• 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

    Multiple system atrophy (MSA) are a rare group of diseases. It is associated with progressive damage to the nervous system. MSA is sometimes called a Parkinson’s plus syndrome because many of the symptoms are similar. MSA has debilitating effects on the nervous system. Once symptoms develop, the average life expectancy is ten years or less.
    Nervous System
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    These three diseases were once considered unrelated because their initial symptoms were different. However, they are now all considered MSAs.
    • Shy-Drager syndrome (MSA-A) —most prominent symptoms are in the autonomic system (actions that do not involve conscious control, like blood pressure)
    • Striatonigral degeneration (MSA-P)— Parkinson -type symptoms, such as slowed movements and rigidity
    • Olivopontocerebellar atrophy (MSA-C)—affects balance, coordination, and speech
    If you suspect you have this condition, contact your doctor.

    Causes

    The cause of MSA is unknown.

    Risk Factors

    These factors are associated with the development of MSA:
    • Men—affected twice as frequently as women
    • Age—the average age that symptoms develop is 54 years of age

    Symptoms

    Symptoms of MSA can vary greatly. If you have any of these symptoms do not assume it is due to MSA. These symptoms may be caused by other conditions. Tell your doctor if you have any of these:
    • Constipation
    • Retention of urine
    • Loss of balance
    • Parkinsonism—symptoms like Parkinson’s disease: slower movements, tremor, stiff muscles, postural instability
    • Poor coordination
    • Abnormal eye movements
    • Orthostatic hypotension —drop in blood pressure within minutes of standing up
    • Erectile dysfunction
    • Swallowing problems
    • Breathing problems
    • Sleep apnea
    • Shuffling
    • Slurred speech

    Diagnosis

    Your doctor will ask about your symptoms and medical history. A neurological exam will be done. You will likely be referred to a specialist. Neurologists focus solely on the nervous system.
    Diagnosing MSA can be difficult. It usually means ruling out other diseases. For example, MSA can look like Parkinson’s disease. Tests may include the following:
    • MRI scan —test that uses magnetic waves to form an image of structures inside the body; used to rule out other nervous system diseases and check for abnormalities in the brain that suggest MSA
    • Tests of autonomic function, such as measuring your heart rate and blood pressure under different circumstances, your response to a medicine called clonidine , and an electromyogram (EMG) (a test using a needle to assess your muscles)
    • Blood tests
    • Lumbar puncture —a test to evaluate spinal fluid

    Treatment

    There is no specific treatment for MSA. Symptoms may be treated. Talk with your doctor about the best plan for you. Options include the following:

    Medications

    Various medications may be used to treat the symptoms of MSA:
    • Levodopa —used to treat Parkinson’s disease; may be used to treat muscle rigidity
    • Paroxetine (Paxil)—may improve mood
    • Increasing dietary salt and fluids, compression stockings, elevating the head of the bed, and medications that raise blood pressure may be used to treat hypotension
    • Other drugs may be used to treat the symptoms of constipation , drooling, urinary, or erectile dysfunction
    • Continuous positive airway pressure (CPAP) for sleep apnea and other breathing problems during sleep

    Physical Therapy

    Therapy may be used to keep muscles strong and maintain range of motion.
    Therapy may be used to keep muscles strong and maintain range of motion.

    Occupational Therapy

    This therapy may be used to improve functions of daily living. This includes help with eating, grooming, and dressing.

    Speech Therapy

    This may help speaking and swallowing. A feeding tube is sometimes inserted in later stages of MSA. It will deliver nutrition directly to the stomach.

    Prevention

    There are no known guidelines to prevent MSA.

    RESOURCES

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

    National Dysautonomia Research Foundation http://www.ndrf.org/

    Shy-Drager/Multiple System Atrophy Support Group http://www.shy-drager.org/

    Worldwide Education and Awareness for Movement Disorders http://www.wemove.org/

    CANADIAN RESOURCES

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

    Canadian Neurological Sciences Federation http://www.ccns.org/

    References

    Bradley WG, Daroff RB, Fenichel GM, Jankovic J, Eds. Neurology in Clinical Practice. 5th edition. Philadelphia:Butterworth Heinemann Elsevier; 2008:Chapter 75, Movement Disorders.

    Fauci AS, Braunwald E, Kasper DL, Hauser SL, Longo DL, Jameson JL, and Loscalzo J, Eds. Harrison's Principles of Internal Medicine .17th edition. United States:The McGraw-Hill Companies; 2008:Chapter 370, Diseases of the Central Nervous System.

    Jankovic JJ, Tolosa E, Eds. Parkinson’s Disease & Movement Disorders. 4th edition. Baltimore:Lippincott Williams & Wilkins;2002:Chapter 13, Secondary Parkinsonism.

    Multiple system atrophy. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/ . Updated September 12, 2011. Accessed September 8, 2012.

    Overview of multiple system atrophy. Worldwide Education and Awareness for Movement Disorders website. Available at: http://www.wemove.org/msa/msa.html . Accessed November 5, 2008.

    Shy-Drager syndrome. DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Accessed September 8, 2012.

    Wenning G, Geser F. Diagnosis and Treatment of Multiple System Atrophy: An Update. ACNR . 2004;3(6):5-10.

    What is multiple system atrophy? National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/msa/msa.htm . Accessed September 8, 2012.

    Esper CD, Factor SA. Current and Future Treatments in Multiple System Atrophy. Current Treatment Options in Neurology. 2007;9:210-223. Available at: http://www.treatment-options.com/article%5Fframe.cfm?PubId=NE09-3-2-04&Type=Article&KeyWords=multiple%20system%20atrophy&HitNum=15 . Accessed November 24, 2008.

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