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Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) with high-frequency oscillations on scalp EEG: A case report 线粒体肌病、脑病、乳酸性酸中毒和脑卒中样发作(MELAS)伴头皮脑电图高频振荡1例报告
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-13 DOI: 10.1016/j.ebr.2025.100754
Keisuke Maeda , Himari Tsuboi , Nami Hosoda , Junichi Fukumoto , Shiho Fujita , Naohiro Ichino , Keisuke Osakabe , Keiko Sugimoto , Gen Furukawa , Naoko Ishihara
Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a rare mitochondrial disease with a prevalence of 16–18 per 100,000 persons. Most patients with MELAS develop epilepsy and require effective control of recurrent attacks. High-frequency oscillations (HFOs) on scalp EEG are transient bursts of EEG activity with frequencies beyond 80 Hz and are a promising biomarker for seizure control in epilepsy. However, reports on scalp HFOs are limited to some epilepsy syndromes. Herein, we report on the appearance of scalp HFOs in a pediatric patient with MELAS. The patient, a 13-year-old boy, presented with a stroke-like episode at age 9 years and was diagnosed with MELAS. The main symptom was visual disturbances, but epilepsia partialis continua (EPC) was also observed. Scalp EEG recordings were made six times: 8 days before the stroke-like episode (day –8 EEG), the day of appearance (day 1 EEG), and 4, 8, 10, and 100 days after the episode. Analysis of scalp HFOs showed that no scalp HFOs were detected in the day –8 EEG, whereas 1.20 scalp HFOs per minute were detected in the day 1 EEG at the appearance of the stroke-like episode with EPC. The scalp HFO detection rate decreased with the loss of EPC, and no scalp HFOs appeared on EEG, although visual disturbances continued to be observed. By contrast, epileptic discharges remained on EEG after EPC disappearance. Scalp HFOs have the potential to be a useful biomarker for reflecting epileptic seizure in patients with MELAS.
线粒体肌病、脑病、乳酸酸中毒和卒中样发作(MELAS)是一种罕见的线粒体疾病,发病率为每10万人中16-18人。大多数MELAS患者发生癫痫,需要有效控制反复发作。头皮脑电图高频振荡(HFOs)是一种频率超过80hz的短暂脑电图活动爆发,是一种很有前景的癫痫发作控制生物标志物。然而,关于头皮氟氯烃的报道仅限于某些癫痫综合征。在此,我们报告了一名MELAS患儿头皮hfo的出现。患者为一名13岁的男孩,在9岁时出现卒中样发作,并被诊断为MELAS。主要症状为视觉障碍,但也有部分持续性癫痫(EPC)。头皮EEG记录6次:卒中样发作前8天(第8天EEG),出现当天(第1天EEG),发作后4、8、10和100天。头皮hfo分析显示,在第8天的脑电图中未检测到头皮hfo,而在第1天脑电图中,在出现卒中样发作时,每分钟检测到1.20个头皮hfo。头皮HFO检出率随EPC的丧失而下降,脑电图上未出现头皮HFO,但仍观察到视觉障碍。而EPC消失后脑电图上仍有癫痫放电。头皮hfo有可能成为反映MELAS患者癫痫发作的有用生物标志物。
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引用次数: 0
Frequently asked questions and answers on Visually-Provoked (Photosensitive) epilepsy 关于视觉诱发性(光敏性)癫痫的常见问题和答案
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-10 DOI: 10.1016/j.ebr.2025.100753
Dorothée Kasteleijn-Nolst Trenité , Jayant Acharya , Fiona Mitchell Baumer , Roy Beran , Dana Craiu , Jaqueline French , Pasquale Parisi , Jessica Solodar , Jerzy P. Szaflarski , Yukitoshi Takahashi , Liu Lin Thio , Ben Tolchin , Arnold Wilkins , Robert S. Fisher
Clinical experts associated with national epilepsy-related societies, led by the Epilepsy Foundation, collected, collated and answered “Frequently asked questions (FAQ)” of broad interest pertaining to visually-provoked seizures. Questions emerged from people with epilepsy, caretakers and healthcare professionals from different countries around the world. Focus is on practical implications of visually-provoked seizures. The top 5 most frequently asked questions were.
