Meena Jagdish P, Seth Rachna, Chakrabarty Biswaroop, Gulati Sheffali, Agrawala Sandeep, Naranje Priyanka
Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
J Pediatr Neurosci. 2016 Oct-Dec;11(4):373-377. doi: 10.4103/1817-1745.199462.
The opsoclonus-myoclonus ataxia syndrome (OMAS) also called "Kinsbourne syndrome" or "dancing eye syndrome" is a rare but serious disorder characterized by opsoclonus, myoclonus, and ataxia, along with extreme irritability and behavioural changes. Data on its epidemiology, clinical features, and outcome are limited worldwide. The aim of the study was to evaluate the clinical profile and outcome of children with OMAS. A retrospective data of all children presented to Pediatric oncology clinic with a diagnosis of opsoclonus-myoclonus from 2013 to 2016 were collected. 6 patients with a diagnosis of OMAS were presented over a 4-year period. All 6 cases had paraneoplastic etiology. All Children had good outcome without any relapse. Paraneoplastic opsoclonus myoclonus had a good outcome in our experience.
眼阵挛-肌阵挛共济失调综合征（OMAS）也被称为“金布尔综合征”或“舞动眼综合征”，是一种罕见但严重的疾病，其特征为眼阵挛、肌阵挛和共济失调，同时伴有极度易怒和行为改变。全球关于其流行病学、临床特征和预后的数据有限。本研究的目的是评估OMAS患儿的临床特征和预后。收集了2013年至2016年在儿科肿瘤门诊诊断为眼阵挛-肌阵挛的所有患儿的回顾性数据。在4年期间共出现6例诊断为OMAS的患者。所有6例均有副肿瘤病因。所有患儿预后良好，无任何复发。根据我们的经验，副肿瘤性眼阵挛-肌阵挛预后良好。
