Muthusamy Karthik, Thomas Maya, Yoganathan Sangeetha, Sudhakar Sniya Valsa
Department of Neurological Sciences, Pediatric Neurology Division, Christian Medical College, Vellore, Tamil Nadu, India.
Department of Radio Diagnosis, Christian Medical College, Vellore, Tamil Nadu, India.
Ann Indian Acad Neurol. 2019 Jul-Sep;22(3):295-301. doi: 10.4103/aian.AIAN_101_18.
Opsoclonus myoclonus syndrome (OMS) is a neuroinflammatory disorder. Indian literature on its clinical profile and outcome is sparse.
The objective of this study is to describe the clinical profile and analyze outcomes and prognostic predictors in a cohort of children with OMS.
This was a retrospective study of children with OMS between 2007 and 2017.
Twenty-two children were included in the study. The mean age at onset of symptom was 20.9 months (standard deviation [SD]: 7.5). The mean duration of delay in diagnosis was 8.4 months (SD 1.26) with acute cerebellitis being the most common misdiagnosis. Eleven children (50%) were diagnosed with tumor during evaluation and follow-up and 11 children (50%) belonged to idiopathic/postinfectious group. Magnetic resonance imaging brain was normal in all children except for one revealing cerebellar atrophy on follow-up. One child in the paraneoplastic group (neuroblastoma) had a positive PNMA2/Ta onconeural antibody. Children in the tumor group had an earlier age of onset (mean 15.5 vs. 26.3 months), shorter time to onset of opsoclonus from initial symptom (2.54 vs. 7.27 weeks), and higher severity score at presentation (13.7 vs. 11.3) compared to the nontumor group. Children in the nontumor group attained their first remission with treatment earlier (10.9 weeks, SD: 4.5) than the children with tumor (18.72 weeks, SD: 5.8). There was no significant difference in the outcome between the groups. Children with multiple relapses (>3) and late surgical intervention for tumor (>6 months after symptom onset) had a poor outcome.
A high index of suspicion coupled with early diagnosis and periodic tumor surveillance (even in the initially negative cases) along with aggressive combined multimechanistic immunotherapies is the key in improving outcomes.
A high index of suspicion in appropriate clinical circumstances and early aggressive immunomodulation might lead to a better outcome.
眼阵挛-肌阵挛综合征（OMS）是一种神经炎症性疾病。印度关于其临床特征和预后的文献较少。
本研究的目的是描述OMS患儿的临床特征，并分析其预后及预后预测因素。
这是一项对2007年至2017年间OMS患儿的回顾性研究。
22名儿童纳入研究。症状出现时的平均年龄为20.9个月（标准差[SD]：7.5）。诊断延迟的平均持续时间为8.4个月（SD 1.26），最常见的误诊为急性小脑炎。11名儿童（50%）在评估和随访期间被诊断为肿瘤，11名儿童（50%）属于特发性/感染后组。除1名儿童随访时显示小脑萎缩外，所有儿童的脑部磁共振成像均正常。副肿瘤组中的1名儿童（神经母细胞瘤）PNMA2/Ta肿瘤相关抗体呈阳性。与非肿瘤组相比，肿瘤组患儿发病年龄更早（平均15.5个月对26.3个月），从最初症状到出现眼阵挛的时间更短（2.54周对7.27周），就诊时严重程度评分更高（13.7对11.3）。非肿瘤组患儿治疗后首次缓解时间早于肿瘤组患儿（10.9周，SD：4.5）（18.72周，SD：5.8）。两组间预后无显著差异。多次复发（>3次）且肿瘤手术干预较晚（症状出现后>6个月）的患儿预后较差。
提高怀疑指数，早期诊断并定期进行肿瘤监测（即使最初检查结果为阴性），同时积极联合多种机制的免疫治疗是改善预后的关键。
在适当的临床情况下提高怀疑指数并早期积极进行免疫调节可能会带来更好的预后。
