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Auras localized to the temporal lobe disrupt verbal memory and learning — Causal evidence from direct electrical stimulation of the hippocampus 位于颞叶的光环破坏了言语记忆和学习——来自海马体直接电刺激的因果证据
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.07.006
Diana Pizarro , Emilia Toth , Auriana Irannejad , Kristen O. Riley , Zeenat Jaisani , Wolfgang Muhlhofer , Roy Martin , Sandipan Pati

Auras (focal aware seizure; FAS) are subjective ictal events with retained consciousness. Epileptiform activities can disrupt cognitive tasks, but studies are limited to seizures with impaired awareness. As a proof of concept, we examined the cognitive effects of direct electrical stimulation to the left hippocampus which induced a habitual FAS in a patient with left mesial temporal lobe epilepsy. During the induced habitual FAS, verbal memory performance declined significantly as compared to pre-stimulation testing. Tasks measuring auditory working memory and psychomotor processing speed were not affected by the stimulation. The study confirms that FAS can impair episodic verbal memory and learning.

先兆(局灶意识发作;FAS是具有保留意识的主观临界事件。癫痫样活动可以扰乱认知任务,但研究仅限于意识受损的癫痫发作。为了证明这一概念,我们研究了直接电刺激左海马诱导左内侧颞叶癫痫患者习惯性FAS的认知效果。在诱导的习惯性FAS期间,与刺激前测试相比,言语记忆表现显著下降。测试听觉工作记忆和精神运动加工速度的任务不受刺激的影响。研究证实,FAS会损害情景性言语记忆和学习能力。
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引用次数: 4
Epilepsy in patients with long QT syndrome type 1: A Norwegian family 1型长QT综合征患者的癫痫:一个挪威家庭
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.09.006
Alba González , Dag Aurlien , Kristina H. Haugaa , Erik Taubøll

The congenital long QT syndrome (cLQTS) is an inherited cardiac disorder and is associated with sudden cardiac death. We describe a Norwegian family with mutations within the KCNQ1 gene causing cLQTS type 1 (LQT1) and epilepsy. The index patient had Jervell and Lange-Nielsen-syndrome (JLNS) with deafness and recurrent episodes of cardiac arrhythmia. The mother and the brother have Romano-Ward syndrome (RWS) with recurrent arrhythmias. Whereas the father has focal epilepsy and genetically verified LQT1, the sister has both focal epilepsy and RWS.

Our findings are consistent with the notion that mutations in the KCNQ1 gene can cause epilepsy.

先天性长QT综合征(cLQTS)是一种遗传性心脏疾病,与心源性猝死有关。我们描述了一个挪威家族,KCNQ1基因突变导致cLQTS 1型(LQT1)和癫痫。指标患者为Jervell and Lange-Nielsen-syndrome (JLNS)伴耳聋和心律失常反复发作。母亲和弟弟患有罗曼诺-沃德综合征(RWS),并伴有反复发作的心律失常。父亲患有局灶性癫痫和遗传上证实的LQT1,而妹妹同时患有局灶性癫痫和RWS。我们的发现与KCNQ1基因突变可导致癫痫的观点一致。
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引用次数: 12
Successful use of pure cannabidiol for the treatment of super-refractory status epilepticus 成功使用纯大麻二酚治疗超难治性癫痫持续状态
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.07.004
Rajsekar R. Rajaraman , Raman Sankar , Shaun A. Hussain

We present the case of a child with long-standing, super-refractory status epilepticus (SRSE) who manifested prompt and complete resolution of SRSE upon exposure to pure cannabidiol. SRSE emerged in the context of remote suspected encephalitis with previously well-controlled epilepsy. We discuss the extent to which response may be specifically attributed to cannabidiol, with consideration and discussion of multiple potential drug–drug interactions. Based on this case, we propose that adjunctive cannabidiol be considered in the treatment of SRSE.

我们提出的情况下,儿童长期,超难治性癫痫持续状态(SRSE)谁表现出迅速和完全解决SRSE暴露于纯大麻二酚。SRSE出现在以前控制良好的癫痫的远程疑似脑炎的背景下。我们讨论了反应可能具体归因于大麻二酚的程度,考虑和讨论了多种潜在的药物-药物相互作用。基于这种情况,我们建议在治疗SRSE时考虑辅助大麻二酚。
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引用次数: 11
Visual hallucinations: A novel complication after hemispherectomy 视幻觉:半脑切除术后的新并发症
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2017.09.005
Jonas Vanags , Monisha Sachdev , Gerald Grant , Mohamad A. Mikati

Two patients at our center experienced florid visual hallucinations following hemispherectomy. The first patient had drug-resistant left hemispheric focal seizures at 20 months of age from a previous stroke. Following functional hemispherectomy at age 3, he experienced frightening hallucinations 1 month post-operatively lasting 3.5 months. Our second patient underwent subtotal hemispherectomy at age 6 for drug-resistant focal seizures from right hemispheric cortical dysplasia. Eighteen months later he developed scary visual hallucinations during which he would shout and throw things. Hallucinations recurred for 6 months. In our experience in these patients, even though symptoms were florid, they were transient and subsided 3–6 months later.

