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Isolated Unilateral EEG Findings in Juvenile Myoclonic Epilepsy: A Case Report. 青少年肌阵挛性癫痫孤立单侧脑电图1例报告。
Pub Date : 2022-12-01 DOI: 10.14581/jer.22014
Merve Aktan Suzgun, Veysi Demirbilek

Juvenile myoclonic epilepsy (JME) has well-defined clinical and electrophysiological features. On the other hand, large case series have shown that focal and asymmetrical discharges may accompany generalized epileptiform activities in JME. Although it is known that these non-generalized electrophysiological findings do not exclude the diagnosis of this syndrome, some findings may create confusion in the differential diagnosis. In this case report, a case of JME with electroencephalographic findings characterized by isolated unilateral epileptiform activities without typical generalized discharges was discussed. The current case clinically presented with involuntary jerk movements in the bilateral upper extremities. It has been determined that these movements are uni/bilateral myoclonic beats based on home video recordings. Metabolic, toxic and structural problems were excluded in the investigations for the etiology of myoclonus. In the electrophysiological examination performed for epileptic processes, epileptiform discharges localized to the isolated right hemisphere were observed. JME was considered primarily due to clinical findings in the patient, and effective seizure control was achieved in a 4-year follow-up under anti-seizure treatment. The peculiarity of the case is the presence of electrophysiology recordings of isolated unilateral epileptiform activity during the 4-year follow-up period. It should be emphasized that there is no case of JME diagnosed with isolated unilateral epileptiform activity in the absence of generalized spike-slow waves or multiple spike-slow waves in the literature.

