Overview

Multiple system atrophy (MSA) is a rare, degenerative neurological disorder affecting your body's involuntary (autonomic) functions, including blood pressure, and motor control.

MSA was formerly called Shy-Drager syndrome, olivopontocerebellar atrophy or striatonigral degeneration. MSA shares many symptoms with Parkinson's disease, such as slow movement, rigid muscles and poor balance.

Treatment includes medications and lifestyle changes to help manage symptoms, but there is no cure. The condition progresses gradually and eventually leads to death.

Symptoms

Multiple system atrophy (MSA) affects many parts of your body. Symptoms usually start in adulthood, usually in the 50s or 60s.

There are two types of MSA: parkinsonian and cerebellar. The type depends on the symptoms you have when you're diagnosed.

Parkinsonian type

This is the most common type of MSA. The signs and symptoms are similar to those of Parkinson's disease, such as:

  • Stiff muscles
  • Difficulty bending your arms and legs
  • Slow movement (bradykinesia)
  • Tremors can occur at rest or when moving your arms or legs
  • Soft voice
  • Problems with posture and balance

Cerebellar type

The main signs and symptoms are problems with muscle coordination (ataxia), but others may include:

  • Impaired movement and coordination, such as unsteady gait and loss of balance
  • Slurred, slow or low-volume speech (dysarthria)
  • Visual disturbances, such as blurred or double vision and difficulty focusing your eyes
  • Difficulty swallowing (dysphagia) or chewing
  • Changes in speech, such as slurred speech

General signs and symptoms

In addition, the primary sign of multiple system atrophy is autonomic failure, which may cause problems with body functions you can't control. This may include:

Postural (orthostatic) hypotension

Postural hypotension is a form of low blood pressure that makes you feel dizzy or lightheaded, or even faint, when you stand up from sitting or lying down. Not everyone with MSA has postural hypotension.

You can also develop dangerously high blood pressure levels while lying down (supine hypertension).

Urinary and bowel dysfunction

  • Constipation
  • Loss of bladder or bowel control (incontinence)

Changes in sweat production

  • Producing less sweat
  • Heat intolerance due to reduced sweating
  • Impaired body temperature control, often causing cold hands or feet

Sleep disorders

  • Agitated sleep due to "acting out" dreams
  • Abnormal breathing at night or a harsh breathing sound (stridor)

Sexual dysfunction

  • Inability to achieve or maintain an erection (impotence)
  • Loss of libido

Cardiovascular problems

  • Color changes in hands and feet caused by pooling of blood

Psychiatric problems

  • Difficulty controlling emotions, such as laughing or crying inappropriately

When to see a doctor

If you develop any of the signs and symptoms associated with multiple system atrophy, see your doctor for an evaluation and diagnosis. If you've already been diagnosed with the condition, contact your doctor if new symptoms occur or if existing symptoms worsen.

Causes

There's no known cause for multiple system atrophy (MSA). Some researchers are studying a possible inherited component or involvement of an environmental toxin in the disease process, but there's no substantial evidence to support these theories.

MSA causes deterioration and shrinkage (atrophy) of portions of your brain (cerebellum, basal ganglia and brainstem) that affect internal body functions and motor control.

Under a microscope, the damaged brain tissue of people with MSA shows nerve cells (neurons) that contain an unusual amount of a protein called alpha-synuclein. Some research suggests that there may be too much buildup of this protein in multiple system atrophy.

Complications

The progression of MSA varies, but the condition does not go into remission. As the disorder progresses, daily activities become more difficult.

Possible complications include:

  • Breathing problems during sleep
  • Injuries from falls caused by poor balance or fainting
  • Progressive immobility that can lead to secondary problems such as a breakdown of your skin
  • Loss of ability to care for yourself in day-to-day activities
  • Vocal cord paralysis, which makes speech and breathing difficult
  • Increased difficulty swallowing

People typically live about 7 to 10 years after multiple system atrophy symptoms first appear. However, the survival rate with MSA varies widely. Death is often due to respiratory problems, infections or blood clots in the lungs (pulmonary embolus).

Multiple system atrophy (MSA) care at Mayo Clinic

July 13, 2022
  1. Daroff RB, et al. Disorders of the autonomic nervous system. In: Bradley and Daroff’s Neurology in Clinical Practice. 8th ed. Elsevier; 2022. https://www.clinicalkey.com. Accessed May 4, 2022.
  2. Factor SA, et al. Multiple system atrophy: Clinical features and diagnosis. https://www.uptodate.com/contents/home. Accessed May 4, 2022.
  3. Multiple system atrophy. Genetics Home Reference. https://rarediseases.org/rare-diseases/multiple-system-atrophy/. Accessed May 4, 2022.
  4. Multiple system atrophy fact sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/msa/detail_msa.htm. Accessed May 4, 2022.
  5. Multiple system atrophy. Merck Manual Consumer Version. http://www.merckmanuals.com/home/brain,-spinal-cord,-and-nerve-disorders/autonomic-nervous-system-disorders/multiple-system-atrophy. Accessed May 4, 2022.
  6. Factor SA, et al. Multiple system atrophy: Prognosis and treatment. http://www.uptodate.com/home. Accessed May 4, 2022.
  7. Drug approval package: Northera (droxidopa) capsules approval letter. U.S. Food and Drug Administration. http://www.accessdata.fda.gov/drugsatfda_docs/nda/2014/203202Orig1s000TOC.cfm. Accessed May 4, 2022.
  8. Multiple system atrophy. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/7079/multiple-system-atrophy. Accessed May 4, 2022.
  9. Ferri FF. Multiple system atrophy. In: Ferri's Clinical Advisor 2017. Philadelphia, Pa.: Elsevier; 2017. https://www.clinicalkey.com. Accessed May 4, 2022.
  10. Coon EA (expert opinion). Mayo Clinic. May 10, 2022.
  11. Nguyen HT. Allscripts EPSi. Mayo Clinic. Jan. 28, 2022.

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