  • 1.
    How does a doctor make a diagnosis of visually-provoked seizures?
  • 2.
    What can I do in general to prevent visually-provoked seizures?
  • 3.
    Will I need antiseizure medications for my visually-provoked seizures?”
  • 4.
    Will I outgrow visually-provoked seizures? How will I know if I’ve outgrown them?
  • 5.
    How do I enable safety features to block content that could trigger seizures on social media, websites, phones, laptops and tablets?
Answers were based on scientific evidence, where such information was available [1] and expert opinion when formal evidence was insufficient. Key answers included distinction of photoparoxysmal EEG findings versus light-provoked seizures. Typical provocation is by flashes at 10–25 per second or certain moving patterns. There is a genetic risk, which is outgrown in about half. Covering one or both eyes can prevent a light-provoked seizure. TV, videogames, virtual reality and 3D images are not in themselves provocative, but their content can be.
Topics covered included: 1. Photosensitive epilepsy diagnosis; 2. Preventing visually-provoked seizures; 3. Do treatments help; 4. Life and behavioral decisions; 5. School; 6. Multi-media; 7. Children and youth.
在癫痫基金会的领导下,与国家癫痫相关协会相关的临床专家收集、整理并回答了与视觉诱发性癫痫有关的广泛关注的“常见问题”(FAQ)。来自世界各地不同国家的癫痫患者、护理人员和卫生保健专业人员提出了问题。重点是视觉诱发性癫痫发作的实际意义。最常被问到的前5个问题是:医生如何诊断视觉诱发性癫痫?一般来说,我能做些什么来预防视觉诱发性癫痫发作?我的视觉诱发性癫痫发作需要抗癫痫药物吗?”我能摆脱视觉诱发性癫痫发作吗?我怎么知道我是否已经长大了?如何启用安全功能来阻止社交媒体、网站、手机、笔记本电脑和平板电脑上可能引发癫痫发作的内容?答案以科学证据为基础,在有科学信息的情况下,在正式证据不足的情况下,以专家意见为基础。关键答案包括光性发作性脑电图结果与光诱发性癫痫发作的区别。典型的挑衅是通过每秒10-25次的闪光或特定的移动模式。有遗传风险,大约一半的人会长大。遮住一只或两只眼睛可以防止光线引起的癫痫发作。电视、电子游戏、虚拟现实和3D图像本身并不具有煽动性,但它们的内容可能具有煽动性。涵盖的主题包括:1。光敏性癫痫诊断;2. 预防视觉诱发性癫痫发作;3. 治疗有帮助吗?4. 生活和行为决定;5. 学校;6. 多媒体;7. 儿童和青年。
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引用次数: 0
Encephalocele-associated temporal lobe refractory epilepsy: Report of two cases 脑膨出性颞叶难治性癫痫2例报告
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-08 DOI: 10.1016/j.ebr.2025.100752
María Zuluaga , Valeria Valencia-Cifuentes , María Alejandra Recio , Juan Camilo Márquez , Juan P. Fernández-Cubillos , Yuri Takeuchi
Encephaloceles are abnormal protrusions of brain tissue, meninges, and cerebrospinal fluid that result from defects in the skull base or cranial vault. These abnormalities can lead to seizure disorders and focal pharmacoresistant epilepsies. However, clinical suspicion and diagnosis are frequently delayed due to the significant challenges in interpreting initial imaging. Interpretation omissions can contribute to false diagnosis as non-lesional epilepsy in patients with encephalocele. This warrants appropriate imaging paradigms and careful interpretation. When clinical suspicion remains, further surgical exploration should be considered. Different treatment approaches, such as lesionectomy, temporal lobectomy, or invasive studies such as stereoelectroencephalography, may be employed. We describe two cases of adult-onset epilepsy associated with encephaloceles: a 28-year-old woman with drug-resistant epilepsy secondary to a right temporal encephalocele that was not initially diagnosed through neuroimaging, and a 43-year-old woman who presented with de novo focal status epilepticus attributed to a left temporal encephalocele, diagnosed during surgical exploration for a misdiagnosed neoplasm. Both cases were successfully treated surgically, with one-year follow-up free of new seizures.
