Epilepsy and Behavior Reports最新文献_第6页

Ultra-long-term subcutaneous EEG recordings in ten epilepsy patients: Experiences and circadian rhythms in epileptiform discharges 10例癫痫患者的超长期皮下脑电图记录：癫痫样放电的经历和昼夜节律

IF 1.8 Q3 CLINICAL NEUROLOGY

Epilepsy and Behavior Reports

Pub Date : 2025-05-20 DOI: 10.1016/j.ebr.2025.100781

S.J. van Norden , K.H. Kho , A.M. Meppelink , J.J. Ardesch , M.C. Tjepkema-Cloostermans , M.J.A.M. van Putten

Recently, a subcutaneous electroencephalography (sqEEG) electrode became available, enabling ultra-long-term EEG recordings. In our PREDYct study, we aim to predict the efficacy of vagus nerve stimulation (VNS) in epilepsy patients. The study records sqEEG for up to fifteen months, including a 2–3 month period before VNS implantation. Here we report on initial patient experiences, compliance and recordings of epileptiform discharges (EDs). We included the first 10 participants from our ongoing PREDYct study. We calculated recording compliance for each patient. Patients rated their pain during electrode implantation, wearing and explantation using the visual analogue scale (VAS). Approximately two weeks of data from each patient were visually assessed and all EDs were annotated. We used these annotations to evaluate circadian rhythms in ED rate and duration and to compare seizure diaries with annotated ictal patterns. Electrode implantation and explantation were well-tolerated with a median VAS of 4 and 2, respectively. Wearing the electrode was also well-tolerated with median VAS scores of 0 and 0.5 at two and fifteen months, respectively. On average, patients wore the electrode for 441 days with an overall compliance of 27 %, resulting in 28,331 h of EEG. A circadian rhythm in ED rate was observed in three patients, while one patient exhibited a circadian rhythm in ED duration. A comparison of reported seizures and annotated ictal patterns revealed discrepancies. Ultra-long-term sqEEG is well-tolerated and has the potential to be a valuable tool for monitoring treatment. Nevertheless, it remains a challenge but crucial to attain high recording compliance.

最近，一种皮下脑电图（sqEEG）电极可用，使超长期脑电图记录成为可能。在我们的PREDYct研究中，我们旨在预测迷走神经刺激（VNS）对癫痫患者的疗效。该研究记录了长达15个月的sqEEG，包括VNS植入前的2-3个月。在这里，我们报告了患者的初始经验，依从性和记录癫痫样放电（EDs）。我们从正在进行的PREDYct研究中纳入了前10名参与者。我们计算每位患者的记录依从性。患者使用视觉模拟量表（VAS）对电极植入、佩戴和取出过程中的疼痛程度进行评分。对每位患者大约两周的数据进行视觉评估，并对所有急诊科进行注释。我们使用这些注释来评估ED发生率和持续时间的昼夜节律，并将癫痫发作日记与注释的发作模式进行比较。电极植入和植入术耐受良好，VAS中值分别为4分和2分。佩戴电极也具有良好的耐受性，在2个月和15个月时，VAS评分中位数分别为0和0.5。平均而言，患者佩戴电极的时间为441天，总体顺应性为27%，EEG时间为28331小时。在3例患者中观察到ED发生率的昼夜节律，而1例患者在ED持续时间上表现出昼夜节律。比较报告的癫痫发作和注释的精神模式揭示了差异。超长期sqEEG耐受性良好，有潜力成为监测治疗的宝贵工具。尽管如此，实现高度的记录遵从性仍然是一个挑战，但也是至关重要的。

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引用次数: 0

Mental health intervention for a child with developmental and/or epileptic encephalopathy with spike-wave activation in sleep (D/EE-SWAS) 发展性和/或癫痫性脑病伴睡眠尖波激活（D/EE-SWAS）儿童的心理健康干预

