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Ictal Cotard delusion as a manifestation of nonconvulsive status epilepticus: A case report and commentary 作为非惊厥性癫痫状态表现的异位科塔德妄想:病例报告和评论
IF 2.3 4区 医学 Q3 Medicine Pub Date : 2024-04-30 DOI: 10.1002/epd2.20221
Bernardo Crespo Pimentel, Giorgi Kuchukhidze, Marta Heyduk, Aljoscha Thomschewski, Eugen Trinka, Julia Höfler

Psychosis of epileptic origin can present a wide range of cognitive and affective symptoms and is often underrecognized. Usually occurring in the inter- and postictal phase, epileptic psychosis is mostly related to temporal lobe epilepsy. Here, we describe the clinical presentation and diagnostic workup including routine EEG recording and brain MRI of a 63-year-old woman expressing isolated nihilistic delusions comprising belief of being dead and denial of self-existence. EEG showed an ictal pattern fulfilling the Salzburg criteria of nonconvulsive status epilepticus and brain MRI revealed extensive peri-ictal hyperperfusion. Delusional symptoms and EEG abnormalities subsided after acute antiseizure treatment. Our case illustrates how nihilistic delusions can occur as a direct clinical correlate of seizure activity, thereby expanding the spectrum of ictal neuropsychiatric phenomena in temporal lobe epilepsy and highlighting the need to consider an epileptic origin in patients presenting with psychotic symptoms.

癫痫性精神病可表现出多种认知和情感症状,但往往未被充分认识。癫痫性精神病通常发生在发作间期和发作后阶段,大多与颞叶癫痫有关。在此,我们描述了一名 63 岁女性患者的临床表现和诊断过程,包括常规脑电图记录和脑部核磁共振成像,该患者表现出孤立的虚无主义妄想,包括相信自己已经死亡和否认自我存在。脑电图显示发作模式符合萨尔茨堡非惊厥性癫痫状态标准,脑磁共振成像显示发作周广泛高灌注。急性抗癫痫治疗后,妄想症状和脑电图异常缓解。我们的病例说明了虚无主义妄想是如何与癫痫发作活动直接相关的,从而扩大了颞叶癫痫发作性神经精神现象的范围,并强调了在患者出现精神症状时考虑癫痫起源的必要性。
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引用次数: 0
A randomized controlled educational pilot trial of interictal epileptiform discharge identification for neurology residents 针对神经内科住院医师的发作间期癫痫样放电识别随机对照教育试点试验
IF 1.9 4区 医学 Q3 CLINICAL NEUROLOGY Pub Date : 2024-04-26 DOI: 10.1002/epd2.20229
Fábio A. Nascimento, Jin Jing, Christopher Traner, Wan Yee Kong, Marcia Olandoski, Srishti Kapur, Erik Duhaime, Roy Strowd, Jeremy Moeller, M. Brandon Westover

Objective

To assess the effectiveness of an educational program leveraging technology-enhanced learning and retrieval practice to teach trainees how to correctly identify interictal epileptiform discharges (IEDs).

Methods

This was a bi-institutional prospective randomized controlled educational trial involving junior neurology residents. The intervention consisted of three video tutorials focused on the six IFCN criteria for IED identification and rating 500 candidate IEDs with instant feedback either on a web browser (intervention 1) or an iOS app (intervention 2). The control group underwent no educational intervention (“inactive control”). All residents completed a survey and a test at the onset and offset of the study. Performance metrics were calculated for each participant.

Results

Twenty-one residents completed the study: control (n = 8); intervention 1 (n = 6); intervention 2 (n = 7). All but two had no prior EEG experience. Intervention 1 residents improved from baseline (mean) in multiple metrics including AUC (.74; .85; p < .05), sensitivity (.53; .75; p < .05), and level of confidence (LOC) in identifying IEDs/committing patients to therapy (1.33; 2.33; p < .05). Intervention 2 residents improved in multiple metrics including AUC (.81; .86; p < .05) and LOC in identifying IEDs (2.00; 3.14; p < .05) and spike–wave discharges (2.00; 3.14; p < .05). Controls had no significant improvements in any measure.