脑膨出是由颅底或颅顶缺陷引起的脑组织、脑膜和脑脊液的异常突出。这些异常可导致癫痫发作障碍和局灶性耐药癫痫。然而,临床怀疑和诊断经常被延迟,因为在解释最初的图像的重大挑战。解释上的遗漏可能导致脑膨出患者被误诊为非病变性癫痫。这需要适当的成像范式和仔细的解释。当临床怀疑仍然存在时,应考虑进一步手术探查。可以采用不同的治疗方法,如病灶切除术、颞叶切除术或立体脑电图等侵入性研究。我们描述了两例与脑膨出相关的成人癫痫病例:一名28岁的女性继发于右侧颞叶脑膨出的耐药癫痫,最初未通过神经影像学诊断;另一名43岁的女性因左侧颞叶脑膨出而出现局灶性癫痫持续状态,在手术探查时诊断为误诊的肿瘤。两例均成功手术治疗,随访一年无新发作。
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引用次数: 0
Gelastic spells in Angelman Syndrome, when laughter isn’t funny 天使综合症的弹性法术,当笑声不有趣时
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-08 DOI: 10.1016/j.ebr.2025.100751
Natasha Varughese , Femke Horn , Robert P. Carson
Angelman syndrome (AS) is a neurodevelopmental syndrome characterized by people with a characteristic happy demeanor, impaired expressive communication, sleep disruptions, and a variety of paroxysmal events, including seizures and movement disorders. Herein, we report three cases, one child and two adults with AS, of gelastic (laugh-related) spells. The phenomenology in all three is most consistent with gelastic syncope. A fourth example demonstrating transient breath-holding while laughing is included for reference. We subsequently review the differential for gelastic spells in AS, including seizures and cataplexy, and expound on the importance of their identification in the context of risk factors for cardiac arrhythmia. This work adds gelastic syncope to the list of paroxysmal events in AS and may serve to inform providers and parents of this phenotype and provide guidance for subsequent evaluation and treatment if indicated.
天使人综合症(AS)是一种神经发育综合症,其特征是人们具有典型的快乐举止,表达性交流受损,睡眠中断以及各种发作性事件,包括癫痫发作和运动障碍。在此,我们报告三例,一名儿童和两名成人AS,有弹性(笑相关)法术。这三种症状的现象学与弹性晕厥最为一致。第四个例子演示了笑时短暂屏气,以供参考。我们随后回顾了AS中弹性发作的区别,包括癫痫发作和猝厥,并阐述了在心律失常危险因素背景下识别它们的重要性。这项工作将弹性晕厥添加到AS发作事件列表中,可能有助于告知提供者和家长这种表型,并为后续评估和治疗提供指导。
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引用次数: 0
Ultrahigh-field imaging (7 Tesla) in DNET: Unmasking microstructural imaging characteristics – A case report DNET中的超高场成像(7特斯拉):揭示微结构成像特征- 1例报告
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-05 DOI: 10.1016/j.ebr.2025.100749
Marta Calvo-Imirizaldu , Daniele Botta , Margitta Seeck , Jan Novy , Jose Federico Ojeda Esparza , Aikaterini Fitsiori , Corrado Santarosa , Kevin Battistini , Karl-Olof Lövblad , Felix T. Kurz
Commercial ultrahigh-field 7 Tesla (T) MRI has been approved for clinical brain imaging, including applications in epilepsy and brain tumors. Increasing magnetic field strength offers significant advantages over lower-field MRI due to improved spatial resolution, signal-to-noise ratio, and contrast-to-noise ratio. These improvements provide better anatomical delineation and gray-white matter tissue-contrast differentiation.
