Multiple System Atrophy

(MSA; Sporadic Olivopontocerebellar Atrophy; Shy-Drager Syndrome; Parkinson’s Plus Syndrome; Multi-system Degeneration; Multi-system Atrophy; Idiopathic Autonomic Failure; Idiopathic Orthostatic Hypotension;)

Definition

Definition

Multiple system atrophy (MSA) is a disorder of the nervous system.

MSA is sometimes called a Parkinson’s plus syndrome because many of the symptoms are similar. There are different types MSA based on symptoms. Once symptoms develop, the average life expectancy is 10 years or less.

Causes

Causes

The cause of MSA is unknown. Genetic factors may play a role in some families.

The symptoms are caused by degeneration of nerves throughout the brain and spinal cord. These nerves control automatic functions like balance and muscle coordination. The damage to the nerve may be caused by a buildup of a specific protein but this is not a confirmed cause.

Nervous System

Copyright © Nucleus Medical Media, Inc.

Risk Factors

Risk Factors

MSA tends to appear after 60 years of age.

Symptoms

Symptoms

Symptoms of MSA can vary greatly. Initial symptoms are similar to those of Parkinson disease. These initial symptoms may also be determined by the type of MSA:

  • Type MSA-A is associated with orthostatic hypotension. This is a problem managing blood pressure when moving from sitting to standing. It can lead to lightheadedness or fainting.
  • Type MSA-P is associated with symptoms similar to initial Parkinson symptoms such as:

    • Slow, stiff movements
    • Tremors
    • Clumsiness—loss of balance and coordination
    • Shuffling
  • Type MSA-C is associated with:

    • Difficulty swallowing
    • Problems speaking and hoarseness
    • Difficulty coordinating muscle movement

As the disease progresses, symptoms will cross over types and become more severe. Many will develop muscle coordination problems and need walking aids. Other problems that may exist across type include:

  • Problems with bladder control
  • Erectile dysfunction
  • Muscle tightening around a joint that prevents free movement
  • Problems with posture, such as leaning to one side and forward head bend
  • Inappropriate laughing or crying
  • Excess yawning
  • Vision problems
  • Changes in writing

Diagnosis

Diagnosis

You will be asked about your symptoms and medical history. An exam of the nervous system will also be done. You will likely be referred to a specialist.

Your bodily fluids may be tested. This can be done with:

Images may be taken of your bodily structures. This can be done with an MRI scan.

Tests will also be done on your heart rate and blood pressure. These tests will help determine what is causing problems with your autonomic nervous system. Nerve impulses to your muscles may also be tested.

Treatment

Treatment

There is no cure for MSA. Treatment will focus on managing the symptoms and supportive care. Talk with your doctor about the best plan for you. Options include the following:

Medications

Various medications may be used to manage the symptoms of MSA. Medication may be given to:

  • Relieve muscle rigidity
  • Stabilize mood
  • Manage orthostatic hypotension
  • Relieve other symptoms such as constipation, urinary control problems, or erectile dysfunction

Therapy

  • Physical therapy—to help keep muscles strong and maintain range of motion
  • Occupational therapy—to improve ability to do daily tasks such as eating, grooming, and dressing
  • Speech therapy—to help with speaking and swallowing
  • Respiratory therapy— Continuous positive airway pressure (CPAP) for sleep apnea and other breathing problems during sleep

Dietary Support

Dietary changes in salt and fluids may help manage orthostatic hypotension.

Soft or pureed foods may be helpful with swallowing problems.

A feeding tube may be needed in later stages of MSA. It will deliver nutrition directly to the stomach.

Prevention

Prevention

There are no known guidelines to prevent MSA since the cause is not clear.

RESOURCES:

National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov

The Multiple System Atrophy Coalition https://www.multiplesystematrophy.org

CANADIAN RESOURCES:

Canadian Institutes of Health Research http://www.cihr-irsc.gc.ca

Health Canada http://www.hc-sc.gc.ca

References:

Multiple system atrophy. EBSCO DynaMed website. Available at:
http://www.ebscohost.com/dynamed
Updated August 27, 2014. Accessed November 30, 2014.

Multiple system atrophy fact sheet. National Institute of Neurological Disorders and Stroke website. Available at:
http://www.ninds.nih.gov/disorders/msa/detail_msa.htm
Updated November 5, 2014. Accessed April 19, 2013.

Multiple system atrophy/Shy-Drager syndrome. Vanderbilt University Medical Center website. Available at:
http://www.mc.vanderbilt.edu/root/vumc.php?site=adc&doc=4791
Accessed November 30, 2014.

NINDS multiple system atrophy with orthostatic hypotension. National Institute of Neurological Disorders and Stroke website. Available at:
http://www.ninds.nih.gov/disorders/msa_orthostatic_hypotension/msa_orthostatic_hypotension.htm
Updated December 5, 2013. Accessed November 30, 2014.

NINDS olivopontocerebellar atrophy information page. National Institute of Neurological Disorders and Stroke website. Available at:
http://www.ninds.nih.gov/disorders/opca/opca.htm
Updated April 16, 2014. Accessed November 30, 2014.

1/20/2015 DynaMed’s Systematic Literature Surveillance
http://www.ebscohost.com/dynamed:
Fanciulli A, Wenning GK. Multiple-system atrophy. N Engl J Med. 2015 Jan 15;372(3):249-263.

Last reviewed December 2014 by Rimas Lukas, MD
Last Updated: 1/20/2015

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