Neurocritical care and target immunotherapy for febrile infection-related epilepsy syndrome

被引:18
作者
Sakuma, Hiroshi [1 ]
Horino, Asako [1 ,2 ]
Kuki, Ichiro [2 ]
机构
[1] Tokyo Metropolitan Inst Med Sci, Dept Brain Dev & Neural Regenerat, Tokyo, Japan
[2] Osaka City Gen Hosp, Dept Pediat Neurol, Childrens Med Ctr, Osaka, Japan
关键词
Febrile infection-related epilepsy syndrome (FIRES); New-onset refractory status epilepticus (NORSE); Burst-suppression coma; Ketogenic diet; Anti-cytokine therapy; REPETITIVE PARTIAL SEIZURES; SYNDROME FIRES; ACUTE ENCEPHALITIS; ACUTE ENCEPHALOPATHY; TERM;
D O I
10.1016/j.bj.2020.03.009
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
Febrile infection-related epilepsy syndrome (FIRES) is an intractable neurological disease characterized by an unexplained refractory status epilepticus triggered by febrile infection. A Consensus definition of FIRES was proposed in 2018, and its clinical features and prognosis are gradually being clarified. However, the development of effective treatments has been hindered as the etiology of this rare disease is as yet unelucidated. The basic approach to the management of FIRES, like other forms of epilepsy, is based on the control of seizures, however seizures are extremely intractable and require intravenous administration of large doses of anticonvulsants, mainly barbiturates. This treatment strategy produces various complications including respiratory depression and drug hypersensitivity syndrome, which make it more difficult to control seizures. Consequently, it is crucial to predict these events and to formulate a planned treatment strategy. As well, it is important to grow out of conventional treatment strategies that rely on only anticonvulsants, and alternative therapies are gradually being developed. One such example is the adoption of a ketogenic diet which may lead to reduced convulsions as well as improve intellectual prognosis. Further, overproduction of inflammatory cytokines in the central nervous system has been shown to be strongly related to the pathology of FIRES which has led to attempts at immunomodulation therapy including anti-cytokine therapy.
引用
收藏
页码:205 / 210
页数:6
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