluohydrocortisone

A pacemaker programmed to stimulate the heart to beat at a rapid rate (faster than 100 beats per minute) may increase blood pressure for some people.

Constipation can be treated with a high-fiber diet and laxatives. Impotence may be treated with drugs that enhance erections.

Outlook (Prognosis)

The outcome is poor. Loss of mental and physical functions slowly get worse. Early death is likely.

Possible Complications

• Progressive loss of ability to walk or care for self
• Difficulty performing daily activities
• Injuries from falls/fainting
• Side effects of medications

When to Contact a Medical Professional

Call your health care provider if you develop symptoms of this disorder.

Call your health care provider if you have been diagnosed with MSA and your symptoms recur, worsen, or progress. Also call 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

See also the specific medication for possible side effects.

Consult with your health care provider if you have a family member with this disorder and his or her condition deteriorates to the point that you are unable to care for the person at home.