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Continuous ictal discharges with high frequency oscillations confined to the non-sclerotic hippocampus in an epileptic patient with radiation-induced cavernoma in the lateral temporal lobe 伴有辐射诱发的颞叶侧海绵状瘤的癫痫患者非硬化海马的持续高频振荡发作放电
Pub Date : 2019-01-01 DOI: 10.1016/j.ebcr.2019.01.003
Nobutaka Mukae , Takato Morioka , Michiko Torio , Ayumi Sakata , Satoshi O. Suzuki , Koji Iihara

Intraoperative electrocorticography recording is recommended for treating cavernoma-related epilepsy. However, interictal paroxysmal epileptiform activity is generally able to be recorded, but is not always identical to the epileptogenic zone.

We surgically treated a 15-year-old girl with drug-resistant epilepsy associated with radiation-induced cavernoma in the right lateral temporal lobe. Electrocorticography revealed paroxysmal activities in the cortex around the cavernoma. Additionally, continuous subclinical “ictal” discharges with high-frequency oscillations confined to the histologically non-sclerotic hippocampus were recorded. Following additional hippocampectomy, a good seizure outcome was obtained.

Intraoperative electrocorticography and high-frequency oscillation analysis revealed high epileptogenicity in the non-sclerotic hippocampus of this patient.

术中皮质电图记录被推荐用于治疗海绵瘤相关癫痫。然而,间隔期发作性癫痫样活动通常能够被记录下来,但并不总是与癫痫区相同。我们手术治疗了一名15岁的女孩,她患有与右侧外侧颞叶放射性海绵状瘤相关的耐药癫痫。皮质电图显示海绵状瘤周围皮层有阵发性活动。此外,记录了持续的亚临床“临界”放电,高频振荡局限于组织学上未硬化的海马。在额外的海马切除术后,获得了良好的癫痫发作结果。术中皮质电图和高频振荡分析显示该患者的非硬化海马具有高致痫性。
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引用次数: 3
Long term seizure freedom on perampanel in highly drug-resistant epilepsy caused by bilateral periventricular nodular heterotopia: A case report perampanel治疗双侧脑室周围结节性异位致高度耐药癫痫1例
Pub Date : 2019-01-01 DOI: 10.1016/j.ebcr.2019.01.009
Makoto Kiyose , Marlies Wagner , Annika Männer , Sarah C. Reitz , Karl Martin Klein , Adam Strzelczyk , Sebastian Bauer , Felix Rosenow
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引用次数: 1
Are there mortality risks for patients with epilepsy who use cannabis treatments as monotherapy? 使用大麻作为单一疗法的癫痫患者是否存在死亡风险?
Pub Date : 2019-01-01 DOI: 10.1016/j.ebcr.2018.11.007
Devon M. Kollmyer , Kyla E. Wright , Nicole M. Warner , Michael J. Doherty

Mortality associated with cannabis used for treatment of epilepsy is not well documented. We discuss two fatalities in the setting of epilepsy and self-determined therapy with cannabis (SDTC). One patient had probable sudden unexpected death in epilepsy, the second death was due to seizure-associated drowning. Both directed SDTC over conventional anti-seizure medications. Where recreational cannabis is legal, decisions to use cannabis are often self-directed and independent of physician advice of cannabis risks, in part because physicians may not be aware of the risk of SDTC. Further study of morbidity and mortality of SDTC in patients with epilepsy is needed.

与用于治疗癫痫的大麻有关的死亡率没有很好的记录。我们讨论癫痫和大麻(SDTC)自我决定的治疗设置两个死亡。1例患者可能因癫痫猝死,2例患者因癫痫相关溺水死亡。两家公司都将SDTC置于常规抗癫痫药物之上。在娱乐性大麻合法的地方,使用大麻的决定往往是自主的,独立于医生关于大麻风险的建议,部分原因是医生可能不知道SDTC的风险。需要进一步研究SDTC在癫痫患者中的发病率和死亡率。
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引用次数: 8
Forced normalization after turning off vagus nerve stimulation in Lennox–Gastaut syndrome lenox - gastaut综合征中关闭迷走神经刺激后的强迫正常化
Pub Date : 2019-01-01 DOI: 10.1016/j.ebcr.2019.01.004
Sydney Lee , Alyssa Denton , Lady Diana Ladino , Karen Waterhouse , Aleksander Vitali , Jose Francisco Tellez-Zenteno

Forced normalization is the development of psychiatric symptoms in a patient experiencing remission of seizures. We present a case of Lennox Gastaut syndrome in which forced normalization developed after vagus nerve stimulation was stopped.

