Open AccessOpsoclonus-myoclonus syndrome in pedriatic patient from Campo Grande (MS): case report
Author(s) -
Mylena Delamare Espindola,
Thaynara Maria Maran de Souza,
Gabriel Loureiro Seleghim Boaventura,
Maria José Martins Maldonado
Publication year - 2021
Language(s) - English
Resource type - Conference proceedings
DOI - 10.5327/1516-3180.131
Subject(s) - medicine , myoclonus , pediatrics , hypotonia , context (archaeology) , opsoclonus , ataxia , encephalopathy , gait ataxia , anesthesia , psychiatry , paleontology , genetics , biology , cell culture , neuroblastoma
Context: Opsoclonys-myoclonus syndrome (OMS) is a rare neurologic disorder characterized by acute or chronic subcortical myoclonus and cerebellar ataxia at 6 months to 3 years-old children with rates of incidence expressed as 0.18 per 1.000.000 person- year. With nonspecific physiopathology, the only definitive finding is an elevated lymphocyte and positive B-cells count on the cerebrospinal fluid (≥11 cels/mm³) along with 50 to 93% cases reported from the National Pedriatric Myoclun Center (1989-2013) presenting oligoclonal bands. The diagnosis is established by clinical evaluation with exclusion criteria based on the presence of structural central nervous system damage and the aggressive treatment includes immunomodulatory therapy for behavior and cognitive stabilization. Case report: This paper aims to describe a case of a 1-year-old premature pediatrician patient presenting OMS in the absence of fetus distress due to pre- eclampsia condition with long-term hospitalization. After hypotonia, psychomotor agitation and vomit episodes, the patient was referred to Campo Grande (MS) where worsened to globus myoclonus, opsoclonus and nystagmus after 25 days of hospitalization, symptomatology responsive to Propranolol 10mg a day with regression of the clinical and neurological condition. Conclusion:Although OMS is a rare condition with variable prognosis, children appear to respond to pharmacological and non-pharmacological treatment improving the quality of life.