What’s multifocal motor neuropathy?

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Multifocal motor neuropathy is a rare autoimmune disease that causes progressive muscle weakness due to the immune system attacking motor nerve fibers. It is treatable with immunomodulatory therapy, and symptoms include cramps, muscle twitches, and reduced grip strength. It is often mistaken for ALS but progresses more slowly and is more common in males.

Multifocal motor neuropathy is a very rare progressive muscle disease that causes a progressive weakening of the sufferer’s muscles over a period of years. It is an autoimmune disease in which a person’s immune system begins to misidentify nerve cells as foreign organisms, causing the person’s motor nerve fibers and the myelin sheath that surrounds the nerves to attack. The damage begins to interfere with nerve transmissions to the muscles, resulting in a gradual decrease in strength. For reasons not yet understood, the damage is concentrated in the motor nerve fibers; sensory nerves are spared and thus multifocal motor neuropathy does not cause significant sensory damage.

The first signs of multifocal motor neuropathy are usually seen in the hands in the form of pulse drop, reduced dexterity, or reduced grip strength. Symptoms are often seen in muscles connected to the ulnar, radial, and median nerves of the arms and the common peroneal nerves of the legs. As multifocal motor neuropathy progresses, it causes increased muscle weakness and a decrease in muscle mass. The disease does not progress symmetrically, and a person may have symptoms in different muscles on different sides of the body.

The disorder is accompanied by cramps and muscle twitches, called fasciculations, which are not necessarily limited to the same areas of the body that suffer from muscle weakness. The sensation in the affected areas remains intact, although some sufferers experience tingling or numbness. The condition is rarely fatal, but if left untreated it can cause significant disability.

Multifocal motor neuropathy is sometimes mistaken for amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease. However, multifocal motor neuropathy does not progress as rapidly and, unlike ALS, is treatable. It is a rare condition, affecting about one in 100,000 people and is about three times more common in males than females. The ultimate cause of the immune system malfunction that causes the symptoms of multifocal motor neuropathy is unknown. Women with this condition sometimes experience worsening symptoms during pregnancy.

Multifocal motor neuropathy is treated with immunomodulatory therapy aimed at stopping the immune system from attacking the sufferer’s motor nerves. Intravenous immunoglobulin, a blood product containing densely concentrated antibodies from blood donors, is the most common treatment used for this purpose. The immunosuppressant cyclophosphamide, given intravenously, is also sometimes used, although it can have serious side effects and is usually used only in cases where treatment with intravenous immunoglobulin has proved ineffective.




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