The patient had drug resistant epilepsy and failed anti-seizure drugs, vagus nerve stimulation, and a partial callosotomy.

The patient had multiple types of seizures including drop attacks, absences, and tonic–clonic seizures. He tried vagus nerve stimulation for two years without success. Forced normalization developed after the vagus nerve stimulator was turned off.

This is the first case to our knowledge to describe forced normalization after turning off the vagus nerve stimulator.

强迫正常化是在经历癫痫发作缓解的患者中精神症状的发展。我们提出了一例Lennox Gastaut综合征,在迷走神经刺激停止后,强迫正常化发展。患者有耐药癫痫和失败的抗癫痫药物,迷走神经刺激和部分胼胝体切开术。患者有多种类型的癫痫发作,包括跌落发作、缺席和强直阵挛发作。他尝试了两年的迷走神经刺激,但没有成功。迷走神经刺激器关闭后,出现了强迫正常化。这是据我们所知的第一个在关闭迷走神经刺激器后描述强迫正常化的病例。
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引用次数: 6
Epileptic spasms as the presenting seizure type in a patient with a new “O” of TORCH, congenital Zika virus infection 先天性寨卡病毒感染TORCH新“O”型患者表现为癫痫性痉挛
Pub Date : 2019-01-01 DOI: 10.1016/j.ebcr.2018.09.002
Jason Lockrow, Hannah Tully, Russell P. Saneto

Congenital TORCH infections are a significant cause of epileptic spasms, an infantile epileptic encephalopathy, through disruptions to several pathways in neurodevelopment. Congenital Zika virus has a similar neurotropism to other TORCH agents, and leads to microcephaly, severe neurodevelopmental impairment, and high rates of early onset seizures. Here we report a child with confirmed congenital Zika virus who developed extensor epileptic spasms and hypsarrhythmia associated with a loss of early developmental milestones. Early treatment led to resolution of epileptic spasms and improved developmental trajectory, though the child continues to have ongoing focal seizures and prominent developmental impairment. Congenital Zika virus infection requires close monitoring as early identification of epileptic spasms is likely important in long term developmental outcome.

先天性TORCH感染是癫痫性痉挛(一种婴儿癫痫性脑病)的重要原因,通过破坏神经发育的几种途径。先天性寨卡病毒与其他TORCH药物具有类似的神经嗜性,可导致小头畸形、严重的神经发育障碍和高发生率的早发性癫痫发作。在这里,我们报告了一名确诊患有先天性寨卡病毒的儿童,他出现了伸展性癫痫痉挛和心律失常,并伴有早期发育里程碑的缺失。早期治疗导致癫痫痉挛的解决和改善的发展轨迹,但儿童仍然有持续的局灶性癫痫发作和突出的发育障碍。先天性寨卡病毒感染需要密切监测,因为癫痫痉挛的早期识别可能对长期发育结果很重要。
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引用次数: 9
Surgically treated intracranial supratentorial calcifying pseudoneoplasms of the neuraxis (CAPNON) with drug-resistant left temporal lobe epilepsy: A case report and review of the literature 手术治疗耐药左颞叶癫痫伴颅内幕上钙化性神经轴假性肿瘤1例报告及文献复习
Pub Date : 2019-01-01 DOI: 10.1016/j.ebcr.2019.02.002
Yuta Tanoue, Takehiro Uda, Kosuke Nakajo, Shugo Nishijima, Tsuyoshi Sasaki, Kenji Ohata

Calcifying pseudoneoplasms of the neuraxis (CAPNON) are rare pathological lesions that can present anywhere in the central nervous system. Symptoms vary depending on the location, though they often include seizures, especially in intracranial and supratentorial lesions. A case of intracranial supratentorial CAPNON presenting with drug-resistant left temporal lobe epilepsy is reported. The patient had a history of drug-resistant focal seizures for over 36 years. The lesion was located in the left mesial temporal lobe, but hippocampal sclerosis and hippocampal invasion were not apparent. The lesion was removed without hippocampectomy, and the patient has been seizure-free for one year.

