Neuropaediatrics Department, CHU of Montpellier, Montpellier University, France.
Neuropaediatrics Department, CHU of Montpellier, Montpellier University, France.
Eur J Paediatr Neurol. 2022 Sep;40:18-27. doi: 10.1016/j.ejpn.2022.07.002. Epub 2022 Jul 13.
Dancing eye syndrome or opsoclonus-myoclonus syndrome (OMS) is a very rare disease (incidence <1/5,000,000 per year), which is more prevalent in young children. Although it is not usually a cause of mortality, the aftermaths are not rare.
We performed an observational retrospective review of children diagnosed with OMS in our neuropediatric department from 1996 to 2020, with the objective of assessing the prognostic value of initial clinical features. All medical data from diagnosis to last follow-up were reviewed. We defined unfavorable evolution of OMS as persistence or worsening of symptoms. Subsequently, based on a literature review, our results and experience, a diagnostic algorithm was developed.
A total of 13 OMS patients were included: 61.5% were male (n = 8), median age at diagnosis was 18 months (IR = 76), median treatment delay was 14 days (IR = 146) and OMS score at onset was 8 (IR = 11). The most frequent etiologies were neuroblastoma-associated and idiopathic OMS (38.46%; n = 5) of the patients, followed by post-infectious OMS (n = 3). All the patients were treated with corticosteroids, five required a surgical intervention (neuroblastoma group), and three required adjunctive immune therapy (immunoglobulins, cyclophosphamide and/or rituximab). We detected neurodevelopmental disorders in 38.46% (n = 5) of the patients, mainly attention deficit (n = 4), and persistent sleep disturbances (n = 4). The median OMS score at the end of follow-up was 1 (IR = 3). An important diagnostic delay, OMS score of ≥10 and age >1 year at onset may correlate with a higher risk of aftermaths. We detected a better prognosis in the post-infectious OMS, with full recovery occurring in 2/3 of patients.
Early clinical suspicion is key to guarantee maximum response of treatment.
舞蹈眼综合征或眼-肌阵挛-肌阵挛综合征（OMS）是一种非常罕见的疾病（发病率<1/500 万/年），更常见于幼儿。尽管它通常不是死亡的原因，但后遗症并不罕见。
我们对 1996 年至 2020 年在我们神经儿科诊断为 OMS 的儿童进行了观察性回顾性研究，目的是评估初始临床特征的预后价值。回顾了从诊断到最后随访的所有医疗数据。我们将 OMS 的不良演变定义为症状持续或恶化。随后，根据文献回顾、我们的结果和经验，制定了一个诊断算法。
共纳入 13 例 OMS 患者：61.5%为男性（n=8），诊断时中位年龄为 18 个月（IR=76），中位治疗延迟为 14 天（IR=146），发病时 OMS 评分为 8（IR=11）。最常见的病因是神经母细胞瘤相关和特发性 OMS（38.46%；n=5），其次是感染后 OMS（n=3）。所有患者均接受皮质类固醇治疗，5 例需要手术干预（神经母细胞瘤组），3 例需要辅助免疫治疗（免疫球蛋白、环磷酰胺和/或利妥昔单抗）。我们发现 38.46%（n=5）的患者存在神经发育障碍，主要是注意力缺陷（n=4）和持续睡眠障碍（n=4）。随访结束时 OMS 评分中位数为 1（IR=3）。重要的诊断延迟、OMS 评分≥10 和发病时年龄>1 岁可能与后遗症风险增加相关。我们发现感染后 OMS 的预后较好，2/3 的患者完全恢复。
早期临床怀疑是保证治疗反应最大化的关键。