We present a case of a presumed dysembryoplastic neuroepithelial tumor (DNET) imaged at 7 T MRI of the second generation, which revealed an unprecedented level of detail of the complex and intricate tumor architecture. Insights of its different components correlate closely with its known histopathological features. These tumors are unique among low-grade neoplasms due to their distinct clinical presentation, imaging features, and histopathological architecture. DNETs are rare, typically occurring in young patients with refractory epilepsy, and are classified by their well-defined histological subtypes. We review the various MRI patterns of DNET, which have been shown to correlate with histological subtypes and the extent of the epileptogenic zone.
Complete tumor resection is essential for long-term control and recurrence prevention, emphasizing the importance of precise preoperative visualization of the tumor and its surrounding tissue. In this case, 7 T images demonstrated superior lesion conspicuity and clearer boundaries, highlighting the advantages of ultrahigh-field MRI in defining the full extent of the lesion. Although 7 T MRI is not yet widely available, it has started to gain an important role in the management of epilepsy, particularly for cases requiring detailed structural analysis.
商用超高场7特斯拉(T) MRI已被批准用于临床脑成像,包括癫痫和脑肿瘤的应用。由于提高了空间分辨率、信噪比和对比噪声比,增加磁场强度比低场MRI具有显著的优势。这些改进提供了更好的解剖描绘和灰质-白质组织对比分化。我们报告了一个假定的胚胎发育异常神经上皮肿瘤(DNET)的病例,在第二代7t MRI成像中,揭示了复杂和复杂的肿瘤结构的前所未有的细节水平。对其不同成分的了解与其已知的组织病理学特征密切相关。由于其独特的临床表现、影像学特征和组织病理学结构,这些肿瘤在低级别肿瘤中是独一无二的。DNETs很少见,通常发生在难治性癫痫的年轻患者中,并根据其明确的组织学亚型进行分类。我们回顾了DNET的各种MRI模式,这些模式已被证明与组织学亚型和癫痫区范围相关。完全切除肿瘤对于长期控制和预防复发至关重要,强调术前精确观察肿瘤及其周围组织的重要性。在本例中,7t图像显示病变明显,边界更清晰,突出了超高场MRI在确定病变全部范围方面的优势。尽管7 T MRI尚未广泛使用,但它已开始在癫痫治疗中发挥重要作用,特别是在需要详细结构分析的病例中。
{"title":"Ultrahigh-field imaging (7 Tesla) in DNET: Unmasking microstructural imaging characteristics – A case report","authors":"Marta Calvo-Imirizaldu ,&nbsp;Daniele Botta ,&nbsp;Margitta Seeck ,&nbsp;Jan Novy ,&nbsp;Jose Federico Ojeda Esparza ,&nbsp;Aikaterini Fitsiori ,&nbsp;Corrado Santarosa ,&nbsp;Kevin Battistini ,&nbsp;Karl-Olof Lövblad ,&nbsp;Felix T. Kurz","doi":"10.1016/j.ebr.2025.100749","DOIUrl":"10.1016/j.ebr.2025.100749","url":null,"abstract":"<div><div>Commercial ultrahigh-field 7 Tesla (T) MRI has been approved for clinical brain imaging, including applications in epilepsy and brain tumors. Increasing magnetic field strength offers significant advantages over lower-field MRI due to improved spatial resolution, signal-to-noise ratio, and contrast-to-noise ratio. These improvements provide better anatomical delineation and gray-white matter tissue-contrast differentiation.</div><div>We present a case of a presumed dysembryoplastic neuroepithelial tumor (DNET) imaged at 7 T MRI of the second generation, which revealed an unprecedented level of detail of the complex and intricate tumor architecture. Insights of its different components correlate closely with its known histopathological features. These tumors are unique among low-grade neoplasms due to their distinct clinical presentation, imaging features, and histopathological architecture. DNETs are rare, typically occurring in young patients with refractory epilepsy, and are classified by their well-defined histological subtypes. We review the various MRI patterns of DNET, which have been shown to correlate with histological subtypes and the extent of the epileptogenic zone.