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Longitudinal brain functional and structural connectivity changes after hemispherotomy in two pediatric patients with drug-resistant epilepsy 2例小儿耐药癫痫患者脑半球切除术后脑纵向功能和结构连通性的改变
Pub Date : 2019-01-01 DOI: 10.1016/j.ebcr.2018.11.003
Yongxin Li , Ya Wang , Zhen Tan , Qian Chen , Wenhua Huang

The main focus of the present study was to explore the longitudinal changes in the brain executive control system and default mode network after hemispherotomy. Resting-state functional magnetic resonance imaging and diffusion tensor imaging were collected in two children with drug-resistnt epilepsy underwent hemispherotomy. Two patients with different curative effects showed different trajectories of brain connectivity after surgery. The failed hemispherotomy might be due to the fact that the synchrony of epileptic neurons in both hemispheres is preserved by residual neural pathways. Loss of interhemispheric correlations with increased intrahemispheric correlations can be considered as neural marker for evaluating the success of hemispherotomy.

本研究的重点是探讨脑半球切除术后大脑执行控制系统和默认模式网络的纵向变化。对2例接受半脑切开术的耐药癫痫患儿进行静息态功能磁共振和弥散张量成像。两例疗效不同的患者术后脑连通性变化轨迹不同。大脑半球切除失败可能是由于残留的神经通路保留了两个半球癫痫神经元的同步性。半球间相关性的丧失与半球内相关性的增加可以被认为是评估半球切除术成功的神经标志物。
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引用次数: 3
Familial association of genetic generalised epilepsy with limb-girdle muscular dystrophy through a mutation in CAPN3 遗传性全身性癫痫与肢体肌萎缩症通过CAPN3突变的家族关联
Pub Date : 2019-01-01 DOI: 10.1016/j.ebcr.2019.03.003
Alejandro Viloria-Alebesque , Elena Bellosta-Diago , Sonia Santos-Lasaosa , José Ángel Mauri-Llerda
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引用次数: 2
Sub-lobar dysplasia — A comprehensive evaluation with neuroimaging, magnetoencephalography and histopathology 脑叶下发育不良-神经影像学、脑磁图和组织病理学综合评价
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2017.11.002
Kenchaiah Raghavendra , Ganne Chaitanya , Bhargava Goutham , Anita Mahadevan , Ravindranadh Chowdary Mundlamuri , Rose Dawn Bharath , Mariyappa Narayannan , Malla Bhaskar Rao , Arimappamagan Arivazhagan , Parthasarthy Satishchandra , Sanjib Sinha

Sublobar dysplasia, a rare cortical malformation has been defined in only 8 patients to date. It was identified on the basis of histopathological features and MRI findings. We report a right temporal sublobar dysplasia, with detailed evaluation including neuroimaging, magnetoencephalography and histopathology to further characterize the pathology. Additional pathological features included a deep collateral sulcus in the basal right temporal lobe, thinned out right corticospinal tract, and bilateral asymmetric basal ganglia changes. Magnetoencephalograpy localized the seizure focus to the posterior margin of the dysplasia. Histopathological evaluation helped exclude other types of dysplasia. Similar to a previous study, the child had Engel 1a outcome.

叶下发育不良是一种罕见的皮质畸形,迄今为止仅在8例患者中被确诊。根据组织病理学特征和MRI检查结果确定。我们报告一个右侧颞叶下发育不良,详细的评估包括神经影像学,脑磁图和组织病理学进一步表征病理。其他病理特征包括右侧颞叶基底侧深沟,右侧皮质脊髓束变薄,双侧基底节区不对称改变。脑磁图将癫痫病灶定位于发育不良的后缘。组织病理学评估有助于排除其他类型的不典型增生。与之前的研究类似,该儿童的Engel结果为1a。
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引用次数: 2
Language recovery after epilepsy surgery of the Broca's area 布洛卡区癫痫手术后的语言恢复
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2017.06.002
Lilit Mnatsakanyan , Sumeet Vadera , Christopher W. Ingalls , Jie Zheng , Mona Sazgar , Frank P. Hsu , Jack J. Lin

Epilepsy surgery is indicated in select patients with drug-resistant focal epilepsy. Seizure freedom or significant reduction of seizure burden without risking new neurological deficits is the expected goal of epilepsy surgery. Typically, when the seizure onset zone overlaps with eloquent cortex, patients are excluded from surgery.