Significance

This program led to significant subjective and objective improvements in IED identification. Rating candidate IEDs with instant feedback on a web browser (intervention 1) generated greater objective improvement in comparison to rating candidate IEDs on an iOS app (intervention 2). This program can complement trainee education concerning IED identification.

目的评估利用技术强化学习和检索练习教授学员如何正确识别发作间期癫痫样放电(IED)的教育计划的有效性。方法这是一项双机构前瞻性随机对照教育试验,涉及神经内科低年级住院医师。干预措施包括三段视频教程,重点是 IFCN 鉴定 IED 的六项标准,以及通过网络浏览器(干预措施 1)或 iOS 应用程序(干预措施 2)对 500 个候选 IED 进行评级并提供即时反馈。对照组不进行教育干预("非活动对照")。所有居民都在研究开始和结束时完成了调查和测试。结果21 名住院医师完成了研究:对照组(8 人);干预 1 组(6 人);干预 2 组(7 人)。除两人外,其余人员之前均无脑电图经验。干预 1 的住院医师在多个指标上都比基线(平均值)有所提高,包括 AUC(.74;.85;p <;.05)、灵敏度(.53;.75;p <;.05)和识别 IED/让患者接受治疗的信心水平 (LOC)(1.33;2.33;p <;.05)。干预 2 的住院医师在多个指标上都有所改善,包括 AUC(.81;.86;p <;.05)和识别 IED 的 LOC(2.00;3.14;p <;.05)以及尖波放电(2.00;3.14;p <;.05)。该项目在识别简易爆炸装置方面取得了明显的主观和客观进步。与通过 iOS 应用程序(干预 2)对候选 IED 进行评级相比,通过网络浏览器(干预 1)对候选 IED 进行评级并提供即时反馈,客观上有了更大的提高。该程序可作为受训人员简易爆炸装置识别教育的补充。
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引用次数: 0
Anticipatory anxiety of seizures in epilepsy: A common, complex, and underrecognized phenomenon? 癫痫患者对癫痫发作的预期焦虑:一种常见、复杂且未得到充分认识的现象?
IF 2.3 4区 医学 Q3 Medicine Pub Date : 2024-04-16 DOI: 10.1002/epd2.20224
Andres M. Kanner, Enrique Carrazana, Heidi M. Munger Clary, Adrian L. Rabinowicz, Edward Faught

The diagnosis of epilepsy is associated with loss of predictability, which invariably results in the fear of when and if future seizures will occur. For a subset of patients with epilepsy (PWE), there may be a pathological persistent fear of seizure occurrence, resulting in limitations to daily activities through avoidant behaviors. Paradoxically, the research of anticipatory anxiety of seizures (AAS; also referred to as seizure phobia) has been practically nonexistent and, not surprisingly, this condition remains underrecognized by clinicians. The available data are derived from three small case series of patients followed in tertiary epilepsy centers. In this study, we review the available data on the reported clinical manifestations of AAS in PWE, and of the potential role of variables associated with it, such as personal and family psychosocial and psychiatric history and epilepsy-related variables. In addition, we review the need for the creation of screening tools to identify patients at risk of AAS and discuss potential treatment strategies, which could be considered as part of the comprehensive management for PWE.

