TN 16 (03-18)
DI 23022.630 Multiple System Atrophy
COMPASSIONATE ALLOWANCE INFORMATION
MULTIPLE SYSTEM ATROPHY
MSA; Shy-Drager Syndrome; Neurologic Orthostatic Hypotension; Shy-McGee-Drager Syndrome; Parkinson's Plus Syndrome; Striatonigral Degeneration; Sporadic Olivopontocerebellar Atrophy; MSA-P; MSA-C
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 wide spread 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. Symptoms of MSA include: reduced ability to show facial expressions; difficulty chewing or swallowing; disruptions in sleep patterns; dizziness or fainting (postural or orthostatic hypertension); frequent falls; incontinence; loss of fine motor skills; loss of ability to sweat; movement difficulties such as loss of balance, shuffling, gait changes; slow movement, difficulties with posture, and tremors. People with MSA may also experience vision changes, voice, and speech changes. The symptoms of MSA are very similar to Parkinson’s disease, but progress faster. The damage to the nervous system is more widespread in people with MSA. They generally do not experience the cognitive decline that is common among people with Parkinson’s disease. 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. People with MSA generally live 9 to 10 years after symptoms appear. There are no periods of remission from this disease.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
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.
ONSET AND PROGRESSION
People with MSA experience a progressive decline in mental and physical 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 lead to a person becoming bed dependent and death generally occur within 10 years or less after diagnosis.
There is no known treatment for MSA and no known way to prevent the disease from worsening. Treatment centers on controlling symptoms.
Management of symptoms may include:
Levodopa or carbidopa is prescribed to improve movement and balance.
Orthostatic hypotension (low blood pressure) may be treated with fludrocortisones, vasoconstrictors, and other drugs that raise blood pressure.
Cardiac arrhythmias can be managed with drugs and pacemakers.
An artificial feeding tube or breathing tube may be surgically inserted for the management of swallowing and breathing difficulties.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for Evaluation:
Clinical evaluation documenting symptoms of autonomic failure and response to treatment
Neurology consultation report
Activities of daily living report or a similar report completed by relative or caregiver
Suggested Listings for Evaluation:
MSA is most frequently seen in conjunction with Parkinson’s disease.
* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.