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Factors related to possible autoimmune etiology in patients with drug-resistant epilepsy 耐药性癫痫患者可能的自身免疫病因相关因素
Pub Date : 2023-11-01 DOI: 10.2478/joepi-2023-0001
Magdalena Kowalska, E. Nagańska, Urszula Fiszer
SUMMARY Background Between 14% and 20% of cases of drug-resistant epilepsy may be attributed to an impaired cellular or humoral immune response. Aim Our study aimed to assess disorders of the immune response of the humoral or cellular type and their impact on the course of the disease, factors suggesting the diagnosis of an autoimmune etiology. We wanted to analyse these factors, looking for correlations with a history of status epilepticus. Materials and methods This study prospectively analysed 30 patients who were diagnosed with drug-resistant epilepsy. The patients were divided into two groups: those who had previously experienced status epilepticus and those who had not. The study collected and analysed detailed information about the patient’s medical history, routine blood laboratory tests, albumin and immunoglobulin (IgG) levels, neuropsychological evaluations, electroencephalography tests (EEG), general cerebrospinal fluid (CSF) examinations, tests for the presence of oligoclonal bands, IgG index determination, MRZ-reaction (MRZR), chitotriosidase activity, and the presence of anti-herpes type 1 (anti-HSV-1) antibodies and neural autoantibodies. Each patient underwent magnetic resonance imaging (MRI) of the head with intravenous contrast administration using the epileptic protocol. Results There was no statistically significant difference in age, gender, onset and disease duration up to the time of our study. None of the patients showed the presence of the tested antibodies against neuronal surface antigens and oligoclonal bands in the CSF. Conclusion Lack of antibodies against neuronal antigens does not necessarily rule out autoimmune epilepsy. The exact diagnostic criteria are still a subject of debate. A history of SE increases the risk of autoimmune epilepsy.
摘要 背景 14%至 20%的耐药性癫痫可能是由于细胞或体液免疫反应受损所致。目的 我们的研究旨在评估体液或细胞免疫反应紊乱及其对病程的影响,这些因素提示了自身免疫病因的诊断。我们希望分析这些因素,寻找与癫痫状态病史的相关性。材料和方法 本研究对 30 名被诊断为耐药性癫痫的患者进行了前瞻性分析。患者被分为两组:曾经历过癫痫状态的患者和未经历过癫痫状态的患者。研究收集并分析了患者的详细病史、常规血液化验检查、白蛋白和免疫球蛋白(IgG)水平、神经心理评估、脑电图检查(EEG)、一般脑脊液(CSF)检查、寡克隆带检测、IgG指数测定、MRZ反应(MRZR)、壳三糖苷酶活性、抗1型疱疹病毒(抗HSV-1)抗体和神经自身抗体。每位患者都接受了头部磁共振成像(MRI)检查,并按照癫痫治疗方案静脉注射了造影剂。结果 在年龄、性别、发病时间和病程方面,直到我们进行研究时都没有明显的统计学差异。所有患者的脑脊液中均未检测出神经元表面抗原抗体和寡克隆带。结论 缺乏针对神经元抗原的抗体并不一定能排除自身免疫性癫痫。确切的诊断标准仍有争议。有 SE 病史会增加患自身免疫性癫痫的风险。
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引用次数: 0
Cenobamate in the management of focal-onset epilepsy in adults – practical considerations for daily practice 治疗成人局灶性癫痫的塞诺巴马特--日常实践中的实用注意事项
Pub Date : 2023-11-01 DOI: 10.2478/joepi-2023-0002
B. Majkowska-Zwolińska
SUMMARY Introduction Cenobamate (CNB) is a newly approved antiseizure medication in Europe. It is used as an add-on treatment for focal-onset seizures in adult patients with epilepsy that is not responding to other medications. Aim This report discusses the practical aspects of using cenobamate to treat adult patients with epilepsy based on current experiences. Discussion and conclusions Studies have shown that cenobamate is effective in reducing seizure frequency in adult patients with drug-resistant focal onset epilepsy when used as add-on therapy. It also has a high seizure freedom rate, a good treatment retention rate, and a favorable safety profile. The aspects discussed include using cenobamate in special populations and potential interactions with other drugs, management strategies to mitigate the risk of adverse reactions illustrated by a specific clinical case. Further studies involving larger patient groups are necessary to assess the drug’s efficacy and safety profile, particularly in special populations and patients with other types of epileptic seizures.