癫痫的诊断与丧失可预测性有关,这必然会导致对未来何时以及是否会出现癫痫发作的恐惧。对于一部分癫痫患者(PWE)来说,可能会对癫痫发作产生病态的持续恐惧,从而通过回避行为限制日常活动。令人不解的是,对癫痫发作预期性焦虑(AAS,也称为癫痫发作恐惧症)的研究几乎不存在,临床医生对这种情况的认识不足也就不足为奇了。现有数据来自三个小型病例系列,这些病例都是在三级癫痫中心随访的患者。在本研究中,我们回顾了现有数据,这些数据涉及所报告的癫痫蛛网膜下腔出血的临床表现,以及与之相关的变量的潜在作用,如个人和家庭的心理社会史和精神病史以及癫痫相关变量。此外,我们还回顾了创建筛查工具的必要性,这些工具可用于识别有可能患羊角风的患者,我们还讨论了潜在的治疗策略,这些策略可作为对患有羊角风的患者进行综合管理的一部分。
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引用次数: 0
Battery depletion in patients treated with anterior thalamic stimulation for drug-resistant epilepsy 接受丘脑前部刺激治疗耐药性癫痫患者的电池耗尽情况
IF 1.9 4区 医学 Q3 CLINICAL NEUROLOGY Pub Date : 2024-04-16 DOI: 10.1002/epd2.20226
Michał Sobstyl, Magdalena Konopko, Aleksandra Wierzbicka
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引用次数: 0
Ma2 antibody-associated limbic encephalitis: The early etiology treatment may modify the disease clinical trajectory Ma2抗体相关性肢端脑炎:早期病因治疗可改变疾病的临床轨迹
IF 2.3 4区 医学 Q3 Medicine Pub Date : 2024-04-15 DOI: 10.1002/epd2.20225
Pietro Mattioli, Stefano Grisanti, Federico Massa, Silvia Morbelli, Silvia Boni, Alessandro Beronio, Giacomo Rebella, Luca Roccatagliata, Lino Nobili, Diego Franciotta, Elisa Micalizzi, Flavio Nobili, Dario Arnaldi, Flavio Villani, Luana Benedetti

Paraneoplastic encephalitis includes neurological conditions with autoantibodies against neuronal proteins, likely triggered by the underlying tumor antigens.1 We report two patients with paraneoplastic limbic encephalitis1 with “high-risk-for-cancer” antibodies against Ma2 antigen and non-typical tumors. These cases allow consideration of autoimmune epilepsy's pathogenesis, the importance of early diagnosis, and therapy.

A 21-year-old male admitted to the Infectious Disease Unit due to confusion, visual hallucinations, and upper limbs myoclonus, following a 4-month history of antipyretic-resistant fever, sore throat, and hyperphagia. Cerebrospinal fluid (CSF) analysis was normal, blood examinations showed pancytopenia. Brain MRI, [18F]FDG-PET, and EEG (Figure 1A–D) suggested central nervous system inflammation involving basal ganglia and mesial temporal lobes. Screening for infections was negative. Anti-Ma2 antibodies were found in serum and CSF (indirect immunofluorescence on primate cerebellum, and dot-blot; Euroimmun, Germany). IV methylprednisolone (1 g/day for 5 days) and immunoglobulins (IVIg) (.4 g/kg/day for 5 days) yielded no clinical improvement. Total body CT, [18F]FDG-PET, pelvis, and testicular ultrasound excluded neoplasms, but bone marrow analysis, 6 months after symptoms' onset, showed a clonal population of CD19/CD20dim/CD21 B lymphocytes, indicating a mature B-cell indolent lymphoproliferative disorder not requiring treatment. Hospital-acquired sepsis hindered further immunosuppressive therapies and led to death (7 months after symptoms' onset).

The second patient, a 30-year-old male, presented with asthenia, insomnia, mild fever, and daily, short-lasting episodes of shivering, horripilation, heart palpitations, and unpleasant taste. Subtle sleep-related involuntary movements were reported. No EEG was performed at the time. 1 month after symptom onset, a right testicular mass was noticed and orchiectomy performed. Pathology disclosed a post-puberal teratoma. 2 months later, a scheduled total body CT showed enlargement of retroperitoneal lymph-nodes, which were completely removed. Pathology disclosed a post-puberal teratoma with embryonal cancer focus. The patients started clinical and radiological follow-up, with no further localizations detected. 5 months later, he presented a focal-to-bilateral seizure, promptingantiseizure medications. Brain MRI and [18F]FDG-PET indicated limbic encephalitiswith EEG showing interictal epileptiform discharges in left temporal lobe (Figure 1E–H). Anti-Ma2 antibodies were detected in serum and CSF (indirect immunofluorescence on primate cerebellum, and dot-blot; Euroimmun, Germany), but not anti-NMDAR (antigen-specific cell-based assay (Euroimmun, Germany)). IVIg (.4 g/kg/day for 5 days), methylprednisolone (500 mg/day for 3 days) followed by oral prednisone (50 mg/day) led to seizure frequency reduc