IF 1.8 Q3 CLINICAL NEUROLOGY

Epilepsy and Behavior Reports

Pub Date : 2025-05-17 DOI: 10.1016/j.ebr.2025.100780

C. Reilly , Roz Shafran , Helen Cross , Millie Wagstaff , Bhavna Sidhpara , J. Idowu , C. Meades , S.D. Bennett

Children with Developmental and Epileptic Encephalopathies experience neurodevelopmental problems from both the epileptic activity and the underlying cause of the condition. Children with Developmental and/or Epileptic Encephalopathy with spike-wave activation in sleep (D/EE-SWAS) experience cognitive and behavioral regression/stagnation that occurs in tandem with marked spike-wave activation in sleep (SWAS). Children with epilepsy often have co-occurring mental health problems that are often not recognized or supported. There is increasing evidence these co-occurring mental health problems can be treated with psychological interventions. The mental health and cognitive difficulties that accompany SWAS have a significant impact on child and family quality of life. There is limited data on the treatment of mental health difficulties for children with D/EE-SWAS. We describe the use of the Mental Health Intervention in Children with Epilepsy (MICE) in a child with D/EE-SWAS who had experienced significant cognitive regression and behavioral difficulties. The intervention was delivered with the child’s parents via video and consisted of 19 sessions delivered by an assistant psychologist. The focus of the intervention was reducing behaviours of concern via evidence-based behavioural parenting strategies. The intervention resulted in clear progress towards parental chosen goals (Goal Based Outcomes) and a reduction of symptoms on validated measures of behavior. Qualitative feedback via parental interview was that the intervention was very useful, giving the parents everyday strategies that they could effectively employ with the child in the home environment. This case demonstrates that a mental health intervention based on behavioural parenting strategies can be useful for children with D/EE-SWAS.

儿童发展性和癫痫性脑病经历神经发育问题从癫痫活动和条件的根本原因。发展性和/或癫痫性脑病伴睡眠中尖峰波激活（D/EE-SWAS）的儿童会经历认知和行为倒退/停滞，这与睡眠中显著的尖峰波激活（SWAS）同时发生。患有癫痫的儿童往往同时出现精神健康问题，而这些问题往往得不到承认或支持。越来越多的证据表明，这些同时发生的精神健康问题可以通过心理干预来治疗。SWAS伴随的心理健康和认知困难对儿童和家庭的生活质量有重大影响。关于D/EE-SWAS儿童心理健康困难治疗的数据有限。我们描述了在患有D/EE-SWAS的儿童中使用癫痫儿童心理健康干预（MICE），这些儿童经历了显著的认知退化和行为困难。干预是由一名助理心理学家通过视频与孩子的父母一起进行的，包括19次会议。干预的重点是通过基于证据的行为养育策略减少担忧行为。干预导致朝着父母选择的目标（基于目标的结果）取得明显进展，并减少了经过验证的行为测量的症状。通过父母访谈得到的定性反馈是，干预非常有用，为父母提供了他们可以在家庭环境中有效地与孩子相处的日常策略。这一案例表明，基于行为养育策略的心理健康干预对患有D/EE-SWAS的儿童是有用的。

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引用次数: 0

The challenges in establishing and Upholding an adult Level III epilepsy center in an underserved area in the United states 在美国服务不足的地区建立和维护成人III级癫痫中心的挑战

IF 1.8 Q3 CLINICAL NEUROLOGY

Epilepsy and Behavior Reports

Pub Date : 2025-05-13 DOI: 10.1016/j.ebr.2025.100779

Kevin T. Dao , Clins Chacko , Shaan Braich , Lauren Liu , Neela Zalmay , Chrystal Nguyen , Kurupath Radhakrishnan , Charles Y. Liu , Hari Prasad Kunhi-Veedu

Despite vast improvement, even today, care of people with epilepsy (PWE) in many parts of the USA remains unevenly distributed and disjointed. We reviewed the experience of the Kern Medical Epilepsy Center (KMEC) to highlight the challenges in establishing and maintaining an epilepsy center in an underserved region of Southern California. We analyzed the prospectively collected data from 2018 through 2021 of KMEC, a National Association of Epilepsy Centers Level III accredited facility that serves PWE people residing in Central Valley, California. During the study period, there were 246 admissions to the Epilepsy Monitoring Unit, KMEC for detailed evaluation of difficult to treat epilepsy. Most patients were either of Hispanic descent or Caucasian. Thirty-seven patients underwent surgical interventions (8 resective surgery, and 23 vagus nerve stimulators and 6 responsive neurostimulators) with favorable outcomes in the majority. Many of them required referrals to the Level IV center at University of Southern California, Los Angeles, which were hindered by difficulties with getting appointments, transportation, insurance coverage, payment for treatment, and sluggish treatment processes. Our experience demonstrates that, despite considerable challenges, epilepsy care can be coordinated across complex and competing health systems separated by large geographic distances through creative, physician-driven strategies of resource sharing and goal alignment across the health care ecosystem. We hope that our experience will facilitate future efforts to integrate epilepsy care beyond this region in the USA, and elsewhere in the world. sluggish treatment processes. and elsewhere in the world.

