Department of Neurology, 28856Lady Hardinge Medical College, New Delhi, India.
Department of Pediatrics (Neurology Division), 28856Lady Hardinge Medical College, New Delhi, India.
J Child Neurol. 2022 May;37(6):491-496. doi: 10.1177/08830738221085158. Epub 2022 Mar 9.
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. 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. 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. 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.
亚急性硬化性全脑炎是一种由突变麻疹病毒持续存在引起的进行性破坏性疾病,影响儿童和青少年,其特征为肌阵挛、癫痫发作和神经精神问题。除肌阵挛外,运动障碍据报道并不常见。我们旨在描述亚急性硬化性全脑炎患儿中运动障碍的频率和比例,假设这些运动障碍比以前报道的更为常见。 在这项横断面研究中,我们招募了符合改良 Dyken 标准的亚急性硬化性全脑炎诊断标准的 1 个月至 18 岁的亚急性硬化性全脑炎患儿,并对他们进行了运动障碍检查。我们还通过 Jabbour 分期和改良 Rankin 量表评分评估了他们的临床特征和疾病严重程度。我们比较了有和无运动障碍的患儿的人口统计学、临床和实验室特征。 我们共招募了 50 名儿童(36 名男性;72%)(年龄 1.5-14 岁)。其中,28 名(56%)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 例;18%)、舞蹈手足徐动症(7 例;14%)、肌张力障碍(6 例;12%)、震颤(4 例;8%)、重复行为(4 例;8%)和帕金森病(3 例;6%)。7 名患儿以运动障碍为亚急性硬化性全脑炎的首发症状。有和无运动障碍的患儿在临床或实验室特征方面无显著差异。 运动障碍在亚急性硬化性全脑炎中较为常见。多动障碍占主导地位。非肌阵挛性运动障碍中更常见的是肌张力障碍和舞蹈手足徐动症。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,并且可能是首发症状。认识到这些特征对于计划管理和降低发病率很重要。