</div><div>Complete tumor resection is essential for long-term control and recurrence prevention, emphasizing the importance of precise preoperative visualization of the tumor and its surrounding tissue. In this case, 7 T images demonstrated superior lesion conspicuity and clearer boundaries, highlighting the advantages of ultrahigh-field MRI in defining the full extent of the lesion. Although 7 T MRI is not yet widely available, it has started to gain an important role in the management of epilepsy, particularly for cases requiring detailed structural analysis.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100749"},"PeriodicalIF":1.8,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143377715","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Myoclonic status epilepticus with dystonia-like symptoms in patients with dementia: Report of two cases 痴呆患者伴张力障碍样症状的肌阵挛性癫痫持续状态:两例报告
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-04 DOI: 10.1016/j.ebr.2025.100750
Rosario V. Rossi, Rosanna Melis, Noemi Murdeu, Sara Lizzos, Maria Luigia Piras, Loretta Racis, Silvia Serusi, Maria Valeria Saddi
We report cases of two elderly women with dementia who presented with a new-onset seizure disorder characterized by subtle, rhythmic muscular contractions involving the buccolingual region and the four limbs, persistent jaw opening, and abnormal cervical posture that mimicked myoclonus-dystonia syndrome and oromandibular dystonia. The symptoms lasted several minutes to a few hours. Video-polygraphic recordings revealed an electromyographic (EMG) pattern of brief, shock-like muscular contractions consistent with myoclonus that correlated with a high-amplitude (70–90 µV), 11–14 Hertz, bilaterally symmetric electroencephalographic (EEG) rhythm over the frontocentral regions. A time-locked relationship between the frontocentral EEG activity and the EMG myoclonic potentials demonstrated the cortical origin of myoclonus and therefore the epileptic nature of the disorder, whereas the oromandibular and cervical dystonic-like postures suggested the pathogenic involvement of subcortical structures. The intravenous administration of diazepam suppressed the clinical symptoms and the EEG–EMG correlate of myoclonus. The clinical and neurophysiological findings illustrate a form of myoclonic status epilepticus (SE) with dystonia-like symptoms resulting from the functional involvement of cortical and subcortical structures. The manifestation of subtle, rhythmic myoclonus and dystonic-like postures in patients with atypical EEG patterns of SE may require challenging differential diagnoses with myoclonus-dystonia syndrome and oromandibular dystonia.
我们报告了两例老年痴呆妇女,她们表现为新发作的癫痫发作障碍,其特征是微妙的,有节奏的肌肉收缩,涉及颊舌区和四肢,持续的下颌张开,以及异常的颈部姿势,模仿肌颤-肌张力障碍综合征和口腔下颌肌张力障碍。症状持续几分钟到几个小时。视频polygraphic记录显示肌电图(EMG)显示短暂的,与肌阵挛一致的休克样肌肉收缩模式,并与额中央区域的高振幅(70-90µV), 11-14赫兹,双侧对称脑电图(EEG)节律相关。额中央脑电图活动和肌电图肌阵挛电位之间的时间锁定关系表明了肌阵挛的皮层起源,因此表明了这种疾病的癫痫性质,而口下颌和颈部肌肉张力样姿势表明了皮层下结构的致病参与。静脉给药地西泮可抑制肌阵挛的临床症状和脑电图肌电图相关性。临床和神经生理学的发现说明了一种形式的肌阵挛性癫痫持续状态(SE)与肌张力障碍样症状,由皮层和皮层下结构的功能受累引起。不典型脑电图的SE患者表现为细微的、节律性肌阵挛和肌张力样姿势,可能需要与肌阵挛-肌张力障碍综合征和口下颌肌张力障碍鉴别诊断。
{"title":"Myoclonic status epilepticus with dystonia-like symptoms in patients with dementia: Report of two cases","authors":"Rosario V. Rossi,&nbsp;Rosanna Melis,&nbsp;Noemi Murdeu,&nbsp;Sara Lizzos,&nbsp;Maria Luigia Piras,&nbsp;Loretta Racis,&nbsp;Silvia Serusi,&nbsp;Maria Valeria Saddi","doi":"10.1016/j.ebr.2025.100750","DOIUrl":"10.1016/j.ebr.2025.100750","url":null,"abstract":"<div><div>We report cases of two elderly women with dementia who presented with a new-onset seizure disorder characterized by subtle, rhythmic muscular contractions involving the buccolingual region and the four limbs, persistent jaw opening, and abnormal cervical posture that mimicked myoclonus-dystonia syndrome and oromandibular dystonia. The symptoms lasted several minutes to a few hours. Video-polygraphic