ALTERNATE NAMES

MSA; Shy-Drager Syndrome; Neurologic Orthostatic Hypotension; Shy-McGee-Drager Syndrome; Parkinson's Plus Syndrome; Striatonigral Degeneration; Sporadic Olivopontocerebellar Atrophy; MSA-P; MSA-C

DESCRIPTION

Multiple System Atrophy (MSA) is the current name for disorders once known individually as Striatonigral Degeneration, Sporadic Olivopontocerebellar Atrophy, and Shy-Drager Syndrome. MSA is a rare progressive neurological disorder that causes widespread damage to the autonomic and motor functions of the nervous system. The autonomic nervous system controls organ functions such as heart rate, blood pressure, digestive system muscles, perspiration, and urination.

Physical symptoms of MSA are similar to Parkinson’s disease, but progress faster with more damage to the nervous system. Vision, voice, and speech may also be impacted. The pathological lesions of MSA consist of accumulation of abnormal alpha-synuclein protein in glial cells, but the cause of MSA is unknown. It occurs in both men and women, but is more prevalent in men in the late 50s to early 60s.

The effects of MSA are irreversible and ultimately fatal.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: Diagnosis is made when a neurologist evaluates the signs and symptoms of the disease, and is based on history or symptoms, findings during physical examination, and by ruling out other causes of symptoms. There is no specific test to confirm this disease. An MRI of the head showing abnormalities in the striatum, pons, and cerebellum is highly suggestive of MSA.

Physical findings: Physical symptoms include:

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  Diminished facial expressiveness;

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  Difficulty chewing or swallowing;

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  Disruptions in sleep patterns;

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  Dizziness or fainting (postural or orthostatic hypotension);

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  Movement difficulties such as loss of balance, shuffling, gait changes, and frequent falls;

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  Incontinence and impotence;

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  Loss of fine motor skills;

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  Loss of ability to sweat;

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  Difficulties with posture; and

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  Tremors.

ICD-9: 333.0

ICD-10: G90.3

PROGRESSION

People with MSA experience a progressive decline in neurologic functions. The rate of progression and the speed of decline may vary among people with MSA. The prognosis is poor. Progressive loss of motor skills eventually leads to total paralysis. Death generally occurs within 10 years after diagnosis.

TREATMENT

There is no cure for MSA and currently no means of slowing progression. Treatment centers on controlling symptoms.

Management of symptoms may include:

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  Levodopa or carbidopa to improve movement and balance;

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  Fludrocortisones, vasoconstrictors, and other drugs to treat orthostatic hypotension (low blood pressure);

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  Drugs and pacemakers to manage cardiac arrhythmias; and

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  Artificial feeding or breathing tubes to aid swallowing and respiration.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical evaluation documenting symptoms of autonomic failure and response to treatment;

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  Neurology consultation report; and

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  Activities of daily living report or a similar report completed by relative or caregiver.

DETERMINATION

LISTING

REMARKS

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MSA is most frequently seen in conjunction with Parkinson’s disease.

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