摘要 简介 塞诺巴马特(CNB)是欧洲新批准的一种抗癫痫药物。它可作为一种附加治疗药物,用于治疗对其他药物无效的成年癫痫患者的局灶性发作。目的 本报告根据目前的经验,讨论了使用仙诺巴马特治疗成年癫痫患者的实际问题。讨论与结论 研究表明,仙诺巴马特作为附加疗法可有效减少成人耐药性局灶性癫痫患者的癫痫发作频率。它还具有较高的癫痫发作自由率、良好的治疗保持率和良好的安全性。讨论的方面包括在特殊人群中使用仙诺巴马特、与其他药物的潜在相互作用、减轻不良反应风险的管理策略,并通过一个具体的临床病例加以说明。有必要对更大的患者群体进行进一步研究,以评估该药物的疗效和安全性,尤其是在特殊人群和其他类型癫痫发作患者中的疗效和安全性。
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引用次数: 0
How can we distinguish postictal Todd’s Paralysis from acute ischemic stroke in the prehospital and early hospital setting? 我们如何在院前和医院早期区分托德麻痹和急性缺血性中风?
Pub Date : 2022-08-30 DOI: 10.2478/joepi-2022-0002
Natasza Blek
Summary Introduction Acute Ischemic Stroke (AIS) is a medical emergency with focal neurological deficits. Todd’s paralysis (TP) is defined as a transient loss of motor ability and weakness that lasts hours to days and typically occurs after a focal seizure. Given the high prevalence of stroke and the rising availability of reperfusion therapies, timely detection of eligible patients is critical. Pre- and early-hospital differential diagnosis of various conditions with comparable clinical presentations is still difficult. Aim This review discusses Todd’s post-epileptic paralysis, one of the most common stroke mimics (SM), in pre- and early-hospital settings. Discussion and Conclusions The review covers the most critical findings on the TP and its emergency care as a common stroke mimic. Because TP is an excluding diagnosis, the most severe and curable illnesses must be recognised. Since thrombolysis is safe in SM, delaying or withholding medication may be improper when the advantages of treating a stroke mimic outweigh the dangers of treating a stroke mimic.
摘要简介急性缺血性脑卒中(AIS)是一种以局灶性神经功能缺损为主的医学急症。托德麻痹(TP)被定义为一种短暂的运动能力丧失和虚弱,持续数小时至数天,通常发生在局灶性癫痫发作后。鉴于卒中的高患病率和再灌注治疗的可用性不断提高,及时发现符合条件的患者至关重要。各种具有可比临床表现的疾病的院前和院早期鉴别诊断仍然很困难。目的本综述讨论托德的癫痫后瘫痪,最常见的中风模仿(SM)之一,在医院前和早期设置。讨论和结论本综述涵盖了TP及其作为一种常见卒中模拟物的急诊护理的最重要发现。由于TP是一种排除性诊断,因此必须认识到最严重和可治愈的疾病。由于溶栓在SM中是安全的,当治疗卒中模拟的好处大于治疗卒中模拟的危险时,延迟或不给药可能是不合适的。
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引用次数: 1
A patient with Beta-Propeller Protein-Associated Neurodegeneration: a new missense mutation of the WDR45 gene β -螺旋桨蛋白相关神经变性患者:WDR45基因的新错义突变
Pub Date : 2022-07-01 DOI: 10.2478/joepi-2022-0001
A. Ivanova, S. Kulikova, L. Sivitskaya, N. Danilenko, O. Davydenko
Summary Introduction Beta-propeller protein-associated neurodegeneration (BPAN) is a neurodegenerative disorder; its estimated prevalence is 2 to 3 per million individuals. All published cases of BPAN have been sporadic, with a clear female predominance and mutations in various exons of the WDR45 gene. Case presentation The study aimed to confirm the diagnosis of BPAN in a 9-year-old girl with a developmental delay since early childhood complicated with intellectual disability, lack of speech and febrile and non-febrile tonic-clonic seizures. The patient also had autistic symptoms as well as some Rett-like symptoms: stereotypical movements of the hands–twisting objects, putting hands in the mouth. Discussion Clinical exome analysis and Sanger sequencing of the proband have been performed to confirm the diagnosis. The novel heterozygous missense mutation c.755T>C of the WDR45 «autophagy» gene was revealed. Sanger sequencing of the trio (proband and parents) proved the de novo nature of mutation; its clinical significance has been defined as probably pathogenic. Thus, we report a new missense variant of the WDR45 gene in a girl with a clinical picture of BPAN. The use of NGS made it possible to get a correct diagnosis during rather a short period before the second debilitating phase of the disease started so that the physicians and the family would have time to prepare and hopefully choose the way to resist.