神经轴钙化假性肿瘤(CAPNON)是一种罕见的病理病变,可以出现在中枢神经系统的任何地方。症状因部位不同而不同,但通常包括癫痫发作,尤其是颅内和幕上病变。报告1例颅内幕上CAPNON表现为耐药左颞叶癫痫。患者有超过36 年的耐药局灶性癫痫发作史。病变位于左侧颞叶内侧,但海马硬化和海马浸润不明显。病变未切除海马,患者已一年无癫痫发作。
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引用次数: 10
Epilepsy phenotype in patients with Xp22.31 microduplication Xp22.31微重复患者的癫痫表型
Pub Date : 2019-01-01 DOI: 10.1016/j.ebcr.2018.10.004
Mario Brinciotti , Francesca Fioriello , Antonio Mittica , Laura Bernardini , Marina Goldoni , Maria Matricardi

The clinical significance of Xp22.31 microduplication is still unclear. We describe a family in which a mother and two children have Xp22.31 microduplication associated with different forms of epilepsy and epileptiform EEG abnormalities. The proband had benign epilepsy with centrotemporal spikes with dysgraphia and dyscalculia (IQ 72), the sister had juvenile myoclonic epilepsy, and both had bilateral talipes anomalies. The mother, who was the carrier of the microduplication, was asymptomatic. The asymptomatic father did not possess the microduplication. These data contribute to delineate the phenotype associated with Xp22.31 microduplication and suggest a potential pathogenic role for an epilepsy phenotype.

Xp22.31微重复的临床意义尚不清楚。我们描述了一个家庭,其中母亲和两个孩子有Xp22.31微重复与不同形式的癫痫和癫痫样脑电图异常相关。先证者为良性癫痫,伴有书写障碍和计算障碍(IQ 72),妹妹为青少年肌阵挛性癫痫,两人均有双侧taltales异常。母亲是微复制的携带者,没有症状。无症状的父亲没有微型复制。这些数据有助于描述与Xp22.31微重复相关的表型,并提示癫痫表型的潜在致病作用。
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引用次数: 3
A case of ictal burst-suppression 致命突发抑制一例
Pub Date : 2019-01-01 DOI: 10.1016/j.ebcr.2018.11.005
Pegah Afra , Verena Clarissa Samara , Lilly Fagatele , Bola Adamolekun

“Burst-suppression” pattern consists of complete attenuation of background between bursts of mixed frequencies, variable morphology and waveforms. It is a subgroup of periodic patterns seen in severe cerebral damage, anesthesia or prematurity. Here, we present a 46-year-old woman with post-anoxic encephalopathy on cooling protocol with two electrographically similar patterns of burst-suppression (one with a clinical ictal correlate of isolated eye movements), as well as three electroclinical seizures. The literature on rare clinical phenomenon of isolated eye movements associated with burst-suppression is reviewed, with the conclusion that the presented case suggests an ictal origin.

“突发抑制”模式由混合频率、可变形态和波形的突发之间的背景完全衰减组成。它是在严重脑损伤、麻醉或早产中出现的周期性模式的一个亚组。在这里,我们报告了一名46岁的女性缺氧后脑病患者,在冷却方案中出现了两种电图相似的爆发抑制模式(一种具有孤立眼动的临床相关),以及三次电临床癫痫发作。文献罕见的临床现象,孤立的眼动与爆发抑制相关的回顾,与结论,提出的情况下,一个关键的起源。
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引用次数: 3
Preserved evoked conscious perception of phosphenes with direct stimulation of deafferented primary visual cortex 通过直接刺激失传入的初级视觉皮层,保存诱发了有意识的光幻视知觉
Pub Date : 2019-01-01 DOI: 10.1016/j.ebcr.2018.12.002
Kelly L. Collins , Devapratim Sarma , Shahin Hakimian , Jeff J. Tsai , Jeffrey G. Ojemann

The premise of neuro-rehabilitation after injury is to access the residual capacity of the nervous system to improve function. We describe a patient who developed a quadrantopsia and drug-resistant focal epilepsy after an arteriovenous malformation hemorrhage. Thirty years later, he underwent placement of subdural electrodes for seizure mapping. Phosphenes were elicited in the blind right visual field with stimulation of occipital cortex. This case demonstrates that visual cortex may retain functional organization after a partial subcortical visual pathway injury. This persistent conscious mapping suggests that disconnected visual cortex could serve as a region for interfacing with neural prosthetic devices for acquired blindness.

