Reversible splenial lesion syndrome in children: Retrospective study and summary of case series
Reversible splenial lesion syndrome in children: Retrospective study and summary of case series
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DOI：
10.1016/j.braindev.2016.04.011
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发表时间：
2016-11-01
影响因子：
1.7
通讯作者：
Mai, Jian-Ning
中科院分区：
文献类型：
--
作者：
Chen, Wen-Xiong;Liu, Hong-Sheng;Mai, Jian-Ning
目的：描述小儿可逆性脾损害综合征（RESLES）的临床特点。方法：回顾性分析RESLES患儿的临床特点，比较重度组与非重度组之间的差异，采用临床总体印象量表进行分类;总结病例系列中轻度脑炎/脑病伴可逆性脾损害（MERS）患儿的临床特点。结果：分析了15名中国儿童发生的16次RESLES，其中13次为MERS，3次为癫痫。10次发作与各种病原体相关，包括轮状病毒（n = 5）、腺病毒（n = 1）、甲型流感病毒（n = 1）、支原体（n = 2）和空肠弯曲菌（n = 1）。常见的神经系统症状包括癫痫发作、行为改变、意识改变和运动功能减退。胼胝体压部（SCC）、非SCC（n = 2）和非CC（n = 1）病灶T2加权像和FLAIR信号均为高信号，弥散度减低。除1次发作有少量残留外，所有患者的放射学变化均完全消退。8次发作有EEG异常，同时通常显示白色血细胞计数升高、hs-CRP升高和低钠血症。7例给予激素治疗，3例给予抗癫痫药物治疗。与非重症组相比，重症组中意识改变、EEG异常、运动功能减退或SCC外病变的患者数量显著增加。重症组住院时间较长。除重度组1例复发性CC外病灶伴智力障碍外，其余无神经系统后遗症。总结了5个儿科MERS病例系列，包括67次发作（40例男性和27例女性;年龄范围10 m与13 y相似）来自65例患者，其中33例发生在日本，27例发生在中国，7例发生在澳大利亚白人儿童，除1例ID患者外，所有患者均预后良好结论：儿童RESLES预后较好，但重症组，尤其是CC外病变，预后可能有神经系统后遗症。(C)2016日本儿童神经病学学会。Elsevier B. V.出版，保留所有权利。
Objective: To describe clinical features of reversible splenial lesion syndrome (RESLES) in children.Methods: Retrospectively analyzed clinical features of RESLES in children and compared differences between severe and non severe group, classified by clinical global impression-scale; summarized clinical features of children with mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) from case series.Results: 16 episodes of RESLES occurring in 15 Chinese children were analyzed, with 13 episodes having MERS and 3 episodes with epilepsy. 10 episodes were associated with various pathogens including rotavirus (n = 5), adenovirus (n = 1), influenza A (n = 1), mycoplasma (n = 2), and jejunum campylobacter (n = 1). The common neurological symptoms included seizure, behavioral changes, altered consciousness and motor deterioration. The lesions of splenium of corpus callosum (SCC), extra-SCC (n = 2) or extra-CC (n = 1) showed T2-weight and FLAIR hyper-intensity, with the corresponding reduced diffusion. All had complete resolution of radiological changes except 1 episode with small residual. 8 episodes had EEG abnormalities, while elevated white blood count, increased hs-CRP, and hyponatremia were commonly revealed. 7 episodes were given steroid plus therapy, while 3 episodes were treated with antiepileptic drugs. Compared with non-severe group, the number of patients with altered consciousness, EEG abnormalities, motor deterioration, or extra-SCC lesions in severe group was significantly increased. The patients in severe group tended to need longer hospital stay interval. No case caused neurological sequelae, except 1 patient in severe group with recurrent episode and extra-CC lesions having intellectual disability (ID). Five pediatric MERS case series were summarized, including 67 episodes (40 male and 27 female; age ranging 10 m similar to 13 y) from 65 patients, with 33 episodes in Japan, 27 in China, and 7 in Caucasian Australian children, and all patients have a good prognosis except 1 patient with ID (current study).Conclusion: Although RESLES in children tend to be a good outcome, the prognosis of patient in severe group, especially with extra-CC lesions, might have neurological sequelae. (C) 2016 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.