Multisystem Atrophy

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Signs and Symptoms

Symptoms vary and depend on the particular form of MSA. Striatonigral degeneration is practically indistinguishable from Parkinson's disease; OPCA is characterized by progressive ataxia (an inability to coordinate voluntary muscular movements) of the gait and arms and dysarthria (difficulty articulating words); and Shy-Drager syndrome is characterized by Parkinsonism and a much more pronounced failure of the autonomic nervous system.

Striatonigral degeneration
Striatonigral degeneration is similar to Parkinson's disease, except tremor is much less common, and cerebellar ataxia (failure of muscle coordination) often develops over time. Pathologically, striatonigral degeneration is defined by abnormalities in the putamen and substantia nigra that are more extensive and localized than what is typical of Parkinson's disease.

Other portions of the basal ganglia (the part of the brain that is responsible for organization of motor movement) are also involved. Striatonigral degeneration is often indistinguishable from Parkinson's, except that it does not respond to medications used to treat Parkinson's. MRI brain images may reveal severe degeneration of the affected structures and may provide additional diagnostic information.

Olivopontocerebellar atrophy (OPCA)
OPCAs are a group of disorders characterized by cerebellar dysfunction manifested by ataxia (imbalance and incoordination) and kinetic tremor. Kinetic tremor is rhythmic, involuntary muscular contraction that occurs during purposeful movement, such as touching your finger to your nose. Some researchers suspect that OPCA may have a genetic basis, but the clinical symptoms can vary considerably even among relatives.

The term "olivopontocerebellar" implies involvement and degeneration of the olives (structures in the medulla, part of the brainstem), the pons (part of the brainstem), and the cerebellum (area of the brain that controls coordination and balance). Symptoms may include the following:

• Ataxia
• Chorea (irregular, spasmodic, involuntary movement of the limbs or facial muscles)
• Degeneration of the retina
• Dysarthria (difficulty articulating words)
• Dystonia (abnormal muscle tension)
• Kintetic tremor
• Neuropathy
• Spasticity (state of increased muscular tone with exaggerated tendon reflexes)
• Weakness

Shy Drager Syndrome
Shy Drager syndrome, also known as "multiple system atrophy with autonomic failure," is similar to Parkinson's disease, but impairment of the autonomic nervous system is much more severe. The autonomic nervous system controls involuntary bodily functions such as blood pressure; gastrointestinal motility; and bladder, bowel, and sexual function.

The most prominent symptom of Shy Drager syndrome is what is known as "postural hypotension." Upon standing or sitting, patients' blood pressure drops to such a low level that they get dizzy, lightheaded, or momentarily blackout.

Other symptoms may include the following:

• Constipation
• Diarrhea
• Difficulties breathing or swallowing
• Difficulty walking
• Double vison or other vision problems
• Erectile dysfunction in men
• Generalized weakness
• Heart beat irregularities
• Incoordination
• Rigidity
• Sensory changes
• Speech impairment
• Tremor
• Urinary incontinence
• Urinary retention

Postural hypotension can be treated by drinking plenty of fluids, increasing salt intake, limiting caffeine intake, and by wearing elastic stockings. It also may be helpful to sleep with the head raised to reduce headaches and dizziness in the morning. Medications such as flucortisone and proamatine may be helpful in elevating blood pressure and preventing the dramatic decrease that occurs when standing.

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