recordings revealed an electromyographic (EMG) pattern of brief, shock-like muscular contractions consistent with myoclonus that correlated with a high-amplitude (70–90 µV), 11–14 Hertz, bilaterally symmetric electroencephalographic (EEG) rhythm over the frontocentral regions. A time-locked relationship between the frontocentral EEG activity and the EMG myoclonic potentials demonstrated the cortical origin of myoclonus and therefore the epileptic nature of the disorder, whereas the oromandibular and cervical dystonic-like postures suggested the pathogenic involvement of subcortical structures. The intravenous administration of diazepam suppressed the clinical symptoms and the EEG–EMG correlate of myoclonus. The clinical and neurophysiological findings illustrate a form of myoclonic status epilepticus (SE) with dystonia-like symptoms resulting from the functional involvement of cortical and subcortical structures. The manifestation of subtle, rhythmic myoclonus and dystonic-like postures in patients with atypical EEG patterns of SE may require challenging differential diagnoses with myoclonus-dystonia syndrome and oromandibular dystonia.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100750"},"PeriodicalIF":1.8,"publicationDate":"2025-02-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143377713","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
First experiences with multiple bilateral insertions of a newly developed microcatheter-compatible endovascular electroencephalogram electrode for humans with epilepsy 首次为癫痫患者多次双侧插入新开发的微导管兼容血管内脑电图电极
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-04 DOI: 10.1016/j.ebr.2025.100748
Yosuke Masuda , Ayataka Fujimoto , Hisayuki Hosoo , Kota Araki , Hiroki Ishida , Mitsuyo Nishimura , Aiki Marushima , Eiichi Ishikawa , Yuji Matsumaru
Identifying epileptogenic zones non-invasively is challenging due to signal interference by the scalp and skull, necessitating invasive methods like subdural recordings and stereoelectroencephalography. Recent microcatheter advancements suggest that a microcatheter-compatible endovascular EEG (eEEG) device could overcome these barriers. We developed a thin, flexible eEEG electrode, the EP-01, for use with current microcatheters. The EP-01, comprising a platinum electrode and alloy wire coated with an electrically non-conductive polymer, was inserted via the jugular veins under local anesthesia. The EP-01 electrodes were planned to be placed in six locations: bilateral transverse sinuses, bilateral cavernous sinuses, and the anterior and posterior superior sagittal sinuses. We conducted a first-in-human study demonstrating the feasibility and efficacy of the EP-01electrodes in simultaneously recording intracranial EEG signals from multiple brain locations. The EP-01 electrodes were successfully placed as planned, except for one, without complications. Simultaneous eEEG and scalp EEG recordings were performed during a Wada test to evaluate efficacy and safety. The eEEG recorded alpha waves and slow-wave activity during propofol administration, corresponding to scalp EEG findings, with amplitudes 3–4 times higher. Post-procedural assessments confirmed cranial vessels’ patency and absence of complications. The EP-01 successfully recorded EEG signals at multiple locations in the human brain using an endovascular approach. Compared to scalp EEG, the present approach seems to have the potential to record higher-amplitude EEG. However, the study was limited to short-term recordings without epileptic discharges. Further investigations, including long-term placement, are thus needed for seizure recordings and safety evaluations.