尽管取得了巨大的进步，但即使在今天，美国许多地区对癫痫患者的护理仍然分布不均，脱节。我们回顾了克恩医疗癫痫中心（KMEC）的经验，以强调在南加州服务不足的地区建立和维持癫痫中心所面临的挑战。我们分析了2018年至2021年KMEC的前瞻性收集数据，KMEC是美国国家癫痫中心协会III级认证机构，为居住在加利福尼亚州中央谷的PWE患者提供服务。在研究期间，有246例入院的癫痫监测单位，KMEC对难治性癫痫进行了详细评估。大多数患者要么是西班牙裔，要么是白种人。37例患者接受手术干预（切除手术8例，迷走神经刺激23例，反应性神经刺激6例），大多数结果良好。他们中的许多人需要转介到南加州大学洛杉矶分校的四级中心，但由于预约、交通、保险、治疗费用以及缓慢的治疗过程等方面的困难，他们的工作受到了阻碍。我们的经验表明，尽管面临相当大的挑战，但通过在整个卫生保健生态系统中采用创造性的、医生驱动的资源共享和目标一致战略，可以在地理距离遥远的复杂和相互竞争的卫生系统中协调癫痫治疗。我们希望我们的经验将促进未来在美国和世界其他地区整合癫痫治疗的努力。治疗过程迟缓。在世界其他地方也是如此。

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引用次数: 0

Reduced VNS settings paradoxically decreases seizure burden in a patient following resolution of sleep disordered breathing 减少VNS设置矛盾地减少癫痫发作的负担，在解决睡眠呼吸障碍的病人

IF 1.8 Q3 CLINICAL NEUROLOGY

Epilepsy and Behavior Reports

Pub Date : 2025-05-06 DOI: 10.1016/j.ebr.2025.100778

Austin Sponaugle , Rebecca S. Stainman , Christopher M. Carosella

Vagus nerve stimulation (VNS) can be a highly effective treatment option for patients with drug resistant epilepsy. Notably, VNS has demonstrated side effects including a unique form of sleep disordered breathing known as vagus nerve stimulator associated sleep disordered breathing (VaS). However, the ways in which VaS interacts with seizure frequency is unknown. We report a case of a 28-year-old woman who presented to our department with complaints of worsening sleep quality 3 years following VNS implantation. Upon polysomnographic (PSG) evaluation, it was discovered that she suffered from VaS. The patient’s VNS output current was then down titrated during a subsequent PSG resulting in resolution of her VaS. Interestingly, despite downward titration of her nighttime VNS output current, the patient demonstrated a prolonged seizure free period, highlighting the complex interactions between VNS, VaS and seizure frequency. In cases where patients present with both epilepsy and VaS, physicians should be aware of this complex relationship. Further, this case highlights that down titration of VNS settings may result in not just improvement in VaS but may also result in improvement in seizure frequency.

迷走神经刺激（VNS）是治疗耐药癫痫的一种非常有效的方法。值得注意的是，VNS已经证明了副作用，包括一种独特的睡眠呼吸障碍形式，即迷走神经刺激相关的睡眠呼吸障碍（VaS）。然而，VaS与癫痫发作频率的相互作用方式尚不清楚。我们报告一位28岁的女性，在VNS植入3年后，因睡眠质量恶化而到我科就诊。经多导睡眠图（PSG）评估，发现她患有VaS。然后在随后的PSG中降低患者的VNS输出电流，从而导致她的VaS分辨率。有趣的是，尽管她的夜间VNS输出电流下降，但患者表现出延长的无癫痫发作期，突出了VNS， VaS和癫痫发作频率之间复杂的相互作用。在患者同时患有癫痫和VaS的情况下，医生应该意识到这种复杂的关系。此外，本病例强调，降低VNS设置的滴定不仅可以改善VaS，还可以改善癫痫发作频率。

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引用次数: 0

Successful treatment of super-refractory focal status epilepticus: Surgery, vagus nerve stimulation, and botox for epilepsia partialis continua 成功治疗超难治性局灶性癫痫持续状态：手术、迷走神经刺激和肉毒杆菌毒素治疗部分持续性癫痫

