Multiple system atrophy

Definition

Multiple system atrophy (MSA) is a rare condition that causes symptoms similar to Parkinson's disease. However, patients with MSA have more widespread damage to the part of the nervous system that controls important functions such as heart rate, blood pressure, and sweating.

Alternative Names

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

Causes

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

Symptoms

MSA damages the nervous system, which can cause the following symptoms:

• Changes in facial expression
  • "Mask" appearance to face
  • May be unable to close mouth
  • Reduced ability to show facial expressions
  • Staring
• Difficulty chewing or swallowing (occasionally)
• Disrupted sleep patterns (especially during rapid eye movement (REM) sleep late at night)
• Dizziness or fainting when standing up or after standing still
• Frequent falls
• Impotence
• Loss of control over bowels or bladder
• Loss of fine motor skills
  • Difficulty eating
  • Difficulty with any activity that requires small movements
  • Writing that is small and hard to read
• Loss of sweating in any part of the body
• Mild decline in mental function (may occur)
• Movement difficulties
  • Loss of balance
  • Shuffling
  • Walking pattern (gait) changes
• Muscle aches and pains (myalgia)
• Muscle rigidity
  • Difficulty bending arms or legs
  • Stiffness
• Nausea and problems with digestion
• Posture difficulties: may be unstable, stooped, or slumped over
• Slow movements
  • Difficulty beginning to walk or starting any voluntary movement
  • Freezing of movement when the movement is stopped, unable to start moving again
  • Small steps followed by the need to run to keep balance
• Tremors
  • May become severe enough to interfere with activities
  • May be worse when tired, excited, or stressed
  • May occur at rest or at any time
  • May occur with any action, such as holding a cup or other eating utensils
  • Finger-thumb rubbing (pill rolling tremor)
• Vision changes, decreased or blurred vision
• Voice and speech changes
  • Difficulty speaking
  • Monotone
  • Slow speaking
  • Voice is low volume

Other symptoms that may occur with this disease:

• Confusion
• Dementia
• Depression
• Sleep-related breathing difficulties, especially sleep apnea or a blockage in the air passage that leads to a harsh vibrating sound

Exams and Tests

The health care provider may perform the following:

• Blood pressure measurement, lying and standing
• Eye examination
• Nerve and muscle (neuromuscular) examination

There are no specific tests to confirm this disease. A neurologist can make the diagnosis based on:

• History of symptoms
• Findings during a physical examination
• Ruling out other causes of symptoms

Testing to help confirm the diagnosis may include:

• MRI of head
• Plasma norepinephrine levels
• Urine examination for norepinephrine breakdown products (urine catecholamines)

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 is usually added to Levodopa to reduce its side effects and make it work better. However, for people with MSA the response to medications may be disappointing. Many people respond poorly to treatment with anticholinergics or Levodopa.

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

• Beta-blockers
• Fludrocortisone (Florinef)
• MAO inhibitors
• Vasoconstrictors (midodrine)
• Vasopressin

A pacemaker that is 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. The typical survival time from the time of diagnosis is 7 to 9 years.

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 return or get worse. Also call if new symptoms appear, including possible side effects of medications:

• Changes in alertness/behavior/mood
• Delusional behavior
• Dizziness
• Hallucinations
• Involuntary movements
• Loss of mental functioning
• Nausea/vomiting
• Severe confusion or disorientation

Contact 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.

References

Jankovic J, Shannon KM. Movement disorders. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J, eds. Bradley: Neurology in Clinical Practice. 5th ed. Philadelphia, Pa: Butterworth Heinemann Elsevier; 2008:chap 75.

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