由于头皮和颅骨的信号干扰,无创识别癫痫区具有挑战性,因此需要硬脑膜下记录和立体脑电图等侵入性方法。最近的微导管进展表明,一种与微导管兼容的血管内脑电图(eEEG)装置可以克服这些障碍。我们开发了一种薄的、柔性的脑电图电极EP-01,用于当前的微导管。EP-01由铂电极和涂有导电聚合物的合金丝组成,在局部麻醉下通过颈静脉插入。EP-01电极计划放置在6个位置:双侧横窦、双侧海绵窦、前后上矢状窦。我们进行了首次人体研究,证明了ep -01电极同时记录来自多个大脑位置的颅内脑电图信号的可行性和有效性。EP-01电极除1个外均按计划顺利放置,无并发症。在Wada测试期间同时进行脑电图和头皮脑电图记录以评估疗效和安全性。脑电图记录了异丙酚给药期间的α波和慢波活动,与头皮脑电图结果相对应,振幅高3-4倍。术后评估证实颅内血管通畅,无并发症。EP-01使用血管内方法成功记录了人脑多个位置的脑电图信号。与头皮脑电图相比,本方法似乎具有记录高振幅脑电图的潜力。然而,这项研究仅限于没有癫痫放电的短期记录。因此,需要进一步调查,包括长期安置,以进行扣押记录和安全评估。
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引用次数: 0
Painful Todd’s: Post-ictal painful hemiparesis as an identifier of insular epilepsy 痛症托德氏症:癫痫发作后痛症偏瘫作为岛状癫痫的标识
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-02 DOI: 10.1016/j.ebr.2025.100747
Julian Larkin , Tudor Munteanu , Emma Dolan , Daniel J. Costello , Kieron Sweeney , Ronan Kilbride , Peter Widdess-Walsh
The insula can generate seizures which mimic frontal, temporal and parietal epilepsies making electroclinical localization difficult. We report the case of a twenty-one-year-old woman who presented with seizure semiology of a left-sided painful somatosensory aura, progressing to bilateral tonic posturing and complex manual automatisms. She described a painful sensation and weakness affecting her left side following the offset of a seizure, with the pain consistenly outlasting the weakness. This would last from hours to days depending on the severity and duration of the seizure. Stereo-electroencephalography (SEEG) demonstrated seizure onset in the limen of the right insula. Extra-operative stimulation of the insula reproduced the clinical symptoms. She underwent radiofrequency thermocoagulation (RFTC) which has resulted in a significant reduction in seizure frequency. This case report describes a lateralized painful Todd’s phenomenon as a feature of insular epilepsy confirmed by SEEG and extra-operative stimulation.
脑岛可产生类似额叶、颞叶和顶叶癫痫的癫痫发作,使电临床定位困难。我们报告一个21岁的妇女谁提出了癫痫符码左侧疼痛的躯体感觉先兆,进展到双侧强直姿势和复杂的手动自动性。她描述了癫痫发作后左侧疼痛和虚弱的感觉,疼痛持续时间比虚弱持续时间长。根据癫痫发作的严重程度和持续时间,这将持续数小时至数天。立体脑电图(SEEG)显示癫痫发作在右脑岛的边缘。手术外对脑岛的刺激可重现临床症状。她接受了射频热凝治疗(RFTC),结果癫痫发作频率显著降低。本病例报告描述了一个侧面疼痛的托德现象作为岛状癫痫的特征,经SEEG和手术外刺激证实。
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引用次数: 0
Antiseizure medications and their differing effects on cardiovascular risk 抗癫痫药物及其对心血管风险的不同影响
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-02-01 DOI: 10.1016/j.ebr.2025.100746
Aleena Abbasi , Bassil Abbasi , Scott Mintzer , Carla LoPinto-Khoury
This review discusses the differing effects of enzyme-inducing and non-inducing antiseizure medications on cardiovascular risk and their implications for the management strategies of epilepsy patients. Traditional risk markers, including low density lipoprotein, high density lipoprotein and triglycerides, can be altered by both enzyme induction and inhibition. Other markers of vascular risk, including c-reactive protein, non-high-density lipoprotein and homocysteine, are affected by antiseizure medications, although adults and children may have different responses. The overall atherosclerotic risk picture is more complex due to indirect effects such as neuroendocrine function and the metabolic syndrome. Large scale data shows an evolving understanding of cardiovascular risk. Long term risks of enzyme inducing antiseizure medications and valproic acid are apparent when studies examine medications individually. Finally, effects of antiseizure medications on cardiac rhythm and possibly autonomic control are discussed with respect to their clinical relevance to the practicing clinician.
