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Spectrum of Movement Disorders Among Children With Subacute Sclerosing Panencephalitis: A Cross-Sectional Study
Journal of Child Neurology ( IF 1.9 ) Pub Date : 2022-03-09 , DOI: 10.1177/08830738221085158
Divyani Garg 1 , Vanshika Kakkar 2 , Ashna Kumar 2 , Dipti Kapoor 2 , Pooja Abbey 3 , Harish Pemde 2 , Sharmila B Mukherjee 2 , Suvasini Sharma 2
Affiliation  

Background: Subacute sclerosing panencephalitis is a progressive devastating condition due to persistence of mutant measles virus, affecting children and adolescents, characterised by myoclonus, seizures, and neuropsychiatric issues. Movement disorders apart from myoclonus are reportedly uncommon. We aimed to describe frequency and proportion of movement disorders among children with subacute sclerosing panencephalitis, hypothesizing that these occur more frequently than previously reported. Methods: In this cross-sectional study, we enrolled children with subacute sclerosing panencephalitis between 1 month and 18 years of age who fulfilled the diagnosis of subacute sclerosing panencephalitis as per modified Dyken criteria, and examined them for movement disorders. We also assessed their clinical profile and disease severity via Jabbour staging and modified Rankin Scale score. We compared demographic, clinical, and laboratory features of children with and without movement disorders. Results: We enrolled 50 children (36 males; 72%) (age range 1.5-14 years). Of these, 28 (56%) had movement disorders. Among movement disorders, the most frequent was myoclonus (92%), followed by ataxia (9; 18%), chorea-athetosis (7; 14%), dystonia (6; 12%), tremor (4; 8%), repetitive behavior (4; 8%), and parkinsonism (3; 6%). Movement disorders were the presenting feature of subacute sclerosing panencephalitis among 7 children. There were no significant differences in clinical or laboratory features among children with and without movement disorders. Conclusions: Movement disorders were frequent in subacute sclerosing panencephalitis. Hyperkinetic disorders were dominant. Dystonia and chorea-athetosis occurred more commonly among nonmyoclonus movement disorders. Movement disorders may manifest even in earlier stages of subacute sclerosing panencephalitis and may be the heralding feature. Recognition of these features is important to plan management and reduce morbidity.



中文翻译:

亚急性硬化性全脑炎患儿运动障碍谱:一项横断面研究

背景:亚急性硬化性全脑炎是一种进行性破坏性疾病,由于突变麻疹病毒的持续存在,影响儿童和青少年,其特征是肌阵挛、癫痫发作和神经精神问题。据报道,除了肌阵挛之外的运动障碍并不常见。我们旨在描述亚急性硬化性全脑炎患儿运动障碍的频率和比例,假设这些发生率比以前报道的更频繁。方法:在这项横断面研究中,我们招募了 1 个月至 18 岁的亚急性硬化性全脑炎儿童,这些儿童符合改良的 Dyken 标准诊断为亚急性硬化性全脑炎,并检查了他们的运动障碍。我们还通过 Jabbour 分期和改良的 Rankin 量表评分评估了他们的临床特征和疾病严重程度。我们比较了患有和不患有运动障碍的儿童的人口统计学、临床和实验室特征。结果:我们招募了 50 名儿童(36 名男性;72%)(年龄范围 1.5-14 岁)。其中,28 人(56%)患有运动障碍。在运动障碍中,最常见的是肌阵挛 (92%),其次是共济失调 (9; 18%)、舞蹈手足徐动症 (7; 14%)、肌张力障碍 (6; 12%)、震颤 (4; 8%)、重复行为 (4; 8%) 和帕金森症 (3; 6%)。运动障碍是7名儿童亚急性硬化性全脑炎的主要表现。有和没有运动障碍的儿童在临床或实验室特征方面没有显着差异。结论:运动障碍常见于亚急性硬化性全脑炎。多动障碍占主导地位。肌张力障碍和舞蹈手足徐动症在非肌阵挛性运动障碍中更常见。运动障碍甚至可能在亚急性硬化性全脑炎的早期阶段表现出来,并且可能是先兆特征。识别这些特征对于计划管理和降低发病率很重要。

更新日期:2022-03-09
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