IF 1.8 Q3 CLINICAL NEUROLOGY

Epilepsy and Behavior Reports

Pub Date : 2025-05-02 DOI: 10.1016/j.ebr.2025.100775

N. Zalmay , G. Nune , C.N. Heck , K.T. Dao , B.T. Ly , J. Ipe , C.Y. Liu , H.P. Kunhi Veedu

Super-refractory status epilepticus (SRSE) is a life-threatening condition characterized by persistent seizures lasting beyond seven days despite anesthetic therapy. Managing SRSE is challenging, particularly in cases resistant to conventional treatments. This case report presents a 41-year-old female with prolonged SRSE secondary to focal epilepsy, successfully treated with surgical resection, vagus nerve stimulation (VNS), and botulinum toxin (BoNT) therapy. The patient initially presented with left focal motor status epilepticus and a right frontal intracerebral hematoma. Despite an emergency craniotomy, her seizures persisted, leading to prolonged SRSE. After multiple failed sedation weaning attempts, a stepwise intervention approach was implemented consisting of an Electrocorticography (ECoG)-guided surgical resection of the residual epileptic focus, VNS implantation for long-term seizure modulation, BoNT therapy for residual epilepsia partialis continua (EPC). The combined approach of surgical resection and VNS implantation successfully terminated the status epilepticus. Residual Epilepsia Partialis Continua (EPC) was effectively managed with botulinum toxin therapy (BoNT). This case underscores the potential efficacy of combining surgical resection and VNS implantation in treating SRSE and highlights the beneficial effects of BoNT in managing residual EPC, offering a potential treatment pathway for similar refractory cases.

超难治性癫痫持续状态（SRSE）是一种危及生命的疾病，其特征是持续发作超过7天，尽管有麻醉治疗。管理SRSE具有挑战性，特别是在对常规治疗有抵抗力的情况下。本病例报告一例41岁女性继发于局灶性癫痫的长期SRSE，通过手术切除、迷走神经刺激（VNS）和肉毒杆菌毒素（BoNT）治疗成功。患者最初表现为左侧局灶性癫痫持续状态和右侧额叶脑内血肿。尽管进行了紧急开颅手术，但她的癫痫持续发作，导致长期的SRSE。在多次失败的镇静脱机尝试后，实施了逐步干预方法，包括在皮质电图（ECoG）引导下手术切除残留的癫痫病灶，植入VNS用于长期癫痫发作调节，BoNT治疗残留的部分连续性癫痫（EPC）。手术切除和VNS植入相结合的方法成功地终止了癫痫持续状态。肉毒杆菌毒素治疗（BoNT）可有效治疗残余部分持续性癫痫（EPC）。本病例强调了联合手术切除和VNS植入治疗SRSE的潜在疗效，并强调了BoNT治疗残余EPC的有益作用，为类似难治性病例提供了潜在的治疗途径。

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引用次数: 0

Academic competence and special educational needs as outcomes of early onset epilepsy: A population-based prospective follow-up study 学术能力和特殊教育需求作为早发性癫痫的结局：一项基于人群的前瞻性随访研究

IF 1.8 Q3 CLINICAL NEUROLOGY

Epilepsy and Behavior Reports

Pub Date : 2025-04-30 DOI: 10.1016/j.ebr.2025.100777

Kati Rantanen , Jenna Mäntylä , Eeva Kettunen , Annina Saunajoki , Kai Eriksson

Epilepsy in children is associated with significant academic challenges, particularly among those with early-onset seizures and comorbid intellectual disabilities (ID). This study aims to bridge gaps in existing research by examining long-term academic outcomes and special educational needs (SEN) in a cohort of children with early-onset epilepsy, including those with complicated epilepsy (CE) and major comorbidities. The study followed a population-based cohort of 64 children with epilepsy (CWE) aged 3–6 years, identified from the Pediatric Neurology Unit at Tampere University Hospital. Six years later, 43 children (67 %) participated in the follow-up. Academic competence was assessed using the Teacher Report Form (TRF), which also provided data on SEN. The findings revealed that 67 % of participants had low academic competence, with a significant proportion requiring special educational support. Children with CE had notably lower academic performance compared to those with uncomplicated epilepsy (UE). The need for SEN was higher among children with CE (81 %) than those with UE (24 %), with epilepsy type emerging as a significant predictor for SEN. Despite the small sample size and limitations in generalizability, the study underscores the pervasive impact of early-onset epilepsy on academic outcomes and highlights the necessity for ongoing educational support. Future research should focus on larger cohorts and explore the efficacy of tailored educational interventions to improve the academic performance and quality of life for CWE. Regular screening and collaboration with educators are essential to address the unique needs of these children effectively.

