Resumo

PEDEMONTE, Virginia  e  CERISOLA, Alfredo. Síndrome de opsoclonus-mioclonus secundario a neuroblastoma torácico: Presentación de un caso clínico y discusión de nuevas estrategias terapéuticas. Arch. Pediatr. Urug. [online]. 2013, vol.84, n.3, pp.210-214. ISSN 1688-1249.

The opsoclonus - myoclonus syndrome or Kinsbourne syndrome is a rare disorder characterized by the presence of opsoclonus, myoclonus, ataxia, irritability and sleep disorders. It is associated with neuroblastoma in up to 45% of cases. It is an immune-mediated disorder and its treatment is based on immunosuppressive drugs, immunomodulatory therapies and surgery resection in cases with neuroblastoma. For many years, corticosteroids have been the gold standard treatment. Afterwards, corticosteroids have been associated with intravenous immunoglobulin. Due to high prevalence of corticosteroid dependence, adverse events and the poor neurological prognosis with more than 80 % of sequelaes in children, in the recent years protocols of treatment with multiple drugs have been proposed. The evidence supports the use of multimodal therapy with the addition of a third agent such as rituximab or cyclophosphamide. We report a case of a two- year-old boy with opsoclonus myoclonus syndrome secondary to thoracic neuroblastoma who was treated with corticosteroids, intravenous immunoglobulin, cyclophosphamide and surgery resection. He has had multiple relapses with the decrease dose of corticosteroids. After a year and six months from diagnosis he has recovered from motor symptoms, still treated with low doses of corticosteroids, but he has language impairment

Palavras-chave : OPSOCLONUS-MYOCLONUS SYNDROME; NEUROBLASTOMA; THORACIC NEOPLASMS.

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