损伤后神经康复的前提是利用神经系统的剩余容量来改善功能。我们描述了一个病人谁发展的四角视和抗药性局灶性癫痫后,动静脉畸形出血。30年后,他接受了硬脑膜下电极定位癫痫发作。通过刺激枕叶皮层,在失明的右侧视野中激发光幻视。本病例表明,部分皮层下视觉通路损伤后,视觉皮层可保留功能组织。这种持续的有意识的映射表明,断开的视觉皮层可以作为一个区域,与获得性失明的神经假体装置相连接。
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引用次数: 1
Autosomal dominant temporal lobe epilepsy associated with heterozygous reelin mutation: 3 T brain MRI study with advanced neuroimaging methods 常染色体显性颞叶癫痫与杂合型reelin突变相关:3 采用先进神经成像方法的T脑MRI研究
Pub Date : 2019-01-01 DOI: 10.1016/j.ebcr.2018.10.003
Katarína Česká , Štefánia Aulická , Ondřej Horák , Pavlína Danhofer , Pavel Říha , Radek Mareček , Jan Šenkyřík , Ivan Rektor , Milan Brázdil , Hana Ošlejšková

Purpose

Autosomal dominant lateral temporal epilepsy (ADLTE) is a genetic focal epilepsy syndrome characterized by focal seizures with dominant auditory symptomatology. We present a case report of an 18-year-old patient with acute onset of seizures associated with epilepsy. Based on the clinical course of the disease and the results of the investigation, the diagnosis of ADLTE with a proven mutation in the RELN gene, which is considered causative, was subsequently confirmed. The aim of this study was to use 3 Tesla (3 T) magnetic resonance imaging (MRI) and advanced neuroimaging methods in a patient with a confirmed diagnosis of ADTLE.

Methods

3 T MRI brain scan and advanced neuroimaging methods were used in the standard protocols to analyzse voxel-based MRI, cortical thickness, and functional connectivity.

Results

Morphometric MRI analysis (blurred grey-white matter junctions, voxel-based morphometry, and cortical thickness analysis) did not provide any informative results. The functional connectivity analysis revealed higher local synchrony in the patient in the left temporal (middle temporal gyrus), left frontal (supplementary motor area, superior frontal gyrus), and left parietal (gyrus angularis, gyrus supramarginalis) regions and the cingulate (middle cingulate gyrus) as compared to healthy controls.

Conclusions

Evidence of multiple areas of functional connectivity supports the theory of epileptogenic networks in ADTLE. Further studies are needed to elucidate this theory.

目的常染色体显性颞外侧癫痫(ADLTE)是一种遗传性局灶性癫痫综合征,其特征是局灶性发作伴显性听觉症状。我们提出一个病例报告的18岁患者急性发作癫痫相关的癫痫。根据该疾病的临床病程和调查结果,随后确认了ADLTE的诊断,并证实了RELN基因突变,这被认为是病因。本研究的目的是使用3特斯拉(3 T)磁共振成像(MRI)和先进的神经影像学方法对确诊为ADTLE的患者进行检查。方法3 在标准方案中使用T MRI脑扫描和先进的神经影像学方法分析基于体素的MRI、皮质厚度和功能连通性。结果MRI形态学分析(模糊的灰质连接、基于体素的形态测量和皮质厚度分析)没有提供任何有用的结果。功能连通性分析显示,与健康对照组相比,患者左侧颞叶(颞中回)、左侧额叶(辅助运动区、额上回)、左侧顶叶(角回、边缘上回)和扣带(中扣带回)的局部同步性更高。结论多区域功能连接的证据支持ADTLE的致痫网络理论。需要进一步的研究来阐明这一理论。
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引用次数: 5
期刊
Epilepsy and Behavior Case Reports
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