我们中心的两名患者在半脑切除术后出现了绚丽的视觉幻觉。第一个患者在20个月大时因先前的中风而出现耐药性左半球局灶性癫痫发作。3岁时进行功能性半球切除术,术后1个月出现可怕的幻觉,持续3.5个月。我们的第二位患者在6岁时因右半球皮质发育不良引起的耐药局灶性癫痫接受了半球次全切除术。18个月后,他出现了可怕的视觉幻觉,他会大喊大叫,扔东西。幻觉复发6个月。根据我们对这些患者的经验,尽管症状很丰富,但它们是短暂的,并在3-6个月后消退。
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引用次数: 2
Sign and magnitude scaling properties of heart rate fluctuations following vagus nerve stimulation in a patient with drug-resistant epilepsy 耐药癫痫患者迷走神经刺激后心率波动的体征和幅度标度特征
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.05.003
Eduardo Gutiérrez-Maldonado, Claudia Ivette Ledesma-Ramírez, Adriana Cristina Pliego-Carrillo, José Javier Reyes-Lagos

Vagus nerve stimulation (VNS) therapy has been recently incorporated in Latin America as a treatment for drug-resistant epilepsy. In particular, it is known that linear analysis and fractal parameters of heart rate variability (HRV) are able to indirectly measure cardiac autonomic activity. This case report presents a 17-year-old female with drug-resistant epilepsy implanted with a VNS device. In order to explore cardiac autonomic changes due to VNS, linear and fractal HRV indices were calculated in the presence and absence of neurostimulation. Novel fractal scaling exponents from HRV analysis were obtained from this patient and from a healthy control subject. Our results indicate that fractal indices of HRV, such as short-term scaling parameters from magnitude and sign analyses seem to be sensitive to the presence or absence of VNS, being confirmed by linear classical methods. This study shows that VNS therapy increases the complexity of cardiac fluctuations in a patient with drug-resistant epilepsy, reflecting an augmented HRV non-linearity and a diminished anticorrelated pattern in heart rate fluctuations. A potential clinical use of these parameters includes the early identification of bradycardia, sudden unexpected death (SUDEP) risk and preoperative VNS approaches. Thus, the scaling and magnitude properties of HRV have potential importance as a non-invasive and easy method for adequate diagnostic/prognostic implications in epilepsy treatment.

迷走神经刺激(VNS)疗法最近在拉丁美洲被纳入耐药癫痫的治疗。特别是,已知心率变异性(HRV)的线性分析和分形参数能够间接测量心脏自主活动。本病例报告介绍了一名17岁患有耐药癫痫的女性,植入了VNS装置。为了探讨VNS引起的心脏自主神经变化,我们计算了有和没有神经刺激时的线性和分形HRV指数。从该患者和健康对照者的HRV分析中获得了新的分形标度指数。结果表明,HRV的分形指标,如从数量级和符号分析中得到的短期尺度参数,似乎对VNS的存在或不存在敏感,并得到了线性经典方法的证实。本研究表明,VNS治疗增加了耐药癫痫患者心脏波动的复杂性,反映了HRV非线性的增强和心率波动的反相关模式的减弱。这些参数的潜在临床应用包括早期识别心动过缓、意外猝死(SUDEP)风险和术前VNS入路。因此,HRV的标度和大小特性具有潜在的重要性,作为一种非侵入性和简单的方法,在癫痫治疗中具有充分的诊断/预后意义。
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引用次数: 2
Teratoma-negative anti-NMDA receptor encephalitis presenting with a single generalized tonic–clonic seizure 畸胎瘤阴性抗nmda受体脑炎表现为单一全身性强直-阵挛性发作
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.02.001
Andy Cheuk-Him Ng , Miljan Tripic , Seyed M. Mirsattari

Herein, we describe a case report of anti-NMDA receptor encephalitis characterized by a single generalized tonic–clonic seizure and predominantly psychiatric symptoms, persisting long after EEG abnormalities had resolved. We discuss common presentations of anti-NMDA receptor encephalitis and advocate for the inclusion of this disease entity in the differential diagnosis of patients presenting with one generalized tonic–clonic seizure and prominent psychiatric symptoms.

