Multiple System Atrophy
(MSA; Olivopontocerebellar Atrophy; Shy-Drager Syndrome; Parkinson’s Plus Syndrome)
Definition
Multiple system atrophy (MSA) is a rare disease. It causes 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 body. Once symptoms develop the average life expectancy is ten years or less.
Nervous System
These three diseases were once considered unrelated because their initial symptoms were different. However, they are now all considered MSAs.
- Shy-Drager syndrome —most prominent symptoms are in the autonomic system (actions that don’t involve conscious control, like blood pressure)
- Striatonigral degeneration— Parkinson -type symptoms such as slowed movements and rigidity
- Olivopontocerebellar atrophy—affects balance, coordination, and speech
If you suspect you have this condition, contact your doctor promptly.
Risk Factors
These factors are associated with the development of MSA:
- Men—affected twice as frequently as women
- Age—the mean 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
- 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 but it generally progresses faster.
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 (a test using a needle to assess your muscles)
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
- 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 , 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.
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 to deliver nutrition to the stomach.
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. Mayo Clinic website. Available at: http://www.mayoclinic.org/multiple-system-atrophy/ . Accessed November 5, 2008.
Multiple system atrophy. Merck Manual website. Available at: http://www.merck.com/mmpe/sec16/ch208/ch208d.html . Accessed November 5, 2008.
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 November 11, 2008.
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 November 5, 2008.
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_frame.cfm?PubId=NE09-3-2-04&Type=Article&KeyWords=multiple%20system%20atrophy&HitNum=15 . Accessed November 24, 2008.
Last reviewed November 2008 by Judy Chang, MD, FAASM
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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