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Multiple system atrophy (MSA)

Multiple system atrophy (MSA) is a rare neurological disorder that impairs your body's involuntary (autonomic) functions, including blood pressure, heart rate, bladder function and digestion.

Formerly called Shy-Drager syndrome, the condition shares many Parkinson's disease-like symptoms, such as slowness of movement, muscle rigidity and poor balance.

Multiple system atrophy is a degenerative disease that develops in adulthood, usually in the 50s or 60s.

Treatment for MSA includes medications and lifestyle changes to help manage symptoms. The condition progresses gradually and eventually leads to death.

Symptoms Causes Complications

Multiple system atrophy (MSA) is so named because its signs and symptoms affect multiple parts of your body. Previously called Shy-Drager syndrome, MSA is classified by two types: parkinsonian and cerebellar, depending on which types of symptoms predominate at the time of evaluation.

Parkinsonian type

Predominant signs and symptoms are those of Parkinson's disease, such as:

  • Rigid muscles and difficulty bending your arms and legs
  • Slow movement (bradykinesia)
  • Tremors (rare in MSA compared with classic Parkinson's disease)
  • Impaired posture and balance

Cerebellar type

Predominant signs and symptoms are lack of muscle coordination (ataxia). Signs and symptoms may include:

  • Impairment of 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

General signs and symptoms

In addition, the primary sign of multiple system atrophy is:

  • Postural (orthostatic) hypotension, 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.

You also can develop dangerously high blood pressure levels while lying down.

People with multiple system atrophy may have other difficulties with body functions that occur involuntarily (autonomic), including:

Urinary and bowel dysfunction

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

Sweating abnormalities

  • A reduction in the production of perspiration, tears and saliva
  • Impaired control of body temperature, often causing cold hands or feet as well as heat intolerance due to impaired sweating

Sleep disorders

  • Agitated sleep due to "acting out" one's dreams
  • Abnormal breathing at night

Sexual dysfunction

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

Cardiovascular problems

  • Irregular heartbeat

Psychiatric problems

  • Difficulty controlling emotions

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.

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

MSA is associated with deterioration and shrinkage (atrophy) of portions of your brain (cerebellum, basal ganglia and brainstem) that regulate internal body functions, digestion and motor control.

Evaluation under a microscope of damaged brain tissue of people with MSA reveals nerve cells (neurons) that contain an abnormal amount of a protein called alpha-synuclein. Some research suggests that this protein may be overexpressed in multiple system atrophy.

The rate of progression of multiple system atrophy varies from person to person, but the condition does not go into remission. As the disorder progresses, daily activities become increasingly difficult.

You may experience the following complications:

  • Breathing abnormalities during sleep
  • Injuries from falls associated with impaired walking 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 (from bathing to brushing teeth)
  • Vocal cord paralysis, which makes speech and breathing difficult
  • Increased difficulty swallowing

People typically live about seven to nine years after multiple system atrophy symptoms first appear. Ten-year survival is rare. Death is often due to respiratory problems.

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