这篇综述讨论了酶诱导和非诱导抗癫痫药物对心血管风险的不同影响及其对癫痫患者管理策略的影响。传统的风险标志物,包括低密度脂蛋白、高密度脂蛋白和甘油三酯,可以通过酶的诱导和抑制来改变。其他血管危险指标,包括c反应蛋白、非高密度脂蛋白和同型半胱氨酸,都受到抗癫痫药物的影响,尽管成人和儿童可能有不同的反应。由于神经内分泌功能和代谢综合征等间接影响,总的动脉粥样硬化风险情况更为复杂。大量数据显示,人们对心血管疾病风险的认识在不断发展。当研究单独检查药物时,酶诱导抗癫痫药物和丙戊酸的长期风险是明显的。最后,本文讨论了抗癫痫药物对心律和可能的自主神经控制的影响,以及它们与临床医生的临床相关性。
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引用次数: 0
Usefulness of perampanel as initial monotherapy in children with non-lesional focal epilepsy perampanel作为非病变局灶性癫痫患儿初始单药治疗的有效性
IF 1.8 Q3 CLINICAL NEUROLOGY Pub Date : 2025-01-30 DOI: 10.1016/j.ebr.2025.100743
Hideaki Kanemura , Yoshihiro Miyasato , Yutaro Tomi , Fumikazu Sano
This study aimed to compare carbamazepine (CBZ) and perampanel (PER) in terms of the efficacy against clinical seizures and electroencephalographic abnormalities such as interictal epileptiform discharges (IEDs) and safety as initial monotherapy in children with non-lesional focal epilepsies. This retrospective review included participants recruited from among epilepsy outpatients treated at the authors’ hospital between January 01, 2000, and December 31, 2019 in the CBZ group and between January 01, 2020, and December 31, 2022 in the PER group. The inclusion criterion in both groups was ≥12 months of follow-up. Responders were identified as participants demonstrating complete disappearance (100 % reduction in seizures or IEDs) or response (>50 % reduction in seizure frequency or IEDs). Safety primary outcome was defined as appearance rate of AEs. The study group comprised 247 participants treated with CBZ and 46 participants treated with PER. Total efficacy rate for clinical seizures was significantly higher with PER than with CBZ (p = 0.0148). Moreover, the rate of complete disappearance was significantly higher with PER than with CBZ (p = 0.0133). Total efficacy rate for IED was again significantly higher with PER than with CBZ (p < 0.0001). The appearance of adverse events was significantly lower with PER than with CBZ (p = 0.023). PER may be useful as initial monotherapy in children with non-lesional focal epilepsies.
本研究旨在比较卡马西平(CBZ)和perampanel (PER)作为非病变局灶性癫痫患儿初始单药治疗对临床癫痫发作和脑电图异常(如间期癫痫样放电(IEDs))的疗效和安全性。本回顾性研究纳入了2000年1月1日至2019年12月31日期间在作者所在医院接受治疗的CBZ组和2020年1月1日至2022年12月31日期间在PER组接受治疗的癫痫门诊患者。两组纳入标准均为随访≥12个月。应答者被确定为表现完全消失(癫痫发作或简易爆炸装置减少100%)或反应(癫痫发作频率或简易爆炸装置减少50%)的参与者。安全性主要终点定义为不良事件的出现率。研究组包括247名接受CBZ治疗的参与者和46名接受PER治疗的参与者。PER组临床癫痫发作总有效率显著高于CBZ组(p = 0.0148)。此外,PER组的完全消失率显著高于CBZ组(p = 0.0133)。PER组治疗IED的总有效率再次显著高于CBZ组(p <;0.0001)。PER组不良事件发生率明显低于CBZ组(p = 0.023)。PER可作为非病变局灶性癫痫患儿的初始单药治疗。
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Epilepsy and Behavior Reports
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