在此,我们描述了一个病例报告的抗nmda受体脑炎的特点是单一的全面性强直-阵挛性发作和主要的精神症状,持续很长时间后脑电图异常已经解决。我们讨论抗nmda受体脑炎的常见表现,并提倡将这种疾病纳入以全身性强直阵挛发作和突出精神症状为表现的患者的鉴别诊断。
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引用次数: 1
Neurosurgical treatment of nonconvulsive status epilepticus due to focal cortical dysplasia 局灶性皮质发育不良所致非惊厥性癫痫持续状态的神经外科治疗
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.03.003
Emin Timer , Saeid Charsouei , Nerses Bebek , Betül Baykan , Bilge Bilgiç , Pulat Akın Sabancı , Yavuz Aras , Nail İzgi , Candan Gürses

We present a rare case of focal cortical dysplasia (FCD) and nonconvulsive status epilepticus (NCSE) treated successfully with early surgical intervention. Our case is a 9-year-old boy whose seizures, characterized by short episodes of loss of consciousness, appeared at the age of 7, and he showed progressive cognitive decline in the following years. NCSE was diagnosed, and his MRI revealed FCD in the left frontal region which was the same side as his EEG abnormality. Following lesionectomy, his NCSE disappeared and cognitive functions improved. Histopathologic analysis of the resected tissue revealed type-IIB FCD. This case illustrates the importance of early surgery to help restore cognitive functions by eliminating the clinical and electrophysiological features of NCSE.

我们报告一例罕见的局灶性皮质发育不良(FCD)和非惊厥性癫痫持续状态(NCSE)通过早期手术干预成功治疗。我们的病例是一个9岁的男孩,他的癫痫发作,以短暂的意识丧失为特征,在7岁时出现,他在接下来的几年里表现出进行性认知衰退。诊断为NCSE, MRI示左额区FCD,与脑电图异常同侧。病灶切除后,患者NCSE消失,认知功能改善。切除组织的组织病理学分析显示为iib型FCD。这个病例说明了早期手术通过消除NCSE的临床和电生理特征来帮助恢复认知功能的重要性。
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引用次数: 2
Recurrent seizures from chronic kratom use, an atypical herbal opioid 慢性使用克拉托姆,一种非典型的草药类阿片
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.04.002
William O. Tatum , Tasneem F. Hasan , Erin E. Coonan , Christopher P. Smelick

Kratom is an herbal compound that has been used as a recreational drug though is not regulated by the Food and Drug Administration. We report a 19-year-old male with recurrent seizures that developed during daily Kratom abuse as a self-treatment for anxiety. Following recurrent focal impaired awareness seizures in addition to generalized tonic–clonic seizures, he was begun on anti-seizure drugs. Seizures subsided after completing rehabilitation. Brain MRI at 29 months revealed bilaterally symmetric T1-hyperintensity in globus pallidus, subthalamic nuclei, and cerebral peduncles. Our case suggests Kratom abuse may be associated with structural brain lesions on MRI and symptomatic focal epilepsy.

克拉通是一种草药化合物,虽然不受美国食品和药物管理局的监管,但它已被用作娱乐性药物。我们报告一个19岁的男性复发性癫痫发作,在日常滥用Kratom作为焦虑的自我治疗。继反复出现局灶性意识受损癫痫发作以及全身性强直阵挛性癫痫发作后,他开始服用抗癫痫药物。康复完成后癫痫发作消退。29 个月时的脑部MRI显示,苍白球、丘脑下核和脑梗两侧对称t1高信号。我们的病例表明,Kratom滥用可能与MRI上的结构性脑损伤和症状性局灶性癫痫有关。
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引用次数: 32
Drug resistant epilepsy with mesial temporal sclerosis as possible late neurological complication in two AML survivors after stem cell transplantation 两例干细胞移植后AML幸存者的耐药癫痫伴内侧颞叶硬化可能的晚期神经系统并发症
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.05.001
Kwan-Ming Karen Yam , Wing-Kwan Alex Leung , Xian-Lun Zhu , Lai-Wah Eva Fung
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引用次数: 3
Long-term use of methylphenidate in a boy with hypothalamic tumor, drug-resistant epilepsy and ADHD 长期使用哌甲酯治疗患有下丘脑肿瘤、耐药癫痫和多动症的男孩
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.03.002
Dobrinko Socanski , Nebojsa Jovic , Harald Beneventi , Anita Herigstad
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引用次数: 3
期刊
Epilepsy and Behavior Case Reports
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