总之,与文献1 一致,副肿瘤性脑炎患者应考虑早期病因治疗和继续免疫治疗。Arnaldi D.从 Fidia 公司获得了演讲和参与董事会的酬金;Morbelli S.从 G.E. Healthcare 公司获得了演讲酬金;Nobili F.从罗氏公司获得了参与董事会的酬金,从 Bial e G.E. Healthcare 公司获得了演讲酬金。Villani F. 从 UCB Pharma、Angelini Pharma、EISAI、Lusofarmaco、Bial 和 Jazz Pharma 获得了演讲酬金和参与董事会的费用。Micalizzi E.从安吉里尼制药公司获得了参与董事会的酬金。其他作者没有任何需要披露的信息。
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引用次数: 0
A controversial question: Can morphometry and clinical history be enough to diagnose hippocampal dysplasia? 一个有争议的问题:形态测量和临床病史足以诊断海马发育不良吗?
IF 2.3 4区 医学 Q3 Medicine Pub Date : 2024-04-08 DOI: 10.1002/epd2.20222
Ianne Lucena Arruda, Rivus Ferreira Arruda, Rayanne Maria Brandão da Silveira, Jeana Torres Corso Duarte, Mirian Salvadori Bittar Guaranha, Laura Maria Guilhoto, Henrique Carrete Júnior, Joao Norberto Stavale, Ricardo Silva Centeno, Elza Marcia Targas Yacubian, Jose Eduardo Peixoto-Santos

The presence of dysmorphic neurons with strong cytoplasmatic accumulation of heavy non-phosphorylated neurofilament is crucial for the diagnostics of focal cortical dysplasia type II (FCDII). While ILAE's classification describes neocortical dysplasias, some groups have reported patients with mesial t abnormal neurons in the hippocampus of mesial temporal lobe epilepsy. Here we report a patient with such abnormal neurons in the hippocampus and compared it with previous reports of hippocampal dysplasia. Finally, we discuss the need for diagnostic criteria of hippocampal dysplasia.

畸形神经元胞浆内大量积聚重型非磷酸化神经丝是诊断局灶性皮质发育不良 II 型(FCDII)的关键。虽然 ILAE 的分类描述的是新皮质发育不良,但也有一些研究小组报告了颞叶中叶癫痫患者的海马中侧 t 型异常神经元。在此,我们报告了一名海马神经元异常的患者,并将其与之前的海马发育不良报告进行了比较。最后,我们讨论了制定海马发育不良诊断标准的必要性。
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引用次数: 0
Ictal kissing: Review of literature and report of 5 cases 直肠吻合:文献综述和 5 个病例的报告。
IF 2.3 4区 医学 Q3 Medicine Pub Date : 2024-04-01 DOI: 10.1002/epd2.20208
Parthvi Ravat, Neeraj Jain, Mayur Thakkar, Mayuri Kalika, Sangeeta Ravat

Ictal kissing (IK) is a rare type of automatism observed during epileptic seizures. Despite its uncommon occurrence, understanding the underlying mechanisms, the role of emotions, and the level of consciousness during seizures with IK is essential in providing a comprehensive understanding of epilepsy. We describe five cases (.13%) of IK after performing a retrospective analysis of 3794 long-term, ictal video-EEGs from an epilepsy monitoring unit in Mumbai, India. Our patients with drug-resistant epilepsy showed IK had a wide epileptogenic zone. We discuss the current hypotheses on the mechanisms behind IK, the involvement of temporal lobe structures, and the implications of awareness during seizures. The review concludes by suggesting future directions for research to elucidate the complex phenomenon of IK further.