青少年肌阵挛性癫痫(JME)具有明确的临床和电生理特征。另一方面,大量病例表明,局部和不对称放电可能伴随JME的广泛性癫痫样活动。虽然已知这些非广泛性电生理表现不能排除该综合征的诊断,但一些表现可能会在鉴别诊断中造成混淆。在这个病例报告中,我们讨论了一例脑电图表现为孤立的单侧癫痫样活动而没有典型的全身放电的JME病例。本病例临床表现为双侧上肢不自主抽搐运动。根据家庭录像,已经确定这些运动是单侧/双侧肌阵挛性搏动。在肌阵挛的病因调查中排除了代谢、毒性和结构问题。在对癫痫过程进行的电生理检查中,观察到癫痫样放电局限于分离的右半球。考虑JME主要是由于患者的临床表现,并且在抗癫痫治疗的4年随访中实现了有效的癫痫控制。该病例的特点是在4年随访期间存在孤立的单侧癫痫样活动的电生理记录。应该强调的是,文献中没有一例JME诊断为孤立的单侧癫痫样活动,没有普遍的尖峰-慢波或多重尖峰-慢波。
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引用次数: 0
The Increased Interleukin-6 Levels Can Be an Early Diagnostic Marker for New-Onset Refractory Status Epilepticus 白细胞介素-6水平升高可作为新发难治性癫痫的早期诊断标志
Pub Date : 2022-12-01 DOI: 10.14581/jer.220015
D. Kwack, D. W. Kim
New-onset refractory status epilepticus (NORSE) is a condition defined as the occurrence of refractory status epilepticus in patients without active epilepsy and no other acute causes of seizure. Although there is evidence that immune-mediated pathogenesis has a pivotal role in the epileptogenesis of NORSE, the diagnosis of NORSE is usually made on the clinical observation because there is no established biological marker suggesting the diagnosis of NORSE. We recently encountered a NORSE patient who was successfully treated with immunotherapy including tocilizumab, an anti-interleukin-6 (IL-6) receptor monoclonal antibody, and the markedly increased levels of serum and cerebrospinal fluid IL-6 were the only laboratory abnormality during the early treatment of the patient.
新发难治性癫痫持续状态(NORSE)是指在没有活动性癫痫且没有其他急性发作原因的患者中出现难治性持续状态。尽管有证据表明免疫介导的发病机制在NORSE的癫痫发生中起着关键作用,但NORSE的诊断通常是在临床观察上进行的,因为没有确定的生物学标志物表明诊断为NORSE。我们最近遇到一名NORSE患者,他成功地接受了免疫治疗,包括tocilizumab,一种抗白细胞介素-6(IL-6)受体单克隆抗体,血清和脑脊液IL-6水平显著升高是该患者早期治疗期间唯一的实验室异常。
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引用次数: 1
Evaluating the Role of Perilesional Tissue in Pathobiology of Epileptogenesis of Vascular Malformations of the Central Nervous System 评估病灶周围组织在中枢神经系统血管畸形癫痫发生病理生物学中的作用
Pub Date : 2022-12-01 DOI: 10.14581/jer.220010
R. Rajeswarie, H. Aravinda, A. Arivazhagan, N. Bevinahalli, M. Rao, A. Mahadevan
Background and Purpose Seizures are common presentation of cerebral vascular malformation (CVM). Topography and haemodynamic alterations are proposed as mechanisms for epileptogenesis, but the role of glial/neuronal alterations in perilesional tissue has not received much attention. Identification of the exact pathophysiologic basis could have therapeutic implications. To evaluate whether angioarchitectural factors of CVM or alterations in neuroglial/stroma of the adjacent cortex contribute to seizures. Method The clinical, imaging and histological characteristics of arteriovenous malformation (AVM) and cerebral cavernous malformation (CCM) with and without seizures was evaluated using neuroimaging imaging and digital subtraction angiography parameters and histopathology by morphology and immunohistochemistry. Results Fifty-six cases of CVM were diagnosed over a 2-year study period. Of these, 32 had adequate perilesional tissue for evaluation (AVM, 24; CCM, 8). Seizures at presentation was seen in 12/24 (50%) of AVM and 5/8 (62.5%) CCM. In AVM, hemosiderin deposition and gliosis in parenchyma (p=0.01) had significant association with seizure. Siderotic vessels in the adjacent cortex was exclusively seen only in CCM with seizures (p=0.018). Angioarchitectural features of CVM on imaging and neuronal alterations in adjacent cortex on histology failed to show any statistically significant difference between the two groups (p>0.05). Conclusions We propose that changes in adjacent cortex appear to be epileptogenic rather than the malformation per se. Reactive gliosis and hemosiderin deposits in perilesional tissue in AVM and siderotic vessels in CCM were associated with seizure. This explains the better outcomes following extended lesionectomy that includes epileptogenic perilesional tissues.
背景与目的癫痫发作是脑血管畸形(CVM)的常见表现。地形和血流动力学改变被认为是癫痫发生的机制,但神经胶质/神经元改变在病变周围组织中的作用尚未得到太多关注。确定确切的病理生理基础可能具有治疗意义。评估CVM的血管构建因素或相邻皮质神经胶质/基质的改变是否与癫痫发作有关。方法应用神经影像学和数字减影血管造影参数及形态学和免疫组织化学方法对伴有和不伴有癫痫发作的动静脉畸形(AVM)和脑海绵状血管瘤(CCM)的临床、影像学和组织学特征进行评价。结果在2年的研究期间确诊56例CVM。其中32例有足够的病变周围组织进行评估(AVM, 24例;12/24(50%)的AVM和5/8(62.5%)的CCM在发病时出现癫痫发作。在AVM中,含铁血黄素沉积和实质胶质瘤与癫痫发作有显著相关性(p=0.01)。相邻皮质侧缩血管仅在癫痫发作的CCM中可见(p=0.018)。两组CVM影像学血管建筑学特征及相邻皮层组织学上神经元改变无统计学差异(p < 0.05)。结论:我们认为邻近皮质的改变似乎是癫痫性的,而不是畸形本身。AVM的反应性胶质瘤和含铁血黄素沉积在病灶周围组织和CCM的含铁血黄素血管与癫痫发作有关。这解释了包括致痫性病变周围组织在内的扩展病变切除术的更好结果。
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引用次数: 0
Cerebral Salt Wasting Syndrome Associated with Status Epilepticus 与癫痫状态相关的脑耗盐综合征
Pub Date : 2022-12-01 DOI: 10.14581/jer.220016
Jung-Ju Lee
Cerebral salt wasting syndrome (CSWS) is defined as a renal loss of sodium in cerebral disorders causing hyponatremia and loss of extracellular fluid volume. Similar laboratory findings may be seen in other conditions such as syndrome of inappropriate antidiuretic hormone secretion (SIADH). A 58-year-old male visited our emergency department because of the sudden development of seizures during sleep. Magnetic resonance imaging revealed subtle high signal intensity in the right hippocampus on diffusion-weighted imaging. Ictal rhythmic discharges were observed in the concordant area. Altered metal status, polyuria and laboratory test findings including hyponatremia were compatible with CSWS. After hydration and salt replacement, his mental state and hyponatremia gradually recovered. For diagnosing CSWS, meticulous physical examinations including analysis of fluid balance are essential. CSWS should be considered in patients with hyponatremia and polyuria. Accurate diagnosis of CSWS and SIADH is crucial as the treatment plans for these two conditions are completely different.
脑盐耗综合征(CSWS)被定义为大脑疾病中钠的肾脏损失,导致低钠血症和细胞外液量的损失。类似的实验室发现也可能出现在其他情况下,如抗利尿激素分泌不当综合征(SIADH)。一位58岁的男性因为在睡眠中突然发作而去了我们的急诊室。磁共振成像显示,在扩散加权成像中,右侧海马有细微的高信号强度。在协调区观察到寒冰节律性放电。改变的金属状态、多尿和实验室检查结果(包括低钠血症)与CSWS相容。经过补水和换盐,他的精神状态和低钠血症逐渐恢复。对于诊断CSWS,细致的身体检查,包括液体平衡分析是必不可少的。低钠血症和多尿患者应考虑CSWS。CSWS和SIADH的准确诊断至关重要,因为这两种情况的治疗计划完全不同。
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引用次数: 0
Is Functional Connectivity after a First Unprovoked Seizure Different Based on Subsequent Seizures and Future Diagnosis of Epilepsy? 第一次非诱发性癫痫发作后的功能连通性是否与随后的癫痫发作和未来的癫痫诊断不同?
Pub Date : 2022-12-01 DOI: 10.14581/jer.22011
Ga Eun Koo, Ho Tae Jeong, Young Chul Youn, Su-Hyun Han