儿童癫痫与重大的学业挑战有关，特别是那些有早发性癫痫和共病性智力残疾（ID）的儿童。本研究旨在通过研究一组早发性癫痫儿童的长期学业成果和特殊教育需求（SEN），包括那些患有复杂癫痫（CE）和主要合并症的儿童，来弥补现有研究的空白。该研究对64名3-6岁癫痫患儿（CWE）进行了基于人群的队列研究，这些患儿来自坦佩雷大学医院儿科神经内科。6年后，43名儿童（67%）参加了随访。学术能力评估采用教师报告表（TRF），并提供sen数据。调查结果显示，67%的参与者学术能力较低，其中很大一部分需要特殊的教育支持。与未合并癫痫（UE）的儿童相比，CE儿童的学习成绩明显较低。CE患儿（81%）比UE患儿（24%）对SEN的需求更高，癫痫类型成为SEN的重要预测因素，尽管样本量小且普遍性有限，但该研究强调了早发性癫痫对学业成绩的普遍影响，并强调了持续教育支持的必要性。未来的研究应该集中在更大的队列上，并探索量身定制的教育干预措施对改善CWE学习成绩和生活质量的效果。定期筛查和与教育工作者合作对于有效地解决这些儿童的独特需求至关重要。

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引用次数: 0

Investigation of the relationship between 0.5–1200 Hz signal characteristics of cortical high-frequency oscillations and epileptogenicity through multivariate analysis 通过多变量分析探讨0.5 ~ 1200hz皮层高频振荡信号特征与致痫性的关系

IF 1.8 Q3 CLINICAL NEUROLOGY

Epilepsy and Behavior Reports

Pub Date : 2025-04-24 DOI: 10.1016/j.ebr.2025.100776

Takashi Shibata , Hiroki Tsuchiya , Mari Akiyama , Tomoyuki Akiyama , Masao Matsuhashi , Katsuhiro Kobayashi

Fast ripples (FRs) (250–500 Hz) on the electroencephalogram (EEG) are closely related to epileptogenicity and are important to determine cortical regions resected in epilepsy surgery. However, FR-related epileptogenicity may be variable, and may depend on information associated with FRs. We enrolled nine epilepsy patients who had undergone intracranial 5 kHz-sampling-rate EEG for surgical treatment and had final Engel class I outcomes. Three electrodes were selected from each epileptogenic area (EA) and the unlikely EA (the region outside the EA) in each patient. Up to 100 candidate FRs were automatically detected from interictal nocturnal EEG at each of the selected electrodes and were visually reviewed independently by two researchers. Multivariate logistic regression analysis was performed using the frequency and log-power value of the corresponding FRs, presence of concurrent spike, ripple, very-high-frequency oscillations (vHFO)1 (500–600 Hz), and vHFO2 (600–1200 Hz), and whether the timing of the spectral peak of corresponding FRs was in the peak–trough or trough–peak transition of each slow activity (0.5–1, 1–2, 2–3, 3–4, and 4–8 Hz) as independent variables. Factors significantly related to epileptogenicity were FR power, the concurrent presence of spike and vHFO2, coupling with 0.5–1 and 1–2 Hz slow waves in the peak–trough transition, and coupling with 3–4 and 4–8 Hz slow waves in the trough–peak transition. Multifactorial analysis of FRs may increase their usefulness, potentially leading to improved treatment outcomes in epilepsy surgery.

脑电图（EEG）上的快速波纹（FRs）（250 - 500hz）与癫痫致痫性密切相关，对于确定癫痫手术中切除的皮质区域非常重要。然而，FRs相关的致痫性可能是可变的，并且可能取决于与FRs相关的信息。我们招募了9例癫痫患者，他们接受了颅内5khz采样率脑电图手术治疗，最终获得了Engel I级结果。从每个患者的每个致痫区（EA）和不太可能的EA （EA外的区域）中选择三个电极。在每个选择的电极上，从夜间脑电图中自动检测到多达100个候选FRs，并由两名研究人员独立进行视觉检查。以相应FRs的频率和对数功率值，并发峰、纹波、甚高频振荡（vHFO）1 （500 - 600hz）和vHFO2 （600 - 1200hz）的存在，以及相应FRs的谱峰出现时间是否在各慢活动（0.5-1、1 - 2、2-3、3-4和4 - 8hz）的波谷或波谷过渡为自变量，进行多变量logistic回归分析。与致痫性显著相关的因素有：FR功率、峰值和vHFO2同时存在、峰谷转换时与0.5-1和1-2 Hz慢波耦合、谷峰转换时与3-4和4-8 Hz慢波耦合。对FRs进行多因素分析可能会增加其有效性，从而有可能改善癫痫手术的治疗效果。

