Acute encephalopathy in children with tuberous sclerosis complex.
Acute encephalopathy in children with tuberous sclerosis complex.
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DOI：
10.1186/s13023-020-01646-8
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发表时间：
2021-01-06
影响因子：
3.7
通讯作者：
Okumura A
中科院分区：
文献类型：
--
作者：
Numoto S;Kurahashi H;Sato A;Kubota M;Shiihara T;Okanishi T;Tanaka R;Kuki I;Fukuyama T;Kashiwagi M;Ikeno M;Kubota K;Akasaka M;Mimaki M;Okumura A
我们研究了急性脑病(AE)的临床表现，并确定了结节性硬化症(TSC)儿童发生AE的危险因素。收集13家医院11例临床诊断为TSC合并AE的儿童和109例4岁及以上的临床诊断为TSC的儿童的临床资料。11例癫痫患儿中，5例有发热性惊厥(FS)史，均有发热性癫痫持续状态(FSE)史。所有癫痫发作持续30分钟或更长时间的儿童在发烧后24小时内发生AE。所有儿童在惊厥停止后均出现昏迷。头部核磁共振成像(MRI)显示大脑皮质、皮质下白质、穹窿体部、基底节和丘脑广泛异常。一个孩子死亡；七个孩子有严重的神经后遗症；另外三个孩子有轻微的后遗症。Logistic回归分析显示，FSE病史与AE的发生有关。儿童TSC的AE的特点是发热后突然发病，然后昏迷，MRI上可见广泛的脑水肿，预后差。FSE病史是AE发生的危险因素。
We examined the clinical manifestations of acute encephalopathy (AE) and identify risk factors for AE in children with tuberous sclerosis complex (TSC). The clinical data of 11 children with clinically diagnosed TSC associated with AE and 109 children with clinically diagnosed TSC alone aged 4 years or older were collected from 13 hospitals. Of the 11 children with AE, 5 had histories of febrile seizures (FS), and all had histories of febrile status epilepticus (FSE). AE developed within 24 h after fever onset in all children with seizures lasting 30 min or longer. All children developed coma after seizure cessation. Head magnetic resonance imaging (MRI) revealed widespread abnormalities in the cerebral cortex, subcortical white matter, corpus callosum, basal ganglia, and thalamus. One child died; seven had severe neurological sequelae; and the other three, mild sequelae. Logistic regression analysis revealed that a history of FSE was correlated with the development of AE. AE in children with TSC was characterized by sudden onset after fever, followed by coma, widespread brain edema evident on MRI, and poor outcomes. A history of FSE was a risk factor for the development of AE.
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结节性硬化症复杂诊断标准更新：2012年iinternation Tuberous硬化症复杂共识会议的建议。
影响因子：
3.8
作者：
Northrup H;Krueger DA;International Tuberous Sclerosis Complex Consensus Group
通讯作者：
International Tuberous Sclerosis Complex Consensus Group
对医疗统计信息的自由使用的易于使用的软件“ EZR”的调查。
影响因子：
4.8
作者：
Kanda Y
通讯作者：
Kanda Y
影响因子：
2.6
作者：
Brigo, Francesco;Nardone, Raffaele;Trinka, Eugen
通讯作者：
Trinka, Eugen
影响因子：
5.6
作者：
Hesdorffer, Dale C.;Shinnar, Shlomo;Sun, Shumei
通讯作者：
Sun, Shumei
影响因子：
5.6
作者：
Kossoff, EH;Thiele, EA;Freeman, JM
通讯作者：
Freeman, JM