We present a patient with drug-resistant frontal lobe epilepsy who underwent successful surgery with resection of Broca's area, primarily involving the pars triangularis (BA 45). We report transient expressive aphasia followed by recovery of speech. This case provides new insights into adult neuroplasticity of the language network.

癫痫手术适用于部分耐药局灶性癫痫患者。癫痫手术的预期目标是癫痫发作自由或显著减少癫痫发作负担而不冒新的神经功能障碍的风险。通常,当癫痫发作区与雄辩皮层重叠时,患者被排除在手术之外。我们报告了一位患有耐药性额叶癫痫的患者,他成功地切除了布洛卡区,主要涉及三角部(ba45)。我们报告短暂的表达性失语症,随后语言恢复。本案例为成人语言网络的神经可塑性提供了新的见解。
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引用次数: 4
A case of interictal dysphoric disorder comorbid with interictal psychosis: Part of the same spectrum or separate entities? 间断性烦躁不安与间断性精神病共病1例:是同一谱系的一部分还是不同的实体?
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.07.007
Susumu Morita , Go Taniguchi , Hidetaka Tamune , Yousuke Kumakura , Shinsuke Kondo , Kiyoto Kasai

Depressive disorders in epilepsy often present characteristic clinical manifestations atypical in primary, endogenous depression. Here, we report a case of a 64-year-old woman with right mesial temporal lobe epilepsy, who complained of bizarre, antipsychotic-refractory cenesthetic hallucinations in her interictal phase, and was hospitalized after a suicide attempt. Detailed clinical observations revealed mood symptoms, which led to the diagnosis of interictal dysphoric disorder comorbid with interictal psychosis. Sertraline with low-dose aripiprazole markedly alleviated both depressive and psychotic symptoms. This case suggested that the two diagnostic entities may overlap and that depressive symptoms tend to be concurrent when concurring with psychosis, which hampers the appropriate choice of a treatment option.

癫痫抑郁障碍通常表现为原发性内源性抑郁的典型临床表现。在此,我们报告一例64岁女性右内侧颞叶癫痫患者,她主诉在间歇期出现奇异的抗精神病难治性幻觉,并在自杀未遂后住院。详细的临床观察显示情绪症状,导致诊断为间歇期烦躁不安与间歇期精神病共病。舍曲林联合小剂量阿立哌唑可显著缓解抑郁和精神病症状。本病例提示,这两种诊断实体可能重叠,当抑郁症状与精神病同时发生时,往往是同时发生的,这妨碍了治疗方案的适当选择。
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引用次数: 0
Continuous monitoring devices and seizure patterns by glucose, time and lateralized seizure onset 连续监测设备和癫痫发作模式的葡萄糖,时间和侧化癫痫发作
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.03.001
Katie M. Kinnear, Nicole M. Warner, Alan M. Haltiner, Michael J. Doherty

Objectives

To investigate if glucose levels influence seizure patterns.

Materials and methods

In a patient with RNS/NeuroPace implanted bi-temporally and type 1 diabetes mellitus, seizure event times and onset locations were matched to continuous tissue glucose.

Results

Left focal seizure (LFS, n = 22) glucoses averaged 169 mg/dL, while right focal seizure (RFS, n = 23) glucoses averaged 131 mg/dL (p = 0.03). LFS occurred at mean time 17:02 while RFS occurred at 04:23. LFS spread to the contralateral side (n = 19) more than RFS (n = 2).

Conclusion

Seizure onset laterality and spread vary with glucose and time of seizure.

