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持续性部分性癫痫:综述

Epilepsia partialis continua: A review.

作者信息

Mameniškienė Rūta, Wolf Peter

机构信息

Vilnius University, Department of Neurology and Neurosurgery, Center for Neurology, Vilnius, Lithuania.

Danish Epilepsy Centre, Dianalund, Denmark; Serviço de Neurologia, Departamento de Clínica Médica, Hospital Universitário, Universidade Federal de Santa Catarina (UFSC), Florianópolis, SC, Brazil.

出版信息

Seizure. 2017 Jan;44:74-80. doi: 10.1016/j.seizure.2016.10.010. Epub 2016 Oct 18.

DOI:10.1016/j.seizure.2016.10.010
PMID:28029552
Abstract

Epilepsia partialis contina (EPC) in a narrow definition is a variant of simple focal motor status epilepticus in which frequent repetitive muscle jerks, usually arrhythmic, continue over prolonged periods of time. In a broader definition (used in this review) it also includes non-motor manifestations otherwise known as aura continua. EPC may occur as a single episode, repetitive episodes, it may be chronic progressive or non-progressive. It appears as an unusual manifestation of epilepsy in which more typical paroxysmal events are partly or entirely replaced by the sustained repetition of seizure fragments in rapid succession. The minimum duration is defined as one hour but EPC may continue for up to many years. There are multiple possible etiologies which can be local or systemic, including two disease entities, Rasmussen encephalitis and Russian tick-borne spring-summer encephalitis. Among systemic brain disorders, mitochondrial diseases and non-ketotic hyperglycemia are particularly likely to cause EPC whereas stroke is a frequent cause of acute EPC. The symptoms of motor EPC have been interpreted as cortical reflex myocloni but the pathophysiology is probably not uniform for all cases. In pathophysiological terms, EPC seems to represent an oscillation of excitation and inhibition in a feedback loop whose mechanisms are still poorly understood. However, EPC only seems to occur rarely in an otherwise healthy brain. Treatment has to take account of the etiology but, in general, EPC tends to be drug-resistant. Epilepsy surgery is often indicated in Rasmussen encephalitis.

摘要

狭义的持续性部分性癫痫(EPC)是单纯局灶性运动性癫痫持续状态的一种变体,其中频繁的重复性肌肉抽搐(通常无节律)会持续很长时间。在更宽泛的定义中(本综述采用此定义),它还包括其他被称为持续性先兆的非运动性表现。EPC可能以单次发作、重复性发作的形式出现,可能是慢性进行性或非进行性的。它表现为癫痫的一种不寻常表现形式,其中更典型的阵发性事件部分或完全被快速连续的癫痫发作片段的持续重复所取代。最短持续时间定义为1小时,但EPC可能持续长达数年。有多种可能的病因,可分为局部性或全身性,包括两种疾病实体,即拉斯穆森脑炎和俄罗斯蜱传春夏脑炎。在全身性脑部疾病中,线粒体疾病和非酮症高血糖特别容易导致EPC,而中风是急性EPC的常见病因。运动性EPC的症状被解释为皮质反射性肌阵挛,但所有病例的病理生理学可能并不一致。从病理生理学角度来看,EPC似乎代表了一个反馈回路中兴奋与抑制的振荡,其机制仍知之甚少。然而,EPC似乎很少发生在其他方面健康的大脑中。治疗必须考虑病因,但一般来说,EPC往往耐药。癫痫手术常用于拉斯穆森脑炎。

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