Singhi Pratibha, Sahu Jitendra Kumar, Sarkar Jhuma, Bansal Deepak
1Pediatric Neurology Division, Department of Pediatrics, Post-Graduate Institute of Medical Education & Research, Chandigarh, India.
J Child Neurol. 2014 Jan;29(1):58-61. doi: 10.1177/0883073812471433. Epub 2013 Jan 14.
The opsoclonus-myoclonus syndrome is a distinct disorder characterized by opsoclonus, myoclonus, and ataxia, along with marked irritability and behavioral changes. Worldwide, data on its epidemiology, clinical features, and outcome are scarce. The aim of the study was to determine the clinical profile and outcome of children with this disorder. A retrospective study of all children admitted with a diagnosis of opsoclonus-myoclonus from 2000 to 2012 was done. Outcome was assessed on follow-up by direct assessment and by telephonic interview. Eleven patients with a diagnosis of opsoclonus-myoclonus were admitted over a 12-year period. Of the 11, 4 had paraneoplastic etiology. Children with paraneoplastic opsoclonus had more relapses and a poor outcome as compared to an idiopathic group. Paraneoplastic opsoclonus had a poor outcome in our experience.
眼阵挛-肌阵挛综合征是一种独特的疾病，其特征为眼阵挛、肌阵挛和共济失调，同时伴有明显的易激惹和行为改变。在全球范围内，关于其流行病学、临床特征和预后的数据都很稀少。本研究的目的是确定患有这种疾病的儿童的临床特征和预后。对2000年至2012年期间所有诊断为眼阵挛-肌阵挛而入院的儿童进行了一项回顾性研究。通过直接评估和电话访谈对随访时的预后进行评估。在12年期间，有11例诊断为眼阵挛-肌阵挛的患者入院。其中11例中，4例有副肿瘤病因。与特发性组相比，副肿瘤性眼阵挛的儿童复发更多且预后较差。根据我们的经验，副肿瘤性眼阵挛预后不良。