目的探讨血糖水平对癫痫发作模式的影响。材料与方法1例双颞部植入RNS/NeuroPace的1型糖尿病患者,癫痫发作次数和发作部位与连续组织葡萄糖相匹配。结果左局灶性发作(LFS, n = 22)平均血糖为169 mg/dL,右局灶性发作(RFS, n = 23)平均血糖为131 mg/dL (p = 0.03)。LFS发生在平均时间17:02,RFS发生在平均时间04:23。LFS向对侧扩散(n = 19)多于RFS (n = 2)。结论癫痫发作的侧边性和扩散随血糖和发作时间的不同而不同。
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引用次数: 4
Responsive neurostimulation for treatment of pediatric drug-resistant epilepsy 反应性神经刺激治疗小儿耐药癫痫
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.02.002
Nilika S. Singhal , Adam L. Numis , Morgan B. Lee , Edward F. Chang , Joseph E. Sullivan , Kurtis I. Auguste , Vikram R. Rao

Responsive neurostimulation for epilepsy involves an implanted device that delivers direct electrical brain stimulation in response to detection of incipient seizures. Responsive neurostimulation is a safe and effective treatment for adults with drug-resistant epilepsy, but although novel treatments are critically needed for younger patients, responsive neurostimulation is currently not approved for children with drug-resistant epilepsy. Here, we report a 16-year-old patient with seizures arising from eloquent cortex, who was successfully treated with responsive neurostimulation. This case highlights the potential utility of this therapy for pediatric patients and underscores the need for larger studies.

癫痫的反应性神经刺激包括一种植入式装置,该装置对早期癫痫发作的检测作出直接的脑电刺激。反应性神经刺激是一种安全有效的治疗成人耐药癫痫的方法,但是尽管年轻患者迫切需要新的治疗方法,反应性神经刺激目前尚未被批准用于治疗儿童耐药癫痫。在这里,我们报告一个16岁的患者癫痫发作引起的雄辩皮层,谁是成功的治疗反应性神经刺激。该病例强调了该疗法对儿科患者的潜在效用,并强调了进行更大规模研究的必要性。
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引用次数: 49
Epilepsy and concomitant obsessive–compulsive disorder 癫痫和伴随的强迫症
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.07.001
Jacob S. Bird , Emiy Shah , Paul Shotbolt

People with epilepsy (PWE) often suffer psychiatric symptoms which can impact them more than seizures. Affective and psychotic disorders are well recognized as occurring more frequently in PWE than the general population. Less is known about obsessive–compulsive disorder (OCD) in PWE, despite it being as disabling and distressing. We sought to explore the association between epilepsy and OCD with casereports by identifying ten PWE and concomitant OCD. Demographics, seizure classification, neurological, surgical, psychiatric and psychological treatment as well as quality of life were examined. A detailed analysis was performed for three of them, to explore the lived-experience of patients with the two conditions. This is followed by a discussion of how treatment for co-morbid epilepsy and OCD can be appropriately tailored to be patient specific and provide the greatest potential for improvement.

癫痫患者(PWE)经常遭受精神症状,这对他们的影响比癫痫发作更大。情感和精神障碍被公认为在PWE中比一般人群更频繁发生。人们对PWE患者的强迫症(OCD)知之甚少,尽管它会导致残疾和痛苦。我们试图通过确定10例PWE和伴随的OCD的病例报告来探讨癫痫和OCD之间的关系。调查了人口统计学、癫痫分类、神经学、外科、精神病学和心理治疗以及生活质量。对其中三例进行了详细的分析,以探讨两种情况下患者的生活经历。接下来将讨论如何根据患者的具体情况适当调整癫痫和强迫症的治疗,并提供最大的改善潜力。
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引用次数: 5
Evaluation of perampanel as monotherapy for focal seizures: Experience from open-label extension studies 评价perampanel作为局灶性癫痫的单一疗法:来自开放标签扩展研究的经验
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2017.11.001
Patrick Kwan , Scott Mintzer , Antonio Laurenza , Anna Patten , Karen Cartwright

Perampanel, a selective, non-competitive α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor antagonist, is approved for adjunctive treatment of focal seizures, with or without secondarily generalized seizures, and for primary generalized tonic–clonic seizures in patients with epilepsy aged ≥ 12 years. Perampanel was recently approved for monotherapy use for focal seizures in the U.S.A. Anti-seizure drug monotherapy may be preferable to polytherapy, which is generally associated with increased toxicity, non-compliance, and cost. Here, we report cases where patients had converted to perampanel monotherapy during open-label extension (OLEx) portions of 9 Phase II and III studies.

