Resumen

ZUNIGA-AMAYA, Bency Danilo; RESTREPO-JIMENEZ, Juan Pablo  y  ROJAS-CERON, Christian Andrés. Opsoclonus-mioclonus-ataxia syndrome associated with a mature ovarian teratoma in a pediatric patient. CES Med. [online]. 2019, vol.33, n.3, pp.208-214. ISSN 0120-8705.  https://doi.org/10.21615/cesmedicina.33.3.6.

The opsoclonus-myoclonus-ataxia syndrome is a rare entity that presents with motor, neurocognitive and psychiatric symptoms, often markedly de bilitating. The syndrome is reported more frequently in adults than in chil dren, the etiology is varied, but in pediatrics it occurs in most cases as a paraneoplastic syndrome. In this context, the most frequently associated neoplasm is neuroblastoma followed by gynecological tumors. The current evidence supports the thesis that this is an immune-mediated syndrome because a series of circulating autoantibodies has been described in the affected patients, in addition to many of them responding to immuno suppressive therapy. The importance of recognizing this syndrome is that there is medical/surgical treatment available that could improve the neu rological and psychiatric prognosis. Next, we present the case of a patient who presented with this syndrome in our institution.

Palabras clave : Neoplasms; Teratoma; Encephalitis; Plasmapheresis; Opso clonus-myoclonus-ataxia syndrome.

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