Background and purpose: There are no highly sensitive biomarkers for epilepsy to date. Recently, promising results regarding functional connectivity analysis have been obtained, which may improve epilepsy diagnosis even in the absence of visible abnormality in electroencephalography. We aimed to investigate the differences in functional connectivity after a first unprovoked seizure between patients diagnosed with epilepsy within 1 year due to subsequent seizures and those who were not.

Methods: We compared quantitative electroencephalography power spectra and functional connectivity between 12 patients who were diagnosed with epilepsy (two or more unprovoked seizures) within 1 year and 17 controls (those not diagnosed within 1 year) using iSyncBrain® (iMediSync Inc., Suwon, Korea; https://isyncbrain.com/). In the source-level analysis, the current distribution across the brain was assessed using the standardized low-resolution brain electromagnetic tomography technique, to compare relative power values in 68 regions of interest and connectivity (the imaginary part of coherency) between regions of interest.

Results: In the epilepsy group, quantitative electroencephalography showed lower alpha2 band power in left frontal, central, superior temporal, and parietal regions and higher beta2 power in both frontal, central, temporal, occipital, and left parietal regions compared with the control group. Additionally, epilepsy patients had significantly lower connectivity in alpha2 and beta2 bands than the controls.

Conclusions: Patients experiencing their first unprovoked seizure presented different brain function according to whether they have subsequent seizures and future epilepsy. Our results propose the potential clinical ability to diagnose epilepsy after the first unprovoked seizure in the absence of interictal epileptiform discharges.

背景与目的:目前尚无高度敏感的癫痫生物标志物。最近,在功能连接分析方面取得了令人鼓舞的结果,这可能会在脑电图没有明显异常的情况下提高癫痫的诊断。我们的目的是研究在1年内诊断为癫痫的患者和未诊断为癫痫的患者在首次非诱发性癫痫发作后的功能连通性差异。方法:我们使用iSyncBrain®(iMediSync Inc.,韩国水原)比较了12例1年内被诊断为癫痫(两次或两次以上非诱发性癫痫发作)患者和17例对照组(1年内未被诊断的患者)的定量脑电图功率谱和功能连通性;https://isyncbrain.com/)。在源级分析中,使用标准化的低分辨率脑电磁断层扫描技术评估整个大脑的电流分布,以比较68个感兴趣区域的相对功率值和感兴趣区域之间的连通性(相干性的虚部)。结果:癫痫组定量脑电图显示,与对照组相比,左额叶区、中央区、颞上区和顶叶区α 2频带功率较低,额叶区、中央区、颞叶区、枕叶区和左顶叶区α 2频带功率较高。此外,癫痫患者在α 2和β 2波段的连通性明显低于对照组。结论:第一次非诱发性癫痫发作的患者根据是否有后续癫痫发作和未来癫痫发作表现出不同的脑功能。我们的研究结果提出了潜在的临床能力,诊断癫痫后的第一次非诱发性发作,在没有癫痫样放电间期。
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引用次数: 0
A Case of Perampanel Overdose Presenting with Respiratory Failure Perampanel过量用药并发呼吸衰竭1例
Pub Date : 2022-12-01 DOI: 10.14581/jer.220012
Yangmi Park, Seondeuk Kim, D. Koo, H. Nam
Perampanel is a novel antiepileptic drug that has been used as an adjunctive treatment for focal-onset seizures. No reports to date have documented respiratory suppression as a side effect of perampanel in adults. Herein, we report a 51-year-old man with focal epilepsy presented with type 2 respiratory failure after accidently consuming of 66 mg of perampanel. Clinicians should consider the possibility of respiratory compromise whenever a high dose of perampanel needs to be administered to patients.
培安帕奈是一种新型的抗癫痫药物,已被用作局灶性发作癫痫的辅助治疗。到目前为止,没有任何报告记录呼吸抑制是成年人使用万帕的副作用。在此,我们报告了一名51岁的局灶性癫痫患者,在意外服用66mg的帕痛后出现2型呼吸衰竭。每当需要给患者服用高剂量的帕痛时,临床医生应考虑呼吸系统损害的可能性。
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引用次数: 0
The Increased Interleukin-6 Levels Can Be an Early Diagnostic Marker for New-Onset Refractory Status Epilepticus. 白细胞介素-6水平升高可作为新发难治性癫痫持续状态的早期诊断标志。
Pub Date : 2022-12-01 DOI: 10.14581/jer.22015
Dong Won Kwack, Dong Wook Kim

