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Opsoclonus Syndrome: A Case Study

Decent Essays

Opsoclonus-Myoclonus Syndrome (OMS) is a rare pediatric inflammatory autoimmune disease of the brain, affecting 1:10,000,000 worldwide (Gorman, Pike, Tardieu, 2014). There is no cure; no data from animal testing, and it is so rare that often is misdiagnosed. The FDA has yet to establish a standard of care. This research paper will outline how OMS is diagnosed, the demographics of patients diagnosed with OMS, parts of the body affected, and treatment options. This researcher picked this disease by personally experiencing and witnessing the effects of this disease on a child from birth to the age of nine. For the purpose of this research paper, this patient will be referred to as “Patient C.”
OMS is often referred to as “Dancing eyes, dancing …show more content…

If there is a tumor present in the patient, it will be surgically removed (if possible, depending on location). If surgical resection is not possible, chemotherapy and/or radiation will be used to target the tumor. Steroid use has been determined as effective, however, some children become steroid dependent and symptoms may reoccur once treatment has been completed. The treatment with the best-documented outcomes is referred to as FLAIR therapy, which is a three-agent protocol involving front-loading high dosages of ACTH (Adrenocorticotropin), IVIG (Intravenous Immunoglobulin), and Rituximab (monoclonal antibody). Early and sufficient immunotherapy is vital for treatment. The goal for any treatment regarding OMS is complete neurological remission (Pranzetelli, 2015).
Relapses are common in patients with OMS. With each relapse, attacks on the brain increase dramatically, resulting in further permanent brain damage. With each relapse, treatment for the patient starts at the beginning stages of their therapy (S. Berkley, personal communication, December 1, 2006). This brings us to Patient C, and his case …show more content…

He has experienced a couple of major relapses, and dozens of minor relapses. With each relapse, the potential for permanent brain damage increases (S. Berkley, personal communication, December 1, 2006).
Repeated CT scans have continually proved negative for tumors, so the leading theory for causing a triggering of his OMS is attributed to the four live vaccines that were administered at his year one immunizations. This is somewhat controversial but has been confirmed by Dr. Pranzetelli and Patient C’s family to be the most likely cause (S. Berkley, personal communication, December 1, 2006).
To date, Patient C has had approximately 91 IVIG treatments, 6 rounds of chemotherapy, 5 years worth of steroid shots, 9 spinal taps, numerous x-ray swallow studies, CT scans, and MIBG scans, and he just finished having his 3rd porta catheter placed (S. Berkley, personal communication, December 1,

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