Treating opsoclonus myoclonus syndrome – pro

Opsoclonus Myoclonus Syndrome (OMS), also known as Opsoclonus-Myoclonus-Ataxia (OMA), is a rare neurological disorder of unknown causes which appears to be the result of an autoimmune process involving the nervous system. It is an extremely rare condition, affecting as few as 1 in 10,000,000 people per year. It affects 2 to 3% of children with neuroblastoma. It is paraneoplastic syndrome in thos cases. ACTH has shown improvements in symptoms but can result in an incomplete recovery with residual deficits. FLAIR therapy is a three-agent protocol involving front-loaded high-dose ACTH (corticotropin), IVIg, and rituximab that was developed by the National Pediatric Myoclonus Center for pediatric OMS, and has the best-documented outcomes for moderately severe and severe cases.

Pike M. Opsoclonus-myoclonus syndrome. Handb Clin Neurol 2013;112:1209-11.

Pranzatelli M, Chun K, Moxness M, Tate E, Allison T. Cerebrospinal fluid ACTH and cortisol in opsoclonus-myoclonus: effect of therapy. Pediatr Neurol. 2005;33:121-126.

Tate ED, Pranzatelli MR, Verhulst SJ, et al. Active comparator-controlled, rater-blinded study of corticotropin-based immunotherapies for opsoclonus-myoclonus syndrome. J Child Neurol. 2012 Jul;27(7):875-84.

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