Li Honghua, Wang Bing, Shan Ling, Du Lin, Jia Feiyong
Department of Developmental and Behavioral Pediatrics, The First Hospital of Jilin University, Changchun, Jilin China.
Medicine (Baltimore). 2019 Jul;98(28):e16441. doi: 10.1097/MD.0000000000016441.
West syndrome (WS) is an age-dependent epileptic encephalopathy that is characterized by intractable epileptic seizures, hypsarrhythmia, and observed through electroencephalogram (EEG) and significant neurodevelopmental regression. The spontaneous remission of epileptic seizure is clinically rare and has not previously been reported in a Chinese infant. Herein, we reported a Chinese infant with WS whose seizures disappeared following a human herpesvirus 7 (HHV-7) infection.
The male Chinese infant was born at the gestational age of 36 weeks with a birth weight of 1.65 kg and an Apgar score of 7 at the first minute. At the age of 6 months, the infant developed seizures that manifested as flexor spasms with trunk involvement and mental regression.
Brain magnetic resonance imaging revealed leukomalacia of the posterior horn and a reduction in the size of the periventricular of the bilateral ventricle and the corpus callosum. An EEG revealed hypsarrhythmia and typical spasm seizures. Therefore, the infant was diagnosed with symptomatic WS.
The infant was treated with adequate vitamin B6 intravenous drip and oral treatment with topiramate and levetiracetam.
The observed seizures disappeared spontaneously 40 days after onset, without any changes in the anti-epileptic drug treatment, following a febrile rash due to a HHV-7 infection.
Spontaneous remission of epileptic seizures can occur following viral infection of HHV-7 in children with WS. The mechanism behind this spontaneous remission warrants further research.
韦斯特综合征(WS)是一种与年龄相关的癫痫性脑病,其特征为顽固性癫痫发作、高峰失律,可通过脑电图(EEG)观察到,且伴有明显的神经发育倒退。癫痫发作的自发缓解在临床上较为罕见,此前尚未有中国婴儿的相关报道。在此,我们报告了一名患有WS的中国婴儿,其癫痫发作在感染人类疱疹病毒7(HHV - 7)后消失。
这名中国男婴孕36周出生,出生体重1.65千克,1分钟时阿氏评分7分。6个月大时,该婴儿出现癫痫发作,表现为伴有躯干受累的屈肌痉挛和智力倒退。
脑部磁共振成像显示后角白质软化,双侧脑室和胼胝体周围脑室大小减小。脑电图显示高峰失律和典型的痉挛发作。因此,该婴儿被诊断为症状性WS。
该婴儿接受了足量维生素B6静脉滴注,并口服托吡酯和左乙拉西坦进行治疗。
在因HHV - 7感染出现发热性皮疹后,癫痫发作于发病40天后自发消失,抗癫痫药物治疗未作任何调整。
WS患儿感染HHV - 7病毒后癫痫发作可能会自发缓解。这种自发缓解背后的机制值得进一步研究。