Department of Neurology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders (Chongqing), International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, China.
Brain Behav. 2021 Apr;11(4):e02051. doi: 10.1002/brb3.2051. Epub 2021 Feb 5.
The clinical features and outcomes of subacute sclerosing panencephalitis (SSPE) in younger children are different from those of adults, leading easily to misdiagnosis during the early stage. So far, there are limited data related to SSPE in preschool children.
In order to summarize the clinical data and evolution of SSPE in preschool children and to expand the phenotypes of SSPE, the medical charts of preschool patients diagnosed with SSPE were retrospectively reviewed and analyzed; the clinical outcomes of the enrolled cases were evaluated and followed up.
Overall, we included three cases in the study. Their onset age was 5 years and 2 months, 4 years and 3 months, and 4 years and 2 months, respectively. All patients presented drop attacks or jerks as the onset symptom, and one patient had concurrent gait disturbance. Atypical periodic complexes on electroencephalography (EEG) were recorded in all patients. The brain magnetic resonance imaging (MRI) findings of two cases showed demyelinating lesions predominantly on the white matter. The neurological conditions of all cases deteriorated rapidly. Two children died at 21 months and 6 months after onset, respectively. The other case progressively developed vegetative status and akinetic mutism within 4 months.
In younger children, the characteristic features of SSPE may be seizures and gait instability as onset manifestations, atypical periodic complexes on EEG, and rapid worsening of neurological conditions.
亚急性硬化性全脑炎（SSPE）在幼儿中的临床特征和结局与成人不同，这导致在早期阶段容易误诊。到目前为止，关于学前儿童 SSPE 的数据有限。
为了总结学前儿童 SSPE 的临床数据和演变，并扩展 SSPE 的表型，我们回顾性分析了诊断为 SSPE 的学前患者的病历；评估和随访了纳入病例的临床结局。
总的来说，我们研究中包括了 3 例患者。他们的发病年龄分别为 5 岁 2 个月、4 岁 3 个月和 4 岁 2 个月。所有患者均以跌倒发作或抽搐为首发症状，1 例患者伴有步态障碍。所有患者的脑电图（EEG）均记录到不典型周期性复合波。2 例患者的脑磁共振成像（MRI）显示以白质为主的脱髓鞘病变。所有病例的神经状况均迅速恶化。2 例患儿分别在发病后 21 个月和 6 个月死亡。另 1 例患儿在 4 个月内逐渐发展为植物状态和无动性缄默症。
在幼儿中，SSPE 的特征可能是癫痫发作和步态不稳为首发表现，脑电图上出现不典型周期性复合波，以及神经状况迅速恶化。