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引用次数: 0

Ictal cold shiver caused by autoimmune limbic encephalitis: A case report and literature review 自身免疫性边缘脑炎致急性寒颤1例并文献复习

IF 1.8 Q3 CLINICAL NEUROLOGY

Epilepsy and Behavior Reports

Pub Date : 2025-04-22 DOI: 10.1016/j.ebr.2025.100773

Yosuke Takeuchi , Teruaki Masuda , Hiroyuki Matsuta , Kenichi Yabuuchi , Nobuhiro Hata , Minoru Fujiki , Konen Obayashi , Etsuro Matsubara , Noriyuki Kimura

Ictal cold shiver is a rare manifestation of seizures that originate in the temporal lobes; it is characterized by body trembling and sensations of cold. This study aims to investigate the clinical manifestations and etiological factors in patients with ictal cold shiver. We reviewed 59 cases of ictal cold shiver by using various keywords to search published original articles and case reports, and we described a new case of autoimmune limbic encephalitis. The median age at diagnosis was higher in patients with autoimmune encephalitis than in those with other etiologies (p = 0.007). Autoimmune encephalitis showed more frequent bilateral temporal lobe abnormalities on magnetic resonance imaging (MRI) (p = 0.027) and generalized or bilateral independent epileptiform discharges on electroencephalography (EEG) (p = 0.047). Anti-leucine-rich glioma-inactivated 1 antibodies were most commonly detected in patients with autoimmune encephalitis with ictal cold shiver (85.7 %). This study highlights the importance of considering autoimmune mechanisms in the presence of ictal cold shiver, especially in patients with bilateral temporal lobe lesions on MRI, generalized or bilateral independent epileptiform discharges on EEG, and older onset.

突发性寒战是一种罕见的癫痫发作的表现，起源于颞叶；它的特点是身体颤抖和感觉寒冷。本研究旨在探讨危重性寒颤患者的临床表现及病因。本文采用多种关键词检索已发表的原创文章和病例报告，对59例危重性寒颤病例进行回顾性分析，报道1例新发自身免疫性边缘脑炎病例。自身免疫性脑炎患者的诊断年龄中位数高于其他病因的患者（p = 0.007）。自身免疫性脑炎在磁共振成像（MRI）上显示更频繁的双侧颞叶异常（p = 0.027），脑电图（EEG）上显示全面性或双侧独立癫痫样放电（p = 0.047）。抗富亮氨酸胶质瘤灭活1抗体最常见于自身免疫性脑炎伴急性寒颤患者（85.7%）。这项研究强调了考虑自身免疫机制的重要性，尤其是在MRI显示双侧颞叶病变、脑电图显示全身性或双侧独立癫痫样放电以及发病年龄较大的患者中。

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引用次数: 0

Responsive neurostimulation for patients with refractory mesial temporal lobe epilepsy: A systematic review and meta-analysis 反应性神经刺激治疗难治性内侧颞叶癫痫：一项系统回顾和荟萃分析

IF 1.8 Q3 CLINICAL NEUROLOGY

Epilepsy and Behavior Reports

Pub Date : 2025-04-22 DOI: 10.1016/j.ebr.2025.100774

Eshita Sharma , Beatriz Westphalen Pomianoski , Rabbia Jabbar , Ayesha Ayesha , Yasmin Picanco Silva , Paweł Łajczak , Aisha Rizwan Ahmed , Oguz Kagan Sahin , Mir Wajid Majeed , Mohammed Raake , Walter Fagundes , Giovani Noll

