What’s opsoclonus-myoclonus syndrome?

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Opsoclonus-myoclonus syndrome is an autoimmune disease that attacks the brain, causing uncontrollable twitching of the eyes and muscles, muscle spasms, lack of coordination, and encephalopathy. It often follows a viral infection or tumor growth and is treated with ACTH shots, immunoglobulins, chemotherapy, and steroid therapy.

The rare neurological disorder called opsoclonus-myoclonus syndrome is an autoimmune disease that attacks the brain, often after a viral infection. Also known as “dancing eyes/dancing feet” or Kinsbourne syndrome, this disorder can affect people of any age, initially manifesting as twitching of the eyes and muscles. Eventually, standing or walking becomes tiring, as does talking, eating, or thinking clearly.

Opsoclonus-myoclonus syndrome is characterized by four separate conditions, which can appear almost instantly. Opsoclonus refers to uncontrollable twitching of the eyes, causing a dancing effect. Myoclonus involves muscle spasms that come and go, affecting the whole body. Also common for those suffering from this syndrome is ataxia, a general lack of muscle coordination that hinders the most common movements, from walking to eating. Finally, encephalopathy prevents the brain from thinking clearly. All combine to create a patient who cannot control movement, is lethargic, and often filled with rage.

According to the National Pediatric Myoclonus Center, opsoclonus-myoclonus syndrome often follows after the body has battled a tumor growth or become infected with various viral infections. Tumors are found in about half of the patients who suffer from it, and viral infections have recently been treated in many of the others. While the exact cause of this disorder is unknown as of 2011, many researchers believe that antibodies produced by the body’s immune system may inadvertently attack brain cells in an attempt to rid the body of an invading tumor or virus.

Doctors who treat opsoclonus-myoclonus syndrome first try to determine if a tumor is present by ordering a series of X-ray tests. If a tumor is found, they will try to remove it. This often eradicates the syndrome in children, as the body no longer senses an intrusive presence. For adults, however, the condition is more likely to continue even after a tumor is removed.

Whether or not a tumor is found, doctors routinely prescribe a regimen of ACTH shots to treat opsoclonus-myoclonus syndrome. In a series of injections over 20 days, this hormone causes the body’s adrenal gland to produce more cortisol, which translates into an 80 to 90 percent recovery rate for children, with a lower for adults.

Other medications might be prescribed to push this syndrome into remission. Intravenous immunoglobulins are human antibodies that can enhance the body’s immune response. Chemotherapy and steroid therapy are also commonly recommended. Although most patients recover, many are susceptible to relapse when the body experiences a new disease.




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