Resumo

DANIELS-GARCIA, María José; MOLINARES-NUNEZ, Liliana Patricia; MUNOZ-ALVAREZ, Nelson Armando  e  BARRIOS-REDONDO, Katherine. Idiopathic opsoclonus myoclonus syndrome: Case report in a 13-month-old female patient. Medicas UIS [online]. 2022, vol.35, n.2, e502.  Epub 03-Dez-2022. ISSN 0121-0319.  https://doi.org/10.18273/revmed.v35n2-2022004.

Opsoclonus myoclonus syndrome is a rare neurological entity affecting preschool children. Clinically it is characterized by a classic triad of opsoclonus, myoclonus, and acute ataxia, with a progressive or even incomplete course. Its etiology can be paraneoplastic, in most cases in association with neuroblastomas, as well as postinfectious or parainfectious, autoimmune or idiopathic. The goal of treatment is immunomodulation with first-line therapy with intravenous steroids, although it can be associated with relapses and long-term neurological and behavioral sequelae. The opsoclonus myoclonus syndrome represents a diagnostic challenge in patients with acute ataxia given the variety of clinical presentations, therefore it is important to have a high diagnostic suspicion to ensure timely treatment and aoid future sequelae.

Palavras-chave : Opsoclonus-myoclonus syndrome; Children; Ataxia.

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