New-onset refractory status epilepticus (NORSE) is a condition defined as the occurrence of refractory status epilepticus in patients without active epilepsy and no other acute causes of seizure. Although there is evidence that immune-mediated pathogenesis has a pivotal role in the epileptogenesis of NORSE, the diagnosis of NORSE is usually made on the clinical observation because there is no established biological marker suggesting the diagnosis of NORSE. We recently encountered a NORSE patient who was successfully treated with immunotherapy including tocilizumab, an anti-interleukin-6 (IL-6) receptor monoclonal antibody, and the markedly increased levels of serum and cerebrospinal fluid IL-6 were the only laboratory abnormality during the early treatment of the patient.

新发难治性癫痫持续状态(NORSE)是指在无活动性癫痫且无其他急性发作原因的患者中出现难治性癫痫持续状态。虽然有证据表明免疫介导的发病机制在NORSE的癫痫发生中起着关键作用,但由于没有确定的生物学标志物提示NORSE的诊断,因此NORSE的诊断通常是在临床观察中做出的。我们最近遇到了一位NORSE患者,他成功地接受了免疫治疗,包括tocilizumab,一种抗白细胞介素6 (IL-6)受体单克隆抗体,在患者早期治疗期间,血清和脑脊液IL-6水平显着升高是唯一的实验室异常。
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引用次数: 0
A Case of Perampanel Overdose Presenting with Respiratory Failure. Perampanel过量致呼吸衰竭1例。
Pub Date : 2022-12-01 DOI: 10.14581/jer.22012
Yangmi Park, Seondeuk Kim, Dae Lim Koo, Hyunwoo Nam

Perampanel is a novel antiepileptic drug that has been used as an adjunctive treatment for focal-onset seizures. No reports to date have documented respiratory suppression as a side effect of perampanel in adults. Herein, we report a 51-year-old man with focal epilepsy presented with type 2 respiratory failure after accidently consuming of 66 mg of perampanel. Clinicians should consider the possibility of respiratory compromise whenever a high dose of perampanel needs to be administered to patients.

Perampanel是一种新型抗癫痫药物,已被用作局灶性癫痫发作的辅助治疗。到目前为止,还没有报告表明perampanel对成人有呼吸抑制的副作用。在此,我们报告了一名51岁的局灶性癫痫患者,在意外服用66 mg perampanel后出现2型呼吸衰竭。当需要给患者使用高剂量perampanel时,临床医生应考虑呼吸损伤的可能性。
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引用次数: 0
Clinical Profile and Predictors of In-Hospital Mortality in De Novo Convulsive Status Epilepticus in the Elderly Populace. 老年人群癫痫持续状态患者的临床特征及住院死亡率预测因素
Pub Date : 2022-12-01 DOI: 10.14581/jer.22009
Archana Verma, Alok Kumar, Pooja Pathak, Ashutosh Kumar Mishra

Background and purpose: De novo status epilepticus (SE) had worse outcome in comparison to the patients with SE who had previous history of epilepsy. The aim of the present study was to identify clinical features of de novo convulsive status epilepticus (CSE) and the predictors of in-hospital mortality.

