Monomelic amyotrophy

MMA is characterized by progressive degeneration and loss of motor neurons—the nerve cells in the brain and spinal cord that are responsible for controlling voluntary muscles. It is characterized by weakness and wasting in a single limb, usually an arm and hand rather than a foot and leg. There is no pain associated with MMA.

MMA occurs in males between the ages of 15 and 25. Onset and progression are slow. MMA is seen most frequently in Asia, particularly in Japan and India; it is much less common in North America.

In most cases, the cause is unknown, although there have been a few published reports linking MMA to traumatic or radiation injury. There are also familial forms of MMA.

Diagnosis is made by physical exam and medical history. Other diagnostic tests may include:

• Electromyography (EMG), a special recording technique that detects electrical activity in muscles, shows a loss of the nerve supply, or denervation, in the affected limb
• MRI (magnetic resonance imaging) and CT (computed tomography) scans may show muscle atrophy

People believed to have MMA should be followed by a neuromuscular disease specialist for a number of months to make certain that no signs of other motor neuron diseases develop.

There is no cure for MMA. Treatment consists of muscle strengthening exercises and training in hand coordination.

The symptoms of MMA usually progress slowly for one to two years before reaching a plateau, and then remain stable for many years. Disability is generally slight. Rarely, the weakness progresses to the opposite limb.

There is also a slowly progressive variant of MMA known as O'Sullivan-McLeod syndrome, which only affects the small muscles of the hand and forearm and has a slowly progressive course.

How can I or my loved one help improve care for people with MMA?

Consider participating in a clinical trial so clinicians and scientists can learn more about MMA and related disorders. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.

All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.

For information about participating in clinical research visit NIH Clinical Research Trials and You. Learn about clinical trials currently looking for people with MMA at Clinicaltrials.gov.

Where can I find more information about MMA?

Information may be available from the following resource:

MedlinePlus

Content source: https://www.ninds.nih.gov/health-information/disorders/monomelic-amyotrophy Accessed June 23, 2023.

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