ALTERNATE NAMES

Duchenne–Aran Muscular Atrophy; PMA

DESCRIPTION

Progressive Muscular Atrophy (PMA) is a rare, sporadic, adult-onset motor neuron disease. PMA is a clinical disorder characterized by degeneration of lower motor neurons. These types of motor neurons directly innervate skeletal muscles and control voluntary movements.

Motor neuron diseases are a group of disorders characterized by progressive damage to an individual’s motor neurons, the cells in the nervous system that allow an individual to perform functions such as speech, breathing, and movement.

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

Diagnostic testing: Diagnostic testing for PMA may include:

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  Physical examination;

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  Medical and family history;

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  Blood work;

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  Cerebrospinal fluid analysis;

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  Computed tomography (CT) scans;

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  Electromyography (EMG);

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  Lumbar puncture;

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  Magnetic resonance imaging (MRI) scans;

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  Muscle and nerve biopsies;

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  Nerve conduction velocity (NCV) test; and

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  Urinalysis.

Physical findings: Signs and symptoms of PMA include:

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  Difficulty with motor function leading to clumsiness;

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  Difficulty breathing;

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  Inability to use the arms (flail arm syndrome);

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  Muscle cramps or pain;

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  Muscle twitching;

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  Unexplained weight loss; and

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  Weakness in hands, arms, core, or legs.

ICD-9: 335.29

ICD-10: G11.4; G12.1; G12.25

PROGRESSION

PMA is progressive, which means it will worsen over time. It often progresses very gradually and may affect only one part of the body for several years. PMA is fatal. However, the length of time an individual will live with the disease fluctuates. The life expectancy averages 5 to 10 years.

Individuals with PMA eventually develop upper motor neuron damage, which more resembles an amyotrophic lateral sclerosis (ALS) diagnosis.

TREATMENT

There is no cure for PMA. Treatment is designed to slow down the damage to maintain a good quality of life for as long as possible.

Although there is no standard treatment for PMA, doctors work with patients to develop a treatment plan to help manage symptoms. Treatments may include medication, nutritional counseling, physical therapy, and occupational therapy.

Doctors may prescribe Riluzole, a medication typically used for ALS, to slow down damage to motor neurons. Other medications are used to treat pain, muscle cramping, and twitching.

Suggested MER for Evaluation:

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  Clinical history and examination that describes the diagnostic features of the impairment;

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  Results of imaging (CT scan and MRI);

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  Results of EMG and NCV testing; and

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  Results of lumbar puncture and muscle and nerve biopsy.

Suggested Listings for Evaluation:

Meets

11.22

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