Febrile Infection-Related Epilepsy Syndrome (FIRES): An Overview of Treatment and Recent Patents.

IF 4.2 Q3 Pharmacology, Toxicology and Pharmaceutics Recent patents on inflammation & allergy drug discovery Pub Date : 2018-01-01 DOI:10.2174/1872213X12666180508122450
Kam L Hon, Alexander K C Leung, Alcy R Torres
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引用次数: 33

Abstract

Background: New-Onset Refractory Status Epilepticus (NORSE) refers to a clinical presentation in a patient without active epilepsy or other existing relevant neurological disorder, with new onset of refractory status epilepticus in the absence of a clear acute or active structural, metabolic, or toxic cause. Febrile Infection-Related Epilepsy Syndrome (FIRES) is a subset of NORSE that requires a febrile infection between 24 hours and 2 weeks prior to the onset of refractory status epilepticus, with or without fever at the onset of status epilepticus, and with no restriction to the age of the patient. The literature on FIRES is scarce.

Objective: This article reviews the pathophysiology, clinical features, and various treatment modalities in the treatment of FIRES.

Methods: A Medline/Pubmed search was conducted using Clinical Queries with the key terms "Febrile Infection-Related Epilepsy Syndrome", "FIRES", "New-Onset Refractory Status Epilepticus" and "NORSE". The search strategy included meta-analyses, randomized controlled trials, clinical trials, reviews and pertinent references. Patents were searched using the key term "FIRES", "NORSE" and "Febrile Epilepsy Syndrome" from www.google.com/patents, www.uspto.gov, and www.freepatentsonline.com.

Results: FIRES almost invariably begins with a mild nonspecific febrile illness in an otherwise healthy individual. Twenty-four hours to two weeks later, seizures begin and quickly become very frequent and worsen, becoming status epilepticus. Seizures can be simple motor, complex partial or secondary generalized. The exact etiology is not known. It is possible that the syndrome is caused by an inflammatory or autoimmune mechanism. Seizures in FIRES are notoriously very difficult to treat. Treatment modalities include, among others, various antiepileptic drugs, ketogenic diet, intravenous corticosteroids, intravenous immunoglobulin, and burst-suppression coma. The outcome is poor; most children are left with significant cognitive disability and refractory epilepsy. Recent patents for the management of FIRES are discussed.

Conclusion: FIRES is a rare epilepsy syndrome of unclear etiology in which children, usually of school age, suddenly develop very frequent seizures after a mild febrile illness. Seizures in FIRES are typically difficult to treat. The prognosis is poor.

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发热性感染相关癫痫综合征(FIRES):治疗综述及近期专利
背景:新发难治性癫痫持续状态(NORSE)是指没有活动性癫痫或其他相关神经系统疾病的患者,在没有明确的急性或活动性结构、代谢或毒性原因的情况下,出现新发难治性癫痫持续状态的临床表现。发热性感染相关癫痫综合征(FIRES)是NORSE的一个子集,要求在难耐癫痫持续状态发作前24小时至2周发生发热性感染,癫痫持续状态发作时伴有或不伴有发热,且患者年龄不受限制。关于火灾的文献很少。目的:综述火灾的病理生理、临床特点及各种治疗方法。方法:以“发热性感染相关癫痫综合征”、“FIRES”、“新发难固性癫痫持续状态”和“NORSE”为关键词,通过Medline/Pubmed进行检索。检索策略包括荟萃分析、随机对照试验、临床试验、综述和相关参考文献。使用关键词“FIRES”、“NORSE”和“发热性癫痫综合征”从www.google.com/patents、www.uspto.gov和www.freepatentsonline.com.Results检索专利:FIRES几乎总是以健康个体的轻度非特异性发热性疾病开始。24小时至两周后,癫痫发作开始并迅速变得非常频繁和恶化,成为癫痫持续状态。癫痫发作可为简单的运动性,复杂的局部或继发性全身性。确切的病因尚不清楚。该综合征可能是由炎症或自身免疫机制引起的。众所周知,火灾的发作很难治疗。治疗方式包括,除其他外,各种抗癫痫药物,生酮饮食,静脉注射皮质类固醇,静脉注射免疫球蛋白和突发抑制昏迷。结果很糟糕;大多数儿童留下了严重的认知障碍和顽固性癫痫。本文还讨论了用于管理FIRES的最新专利。结论:FIRES是一种病因不明的罕见癫痫综合征,通常为学龄儿童,在轻度发热性疾病后突然频繁发作。火灾的发作通常难以治疗。预后很差。
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期刊介绍: Recent Patents on Inflammation & Allergy Drug Discovery publishes review articles by experts on recent patents in the field of inflammation and allergy drug discovery e.g. on novel bioactive compounds, analogs and targets. A selection of important and recent patents in the field is also included in the journal. The journal is essential reading for all researchers involved in inflammation and allergy drug design and discovery.
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