间歇性接吻(IK)是癫痫发作时观察到的一种罕见的自动症。尽管这种现象并不常见,但了解其潜在的机制、情绪的作用以及癫痫发作时的意识水平对于全面了解癫痫至关重要。我们对印度孟买一家癫痫监测机构的 3794 份长期发作期视频电子脑电图进行了回顾性分析,并描述了五例 IK 病例(0.13%)。我们的耐药性癫痫患者显示 IK 的致痫区很宽。我们讨论了目前关于 IK 背后机制的假设、颞叶结构的参与以及癫痫发作期间意识的影响。综述最后提出了进一步阐明复杂的 IK 现象的未来研究方向。
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引用次数: 0
Epilepsy and ionic channels 癫痫与离子通道
IF 2.3 4区 医学 Q3 Medicine Pub Date : 2024-03-27 DOI: 10.1684/j.1950-6945.2002.tb00489.x
Patrice Roll, Pierre Szepetowski
Ion channels play critical roles in a broad range of physiological processes. In particular, they represent a common basis for the excitable properties of different tissues. Consequently, ion channels of many different types have been implicated in several human genetic disorders of the heart, brain and skelctal muscle. An increasing number of genes encoding neurotransmitter‐ or voltage‐gated ion channel subunits have proved to be mutated in various idiopathic human epilepsies as well as in different animal models. The epilepsies could thus be considered as one of many paroxysmal disorders that are due to mutations in ion channel genes, the so‐called channelopathies. However, recent data supported the hypothesis that other genes with different properties could also be responsible for human idiopathic epilepsies, thus opening new and exciting areas of research. Effort is still needed to identify the genes responsible for the large variety of other epileptic disorders inherited as Mendelian traits and evaluate the role of these genes in the more common and polygenic forms. Defining the genetic bases of the latter will also require that exhaustive association studies are performed. These studies may help understand the pathophysiology of human epilepsies and represent the first step towards the discovery of new therapeutic targets, as exemplified in the case of the KCNQ potassium channels.
离子通道在广泛的生理过程中发挥着关键作用。特别是,它们是不同组织兴奋特性的共同基础。因此,许多不同类型的离子通道都与多种人类遗传性心脏、大脑和直肠肌肉疾病有关。越来越多的编码神经递质或电压门控离子通道亚基的基因被证明在各种特发性人类癫痫以及不同的动物模型中发生了突变。因此,癫痫可被视为由离子通道基因突变引起的多种阵发性疾病之一,即所谓的通道病。然而,最近的数据支持了这样的假设,即具有不同特性的其他基因也可能是人类特发性癫痫的病因,从而开辟了令人兴奋的新研究领域。目前仍需努力确定导致作为孟德尔遗传性状遗传的大量其他癫痫疾病的基因,并评估这些基因在更常见的多基因形式中的作用。要确定后者的遗传基础,还需要进行详尽的关联研究。这些研究可能有助于了解人类癫痫的病理生理学,并为发现新的治疗目标迈出第一步,KCNQ 钾通道就是一个例子。
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引用次数: 0
Congress & Meetings 大会和会议
IF 2.3 4区 医学 Q3 Medicine Pub Date : 2024-03-27 DOI: 10.1684/j.1950-6945.2000.tb00451.x
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引用次数: 0
Evolution of epilepsy surgery in childhood: the neurologist's point of view 儿童癫痫手术的演变:神经学家的观点
IF 2.3 4区 医学 Q3 Medicine Pub Date : 2024-03-27 DOI: 10.1684/j.1950-6945.1999.tb00332.x
Jean Aicardi
Surgical treatment of epilepsy in children has been slow to develop because of uncertainties regarding the prognosis of early epilepsies, difficulties in the diagnosis of causal lesions, the frequency of severe, extensive brain damage, and technical problems. Surgery for epilepsy in infants and children raises problems quite different from those in adults. This applies especially to severe infantile epilepsies for which the aim of treatment may be more to facilitate neurodevelopment than to control the seizures. In addition, intractability cannot be defined in the same terms at different ages, the time scale being usually much shorter in young children and the operations required often extensive. The results of surgery in childhood are comparable to those in adults. However, indications for surgery and assessment of its results in catastrophic epilepsies of infants requires different rules to those that apply to adults and older children.
由于对早期癫痫预后的不确定性、病因诊断的困难、严重和广泛脑损伤的发生率以及技术问题,儿童癫痫的外科治疗一直发展缓慢。婴幼儿癫痫的手术治疗所面临的问题与成人截然不同。这尤其适用于严重的婴幼儿癫痫,因为治疗的目的可能更多是促进神经发育,而不是控制癫痫发作。此外,难治性在不同年龄段的定义也不尽相同,幼儿的治疗时间通常要短得多,所需的手术通常也很广泛。儿童期手术的效果与成人相当。然而,婴儿灾难性癫痫的手术指征和手术效果评估需要与成人和较大儿童不同的规则。
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引用次数: 0
期刊
Epileptic Disorders
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