Objective To explore the relationship,treatment and prognosis of opsoclonus-myoclonus-ataxia syndrome(OMAS)with Epstein-Barr virus(EBV)infection.Methods The clinical manifestations,auxiliary examinations,treatment and prognosis of one patient with OMAS were reported,and the relevant literature was reviewed and analysed to summarize the characteristics of OMAS cases after EBV infection.Results A total of 7 patients reported in 6 eligible publications were retrieved from Chinese and English databases,and 8 patients were included in the analysis when combined with the case reported here.Four of the 8 patients were of each sex,with onset ages ranging from 20 months to 71 years,and 4 were children.Five patients presented with a series of prodromal symptoms,such as fever,vomit,sore throat,rash,and lymph node enlargement,about 10 d to 2 weeks before the onset of OMAS.Only one of the eight patients did not have significant ophthalmoplegia during the course of the disease,and the remaining seven patients had the ophthalmoplegia-myoclonus-ataxia triad.Of the 8 patients,4 had positive serum viral capsid antigen(VCA)IgM,2 had positive early antigen(EA)IgG,and 1 had EBV DNA by PCR.Six patients underwent cerebrospinal fluid(CSF)examinations,of which 1 had positive EA IgG,1 had EBV DNA by PCR,and 6 showed normal sugar level.Protein was elevated in 2 patients[(0.53-0.73)g/L],and leukocytes were elevated in 3 patients[(11-20)× 106/L].Eight patients underwent head CT and/or MRI,and 2 patients underwent whole-body PET-CT,with no positive findings.Of the 8 patients,3 were treated with methylprednisolone,2 with immunoglobulin,1 with adrenocorticotropic hormone,and 2 without treatment.All patients recovered completely within 2-11 weeks after onset.Neuroblastoma was found 5 months after the onset of illness in one patient(20 months of age,without whole-body PET-CT)who was treated with adrenocorticotropic hormone.Conclusions OMAS can occur secondary to EBV infection,and imaging studies are usually normal in patients in the acute phase.The combination of prodromal symptoms and serological and cerebrospinal fluid tests specific for EBV could help to identify the cause of the disease.Immunosuppressive therapy is effective for OMAS after EBV infection.The prognosis of OMAS after EBV infection is generally good.
万方数据
维普