Of 2245 patients who enrolled in the OLEx studies, we identified 7 patients with drug-resistant focal seizures who discontinued all non-perampanel anti-seizure drugs and were maintained on perampanel monotherapy for ≥ 91 days until the end of data cut-off. Patients received perampanel monotherapy for up to 1099 days (157 weeks), most at a modal dose of 12 mg. Seizure data were available for 6 patients, of whom 5 had a ≥ 90% reduction in overall seizure frequency between baseline and their last 13-week period of monotherapy (3 were seizure-free). Perampanel monotherapy was generally well tolerated and the safety profile during perampanel monotherapy was consistent with clinical and post-marketing experience in the adjunctive setting.

This analysis included a small proportion of patients with highly drug-resistant focal seizures who converted to monotherapy during OLEx studies. While these limited data are encouraging in suggesting that perampanel might be useful as a monotherapy, further studies are required to explore outcomes in a less drug-resistant population, where a larger proportion of patients might benefit from monotherapy.

Perampanel是一种选择性、非竞争性α-氨基-3-羟基-5-甲基-4-异恶唑烯丙酸(AMPA)受体拮抗剂,被批准用于伴或不伴继发性全面性癫痫发作的局灶性癫痫发作的辅助治疗,以及年龄≥12岁癫痫患者的原发性全面性强直-阵挛性癫痫发作。Perampanel最近在美国被批准用于局灶性癫痫的单药治疗,抗癫痫药物单药治疗可能比多药治疗更可取,后者通常与毒性、不依从性和成本增加有关。在这里,我们报告了在9项II期和III期研究的开放标签扩展(OLEx)部分中患者转为perampanel单药治疗的病例。在纳入OLEx研究的2245例患者中,我们发现7例耐药局灶性癫痫患者停用了所有非perampanel抗癫痫药物,并维持perampanel单药治疗≥91天,直到数据截止。患者接受perampanel单药治疗长达1099天(157周),大多数剂量为12 mg。6例患者的癫痫发作数据可用,其中5例在基线和最后13周单药治疗期间总癫痫发作频率降低≥90%(3例无癫痫发作)。Perampanel单药治疗通常耐受性良好,Perampanel单药治疗期间的安全性与辅助治疗的临床和上市后经验一致。该分析包括一小部分在OLEx研究期间转为单药治疗的高度耐药局灶性癫痫患者。虽然这些有限的数据令人鼓舞,表明perampanel可能作为单一疗法有用,但需要进一步的研究来探索耐药程度较低的人群的结果,其中更大比例的患者可能从单一疗法中受益。
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引用次数: 12
Seizure remission and improvement of neurological function in sialidosis with perampanel therapy perampanel治疗唾液中毒的癫痫缓解和神经功能改善
Pub Date : 2018-01-01 DOI: 10.1016/j.ebcr.2018.02.005
Su-Ching Hu , Kun-Long Hung , Hui-Ju Chen , Wang-Tso Lee

A 15-year-old boy experienced myoclonic seizures for 3 years. He initially had occasional myoclonus, gradually progressive ataxia, tremors, and psychomotor and speech regression developed. Eventually, he exhibited nearly continuous myoclonus. He received treatment of sodium valproate, levetiracetam, clobazam, and phenobarbital, without efficacy. A ketogenic diet also proved ineffective. Adjunctive therapy with 4 mg/day of perampanel was started and was gradually titrated to 10 mg/day. The remission of myoclonic seizures was achieved within one month. The patient's neurological and cognitive functions improved to a certain degree during the following 20 months. Sialidosis was confirmed by the mutations of NEU1 gene.

一个15岁的男孩经历了3年的肌阵挛发作。他最初有偶尔的肌阵挛,逐渐进行性共济失调,震颤,精神运动和语言退化。最终,他表现出几乎连续的肌阵挛。他接受了丙戊酸钠、左乙拉西坦、氯巴唑和苯巴比妥的治疗,但没有效果。生酮饮食也被证明无效。辅助治疗开始使用4 mg/天的perampanel,并逐渐滴定至10 mg/天。肌阵挛发作在一个月内得到缓解。在接下来的20个月里,患者的神经和认知功能有了一定程度的改善。唾液中毒由NEU1基因突变证实。
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引用次数: 23
期刊
Epilepsy and Behavior Case Reports
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