Methods: Seventy-seven elderly (≥60 years of age) hospitalized patients with de novo CSE were evaluated for clinical profile, aetiologies and predictors of in-hospital mortality.

Results: The average age of the participants in the study was 65.96±6.72 years. In de novo CSE, the most common aetiologies were acute symptomatic in 68.8% of cases, followed by remote symptomatic in 24.7%. In-hospital mortality in the de novo CSE in the elderly was 30 (38.9%) in our series. Stroke was the leading cause of death among them (acute stroke in 23 cases and old infarct in 1 case), followed by post-traumatic (n=4) and CNS infection (n=2). On multivariate analysis, it was found that variables significantly related to mortality in de novo CSE were low Glasgow coma scale (GCS) (adjusted odds ratio [AOR], 53.5; 95% confidence interval [CI], 5.17-555.14; p=0.001) and lack of response to first line treatment (AOR, 0.06; 95% CI, 0.01-0.50; p=0.01).

Conclusions: In-hospital mortality in de novo CSE patients was linked to a low GCS and a lack of response to first-line therapy. The most efficient strategy to prevent in-hospital mortality in the elderly is to treat de novo CSE promptly and aggressively in the setting of stroke.

背景和目的:与既往有癫痫史的癫痫持续状态(SE)患者相比,重新发作的癫痫持续状态(SE)预后更差。本研究的目的是确定新生惊厥癫痫持续状态(CSE)的临床特征和住院死亡率的预测因素。方法:对77例老年(≥60岁)新生CSE住院患者的临床特征、病因和院内死亡率预测因素进行评估。结果:研究对象平均年龄为65.96±6.72岁。在新生CSE病例中,最常见的病因是急性症状(68.8%),其次是远程症状(24.7%)。在我们的研究中,老年新生CSE患者的住院死亡率为30(38.9%)。脑卒中是其中的主要死亡原因(急性脑卒中23例,老年性脑梗死1例),其次是创伤后(n=4)和中枢神经系统感染(n=2)。在多因素分析中,发现与新生CSE死亡率显著相关的变量是低格拉斯哥昏迷量表(GCS)(校正优势比[AOR], 53.5;95%置信区间[CI], 5.17-555.14;p=0.001)和对一线治疗缺乏反应(AOR, 0.06;95% ci, 0.01-0.50;p = 0.01)。结论:新生CSE患者的住院死亡率与低GCS和对一线治疗缺乏反应有关。预防老年人住院死亡率的最有效策略是在中风的情况下及时和积极地治疗新生CSE。
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引用次数: 0
Cerebral Salt Wasting Syndrome Associated with Status Epilepticus. 脑盐消耗综合征与癫痫持续状态相关。
Pub Date : 2022-12-01 DOI: 10.14581/jer.22016
Jung-Ju Lee

Cerebral salt wasting syndrome (CSWS) is defined as a renal loss of sodium in cerebral disorders causing hyponatremia and loss of extracellular fluid volume. Similar laboratory findings may be seen in other conditions such as syndrome of inappropriate antidiuretic hormone secretion (SIADH). A 58-year-old male visited our emergency department because of the sudden development of seizures during sleep. Magnetic resonance imaging revealed subtle high signal intensity in the right hippocampus on diffusion-weighted imaging. Ictal rhythmic discharges were observed in the concordant area. Altered metal status, polyuria and laboratory test findings including hyponatremia were compatible with CSWS. After hydration and salt replacement, his mental state and hyponatremia gradually recovered. For diagnosing CSWS, meticulous physical examinations including analysis of fluid balance are essential. CSWS should be considered in patients with hyponatremia and polyuria. Accurate diagnosis of CSWS and SIADH is crucial as the treatment plans for these two conditions are completely different.

脑盐消耗综合征(CSWS)被定义为脑疾病引起低钠血症和细胞外液容量损失的肾脏钠流失。类似的实验室结果也可见于其他情况,如抗利尿激素分泌不当综合征(SIADH)。一名58岁男性患者因睡眠中突然发作而来急诊科就诊。磁共振成像显示右侧海马弥散加权成像呈微弱高信号。和谐区有节律性放电。金属状态改变、多尿和实验室检查结果包括低钠血症与CSWS一致。补液补盐后,患者精神状态和低钠血症逐渐恢复。为了诊断CSWS,细致的身体检查包括体液平衡分析是必不可少的。低钠血症和多尿患者应考虑CSWS。CSWS和SIADH的准确诊断至关重要,因为这两种疾病的治疗方案完全不同。
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引用次数: 0
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Journal of epilepsy research
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