Multiple System Atrophy

In 1960, two researchers, Dr. Milton Shy at the National Institutes of Health, and Dr. Glen Drager at Baylor College of Medicine in Houston, Texas, described a common set of neurological manifestations associated with autonomic failure.

Originally called the "Shy-Drager Syndrome" this complex syndrome is currently referred to as "Multiple System Atrophy" or MSA.

The American Autonomic Society has defined MSA in the following manner:

"MSA is a sporadic, progressive, adult onset disorder characterized by autonomic dysfunction, Parkinsonism, and ataxia (a failure of muscular coordination) in any combination. The features of the disorder include:

• Parkinsonism

• Cerebellar or Corticospinal Signs

• Orthostatic Hypotension, Impotence, Urinary Incontinence or Retention, usually preceding or within two years after the onset of the motor symptoms

Parkinsonian and cerebellar features commonly occur in combination. However, certain features may predominate."

When MSA presents with a predominating feature, corresponding terminology is used to describe the syndrome (Table 1). It is important to note that these manifestations may occur in various combinations and may also evolve over time.

Table 1

CLINICAL FEATURES: 

MSA typically presents in the fifth to seventh decade of life, with men predominating the condition by a factor of two to one.

The most common symptoms are those of autonomic failure and parkinsonism - tremor, muscular rigidity, and hypokinesia ( decreased mobility, motor function or activity ). The autonomic failure symptoms include the following:

• Orthostatic Hypotension Including:

Lightheadedness

Tiredness

Blurred Vision

Ataxia ( Failure of Muscular Coordination)

Pain in the Rear of the Neck

• Mild Cognitive Impairment occurs in about 20% of those affected with MSA
• Multiple system atrophy is associated with both upper airway and central apnea, which can be life threatening.
• Nocturnal snoring, and sleep apnea are common and are related to upper airways obstruction
• Dysarthria , or problems with articulation of speech is a common symptom
• Muscular Wasting

Long-Term Outlook

The majority of individuals diagnosed with MSA do not currently have a good prognosis with the survival rate typically being seven years from the time of diagnosis of the disease. The evidence indicates that the more severe the involvement of the autonomic nervous system, and to a lesser extent the striatonigral system, the poorer the prognosis.

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Last modified: Monday January 28, 2008.