Clinical diagnosis and treatment of 9 childhood opsoclonus-myoclonus syndrome patients and application of standard rating scale
10.3760/cma.j.cn113694-20210713-00490
- VernacularTitle:儿童眼球阵挛-肌阵挛综合征9例临床诊治与标准化分级量表的应用
- Author:
Qianqian TANG
1
;
Xinhua WANG
;
Kai LI
;
Shuizhen ZHOU
Author Information
1. 国家儿童医学中心 复旦大学附属儿科医院神经科，上海201102
- Keywords:
Myoclonus;
Ocular motility disorders;
Neuroblastoma;
Children;
Prognosis;
Standard rating scale;
Opsoclonus-myoclonus syndrome
- From:
Chinese Journal of Neurology
2022;55(3):223-228
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To summarize the clinical features of childhood opsoclonus-myoclonus syndrome (OMS), evaluate severity degree and prognosis using OMS Symptom Severity Standard Rating Scale.Methods:The clinical features,diagnosis, therapeutic regimen and follow-up of 9 children with OMS in Department of Neurology and Oncology Department of Children's Hospital of Fudan University between 2011 and 2019 were retrospectively reviewed. Severity degree and prognosis were evaluated using OMS Symptom Severity Standard Rating Scale.Results:Among the 9 children with OMS, 4 were males and 5 were females. The onset age ranged from 14 months to 5 years, with a median of 17 months. Main symptoms were opsoclonus, myoclonus, ataxia, motor function regression, behavior and mood changes and sleep disorders. Eghit patients were combined with tumor. Seven of them with neuroblastoma were confirmed by surgical pathology, the other one showed spontaneous regression and symptom remission without treatment. Eight patients received hormone combined with intravenous immunoglobulin, of which 3 cases received adrenocorticotropic hormone, while 5 cases received methylprednisolone pulse therapy, with prednisone sequential therapy, decreasing dose gradually. Rituximab was administrated in 3 patients whose annual recurrent time was≥2. Nine patients were divided into 3 groups according to OMS Symptom Severity Standard Rating Scale, 1 of mild degree, 3 of moderate degree, 5 of severe degree. There was no recurrence and sequelae in the mild-degree patient after 3 years follow-up. There was no recurrence and sequelae in 2 of the moderate-degree patients after 1-2 years follow-up. Different degrees of motor, cognitive sequelae were found in 1 of moderate-degree patients and all of severe-degree patients after 1-8 years follow-up.Conclusions:Childhood OMS patients have early onset age. Bad symptom severity before treatment and multiphase course are associated with poor prognosis. OMS Symptom Severity Standard Rating Scale is suitable for estimations of long-term prognosis.
