Multiple System Atrophy FS.indd 1 

Multiple 


System 


Atrophy
 

U.S. DEPARTMENT OF HEALTH 

AND HUMAN SERVICES 

Public Health Service 

National Institutes of Health 
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Multiple System Atrophy
 

What is multiple system atrophy? 

Multiple system atrophy (MSA) is a progressive neurodegenerative disorder 

characterized by symptoms of autonomic 

nervous system failure such as fainting 

spells and bladder control problems, combined with motor control symptoms such as 

tremor, rigidity, and loss of muscle coordination. MSA affects both men and women 

primarily in their 50s. The disease tends to 

advance rapidly over the course of 9 to 10 

years, with progressive loss of motor skills, 

eventual confinement to bed, and death. 

There is no remission from the disease. 

There are currently no cures. 

What causes MSA? 

The cause or causes of MSA are unknown, 

but the symptoms reflect the dysfunction 

and eventual loss of nervous system cells in 

several different areas in the brain and spinal 

cord that control the autonomic nervous system 

and coordinate muscle movements. The 

hallmark of MSA is the buildup of a protein 

called alpha-synuclein in oligodendrocytes, 

a type of glial (support) cell. MSA and 

Parkinson’s disease are called synucleinopathies 

because they share a similar buildup of alphasynuclein and cause loss of the dopamineproducing cells that control motor movements. 

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What are the common signs or symptoms? 

The initial symptoms of MSA are often difficult to distinguish from the initial 

symptoms of Parkinson’s disease and include: 

•	 fainting or lightheadedness due to 

orthostatic hypotension, a condition 

in which blood pressure drops rapidly 

when rising from a seated or lying 

down position; 

•	 bladder control problems, such as a 

sudden urge to urinate, difficulty 

emptying the bladder completely, 

or, for men, difficulty achieving 

an erection; 

•	 clumsiness, loss of balance, and 

frequent falls; 

•	 slurred speech, a croaky, quivering 

voice, or difficulty swallowing; and 

•	 decreased spontaneous movement, 

tremor, or rigid muscles. 

Doctors divide MSA into two different 

types, depending on the most prominent 

symptoms at the time an individual 

is evaluated: 

•	 the parkinsonian type (MSA-P) has 

primary characteristics of Parkinson’s 

disease, such as moving slowly, stiff 

muscles, and tremor, along with problems 

of balance, coordination, and autonomic 

nervous system dysfunction; and 

•	 the cerebellar type (MSA-C), with primary 

symptoms featuring difficulty swallowing, 

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slurred speech or a quavering voice, 


along with ataxia (problems with
 

balance and coordination). 


Some people with MSA may start out with 

the symptoms of one type and then develop 

increasingly more severe symptoms of the 

other type as the disease progresses. Most 

people with MSA will require an aid for 

walking, such as a cane or walker, within 

a few years after symptoms begin. 

Additional symptoms of MSA include: 

•	 contractures in the hands or feet that 

prevent the joints from moving freely; 

•	 Pisa syndrome, an abnormal posture in 

which the body appears to be leaning to 

one side like the Leaning Tower of Pisa; 

•	 disproportionate antecollis, in which 

the neck bends forward and the head 

drops down; 

•	 deep, uncontrollable sighing or gasping; 

•	 inappropriate laughing or crying. 

How is MSA diagnosed? 

Making a positive diagnosis of MSA is difficult, particularly in the early 

stages, because it so closely resembles 

Parkinson’s disease or one of the many 

other ataxia disorders. 

After taking a clinical history and performing a brief neurological examination, 

a doctor may order a number of tests to 

help make the diagnosis. These tests include 

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autonomic function tests, sphincter electromyography, urine analysis (and other tests 

to assess bladder function), and magnetic 

resonance imaging (MRI), which may 

reveal characteristic patterns of damage 

in the brain. People with MSA typically 

don’t have any improvement in their symptoms if they are given the anti-Parkinson 

drug levodopa. However, this lack of response 

isn’t a strong enough indicator, by itself, to 

make a positive diagnosis of MSA. Unfortunately, the only definitive diagnosis for MSA 

comes at an autopsy of brain tissue after 

death; however, doctors often use criteria 

developed under the auspices of the National 

Institute of Neurological Disorders and 

Stroke (NINDS) to help in the diagnosis of 

“definite,” “probable,” or “possible” MSA. 

How is it treated? 

There is no cure for MSA. Currently, there are no treatments to delay the progress 

of neurodegeneration in the brain. But there 

are treatments available to help people cope 

with some of the more disabling symptoms 

of MSA. 

The fainting and lightheadedness from 

orthostatic hypotension is often treated 

successfully with simple interventions such 

as adding extra salt to the diet and avoiding 

heavy meals and alcohol. Some people with 

MSA sleep with the head of the bed tilted 

up or use a compression body stocking. The 

drugs fludrocortisone (for those on a high 

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salt diet) and midodrine are sometimes 

prescribed to increase blood volume and 

narrow blood vessels. Drinking a glass or 

two of water before getting out of bed in the 

morning can also help raise blood pressure. 

Bladder control problems are treated 

according to the nature of the problem. 

Anticholinergic drugs, such as oxybutynin, 

may help reduce the sudden urge to urinate. 

Limiting fluid intake after the evening meal 

and taking desmopressin at night can reduce 

episodes of night-time bedwetting. 

Impotence and erectile dysfunction are 

treated in several ways, including injections, 

suppositories, or implants. The drug sildenafil is effective for erectile dysfunction but 

may worsen orthostatic hypotension. 

Difficulties with swallowing and breathing 

eventually require that people with MSA use 

an artificial feeding tube or breathing tube. 

Muscle spasms and contractures usually 

benefit from physical therapy that builds 

strength and encourages people to remain 

mobile for as long as possible. 

What research is being done? 

The NINDS supports research about MSA through grants to major medical 

institutions across the country. A great deal 

of research is ongoing to learn why synuclein 

buildup occurs in MSA and Parkinson’s 

disease, and how to prevent it. 

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Where can I find more information on 

MSA and Parkinson’s disease? 

For more information on neurological disorders or research programs funded 

by the National Institute of Neurological 

Disorders and Stroke, contact the Institute’s 

Brain Resources and Information Network 

(BRAIN) at: 

BRAIN 

P.O. Box 5801 

Bethesda, MD 20824 

800-352-9424 

www.ninds.nih.gov 

The following organizations support MSA 

research and in some cases may be able to 

provide additional information: 

Dysautonomia Foundation 

315 West 39th Street 

Suite 701 

New York, NY 10018 

212-279-1066 

www.familialdyasutonomia.org 

Familial Dysautonomia Hope 

Foundation, Inc. 

121 South Estes Drive 

Suite 205D 

Chapel Hill, NC 27514 

919-969-6636 

www.fdhope.org 

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National Dysautonomia Research Foundation 

P.O. Box 301 

Red Wing, MN 55066-0301 

651-267-0525 

www.ndrf.org 

Shy-Drager/Multiple System Atrophy Support 

Group, Inc. 

P.O. Box 279 

Coupland, TX 78615 

866-SDS-4999 (737-4999) 

www.shy-drager.com 

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Prepared by: 

Office of Communications and Public Liaison 

National Institute of Neurological 

Disorders and Stroke 

National Institutes of Health 

Department of Health and Human Services 

Bethesda, Maryland 20892 2540 

NIH Publication No. 09 5597 June 2009