Responsive neurostimulation (RNS) is a well-established adjuvant therapy for reducing seizure frequency in adults with medically refractory partial-onset seizures, particularly in individuals who are either not candidates for surgical resection or remain seizure-prone post-surgery. However, its effectiveness in patients with mesial temporal lobe epilepsy (MTLE) remains unclear. This meta-analysis evaluates the efficacy of RNS therapy in individuals with medically refractory MTLE. A systematic search of PubMed, Cochrane, and Scopus databases was conducted to identify eligible studies. Outcomes assessed included mean seizure frequency reduction, responder rate (proportion of patients achieving ≥50 % reduction in seizure frequency), and the proportion of patients achieving seizure freedom within six months of follow-up. Statistical analyses were performed using STATA. Seven observational studies involving 207 patients were included. RNS was associated with a mean seizure frequency reduction of 68.76 % (95 % CI 57.16–80.37 %; I² = 81.68 %), a responder rate of 67.58 % (95 % CI 46.51–88.66 %; I² = 94 %), and seizure freedom within six months in 28.94 % of patients (95 % CI 3.03–54.86 %; I² = 88 %). Moderate to high heterogeneity was observed across the studies. RNS may represent a viable therapeutic option for patients with MTLE, demonstrating substantial reductions in seizure frequency and a notable proportion of patients achieving seizure freedom. Additional studies are needed to confirm these findings and to explore the comparative efficacy and safety of RNS therapy in relation to other treatment options.

反应性神经刺激（RNS）是一种公认的辅助治疗方法，用于减少医学上难治性部分发作性癫痫的成人发作频率，特别是对于不适合手术切除或术后仍易发作的个体。然而，其对内侧颞叶癫痫（MTLE）患者的疗效尚不清楚。本荟萃分析评估了RNS治疗难治性MTLE患者的疗效。对PubMed、Cochrane和Scopus数据库进行系统检索，以确定符合条件的研究。评估的结果包括平均癫痫发作频率降低、应答率（癫痫发作频率降低≥50%的患者比例）和在随访6个月内实现癫痫发作自由的患者比例。采用STATA进行统计分析。纳入了7项观察性研究，涉及207例患者。RNS与平均癫痫发作频率降低68.76%相关(95% CI 57.16 - 80.37%；I2 = 81.68%)，应答率为67.58% (95% CI 46.51 ~ 88.66%；I2 = 94%)， 6个月内癫痫发作自由率为28.94% (95% CI 3.03 - 54.86%；i2 = 88%)。在所有研究中观察到中度至高度的异质性。RNS可能是MTLE患者的一种可行的治疗选择，显示癫痫发作频率大幅降低，并且显著比例的患者实现了癫痫发作自由。需要进一步的研究来证实这些发现，并探索与其他治疗方案相比，RNS治疗的疗效和安全性。

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Cerebrotendinous Xanthomatosis: Novel EEG finding of Fixation-Off Sensitivity 脑腱黄瘤病：新的脑电图发现的固定-关闭敏感性

IF 1.8 Q3 CLINICAL NEUROLOGY

Epilepsy and Behavior Reports

Pub Date : 2025-04-18 DOI: 10.1016/j.ebr.2025.100770

Zachary Mills, James Thomas Houston, Ashley Thomas, Kelsey Shoenmeyer

Cerebrotendinous Xanthomatosis (CTX) is a rare autosomal recessive condition resulting in accumulation of cholesterol and cholestanol due to disrupted bile synthesis. Affected tissues include brain, tendons, skin, bone, lungs, and eyes. We report a clinical case presenting with epilepsy, which has been described, however with a particular EEG appearance that appears novel with Fixation-Off Sensitivity (FOS). The patient’s EEG showed significant buildup of abnormal slowing and frontally predominant generalized epileptiform discharges when her eyes were closed, and in contrast essentially normal tracings while eyes were open, eventually showing electrographic evolution and generating a bilateral tonic-clonic seizure. Genetic testing confirmed the diagnosis of CTX, and CTX-specific treatment with chonodeoxycholic acid was initiated in addition to anti-seizure medication.

脑腱黄瘤病（CTX）是一种罕见的常染色体隐性遗传病，由于胆汁合成中断导致胆固醇和胆固醇积累。受影响的组织包括大脑、肌腱、皮肤、骨骼、肺和眼睛。我们报告一个临床病例呈现癫痫，这已经被描述，但有一个特殊的脑电图的外观，似乎新颖的注视-关闭敏感性（FOS）。患者的脑电图显示闭眼时异常缓慢和额侧占优势的全面性癫痫样放电明显增加，而睁眼时基本正常，最终显示电图演变并产生双侧强直-阵挛性发作。基因检测证实了CTX的诊断，并在抗癫痫药物治疗的基础上，开始了针对CTX的软骨去氧胆酸治疗。

引用次数: 0