Profile and Outcome of Children with Opsoclonus Myoclonus Ataxia: A Tertiary Care Hospital Experience from India

IF 0.2 Q4 PEDIATRICS Journal of pediatric neurology Pub Date : 2022-03-12 DOI:10.1055/s-0042-1750762
Bidisha Banerjee, Ayesha Thanvi, S. M. Prabhu
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Abstract

Abstract Opsoclonus myoclonus ataxia syndrome (OMAS) is a rare but treatable, often paraneoplastic neuroimmunologic condition. This is a retrospective chart review of 8 patients diagnosed in the past 11 years at a tertiary care hospital. The mean age of children with OMAS was 21.2 ± 8 months. There was a female preponderance (62.5%). Median symptom duration was 24.5 days (interquartile range [IQR] 12.7; 97.5). All patients had ataxia and irritability; 6 had opsoclonus. An underlying neurogenic tumor was identified in 87.5% (⅞) of the patients by computed tomography (CT)/magnetic resonance imaging. Neuroblastoma was detected in ⅘ with normal 24-hour urinary vanillylmandelic acid and 2 had negative metaiodobenzylguanidine scan. All patients received adrenocorticotropic hormone/steroids for a median of 9.5 months (IQR 5.3; 13.5) with clonazepam. Five received intravenous immunoglobulin (IVIG), including repeated cycles in ⅘. Two received rituximab. One child with relapsing-remitting course received pulse dexamethasone and cyclophosphamide, resulting in improvement. Clonazepam restored ambulation in one with delayed diagnosis and failure of response to steroids. Six patients underwent tumor resection and four needed chemotherapies. Median follow-up was 15 months (IQR 10.7; 23.2). Mean OMAS-severity scale reduced from 10 to 1.4 in the IVIG group and 10.6 to 5.3 in those who did not receive IVIG. Cognitive delay and behavioral issues were seen in 100% treated with steroids only; 50 and 25%, respectively, treated with multimodal immunotherapy. Five had relapses, one with tumor recurrence. Thorax and abdomen CT scan was found to be a sensitive tool for tumor detection. Better motor and cognitive behavioral outcome were noted in patients who received adjunctive IVIG. Future studies on optimum investigation and treatment protocol in various resource settings are needed.
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眼阵挛性肌阵挛性共济失调儿童的概况和结局:来自印度三级医院的经验
摘要:眼阵肌阵性共济失调综合征(OMAS)是一种罕见但可治疗的副肿瘤神经免疫疾病。这是一个回顾性的图表回顾在过去11年确诊的8例患者在三级保健医院。患儿的平均年龄为21.2±8个月。女性为优势(62.5%)。中位症状持续时间为24.5天(四分位数间距[IQR] 12.7;97.5)。所有患者均有共济失调和烦躁;6例有斜视。87.5%(1 / 4)的患者通过计算机断层扫描(CT)/磁共振成像发现潜在的神经源性肿瘤。神经母细胞瘤2例24小时尿香草扁桃酸正常,2例甲氧苄基胍扫描阴性。所有患者接受促肾上腺皮质激素/类固醇治疗的中位时间为9.5个月(IQR 5.3;13.5)用氯硝西泮。5例患者静脉注射免疫球蛋白(IVIG),包括重复周期。其中两人接受了美罗华治疗。一名复发缓解的儿童接受了地塞米松和环磷酰胺脉冲治疗,结果有所改善。氯硝西泮恢复一个延迟诊断和类固醇反应失败的行走。6例患者行肿瘤切除术,4例需要化疗。中位随访时间为15个月(IQR 10.7;23.2)。IVIG组的平均omas严重程度从10降至1.4,未接受IVIG组的平均omas严重程度从10.6降至5.3。100%仅使用类固醇治疗的患者出现认知延迟和行为问题;分别有50%和25%的患者接受多模式免疫治疗。5例复发,1例肿瘤复发。胸腹CT扫描是一种灵敏的肿瘤检测工具。接受辅助IVIG的患者的运动和认知行为结果更好。需要进一步研究不同资源环境下的最佳调查和治疗方案。
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来源期刊
CiteScore
0.40
自引率
0.00%
发文量
52
期刊介绍: The Journal of Pediatric Neurology is a multidisciplinary peer-reviewed medical journal publishing articles in the fields of childhood neurology, pediatric neurosurgery, pediatric neuroradiology, child psychiatry and pediatric neuroscience. The Journal of Pediatric Neurology, the official journal of the Society of Pediatric Science of the Yüzüncü Yil University in Turkiye, encourages submissions from authors throughout the world. The following articles will be considered for publication: editorials, original and review articles, rapid communications, case reports, neuroimage of the month, letters to the editor and book reviews.
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