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    Multiple system atrophy

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

    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.

    Causes

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

    Symptoms

    MSA damages the nervous system. Symptoms can include:

    • Face changes
      • "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
    • 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

    Your doctor or nurse will examine you, and check your eyes, nerves, and muscles.

    Your blood pressure will be taken while you are lying down and standing up.

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

    • History of symptoms
    • Physical examination results
    • 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. 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. Medicines are available to treat impotence.

    Outlook (Prognosis)

    The outcome is poor. Loss of mental and physical functions slowly get worse. Early death is likely. Patients typically live 7 to 9 years after diagnosis.

    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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    • Central nervous system

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      • Central nervous system

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      A Closer Look

        Self Care

          Tests for Multiple system atrophy

            Review Date: 11/2/2012

            Reviewed By: Luc Jasmin, MD, PhD, Department of Neurosurgery, Cedars Sinai Medical Center, Los Angeles and Department of Anatomy, University of California, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, David R. Eltz, and Stephanie Slon.

            The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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