β -螺旋桨蛋白相关神经变性(BPAN)是一种神经退行性疾病;据估计,其流行率为每百万人中2至3人。所有已发表的BPAN病例都是散发性的,具有明显的女性优势和WDR45基因的各种外显子突变。本研究旨在确认一名9岁女童的BPAN的诊断,该女童自儿童早期发育迟缓并伴有智力障碍、言语缺乏和发热性和非发热性强直阵挛性癫痫。患者也有自闭症症状以及一些类似雷特的症状:手扭曲物体的典型动作,把手放进嘴里。已进行临床外显子组分析和先证者Sanger测序以确认诊断。WDR45“自噬”基因的新杂合错义突变C . 755t >C被发现。三人组(先证者和父母)的桑格测序证明了突变的新生性质;其临床意义已被定义为可能致病。因此,我们报告一个新的错义变异的WDR45基因在一个女孩与BPAN的临床图片。使用NGS可以在疾病的第二个衰弱阶段开始之前的相当短的时间内得到正确的诊断,以便医生和家属有时间准备并希望选择抵抗的方式。
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引用次数: 0
Succinic semialdehyde dehydrogenase deficiency (SSADH-D) in an eleven-month-old infant with marked hypotonia and staring episodes: a case report 琥珀酸半醛脱氢酶缺乏症(SSADH-D)在11个月大的婴儿与显著的低张力和凝视发作:一个案例报告
Pub Date : 2022-07-01 DOI: 10.2478/joepi-2022-0003
S. Mouskou, Katerina Anagnostopoulou, Danae Bikouli, E. Paramera, E. Papakonstantinou, A. Korona, Vasiliki Ziaka, Mirsini Mavrikou, A. Skouma
Summary Introduction Succinic semialdehyde dehydrogenase deficiency (SSADH), also known as 4-hydroxybutyric aciduria (OMIM #271980, 610045), is an ultra-rare neurometabolic disorder inherited in an autosomal recessive pattern. It is usually characterised by a relatively nonprogressive encephalopathy in the first two years of life with hypotonia and developmental delay, associated with mild ataxia and hyporeflexia, as well as delays in language and speech development. Case report We report on a case of a four-year-old girl with SSADH deficiency who presented, at the age of 11 months old, with marked hypotonia, global neurodevelopmental delay and epilepsy. The diagnosis of “Succinic semialdehyde dehydrogenase deficiency” was indicated as there was a marked elevation of the levels of 4-hydroxy-butyric and 3,4-dihydroxybutyric acid caused by mutation at the gene ALDH5A1 in the homozygous state, identified with WES technique. Currently, she is four years old and has a severe global psychomotor delay, excessive hypotonia, hyperextensibility, and ataxia and is free of seizures. Conclusion At the early stage of clinical presentation, the condition is difficult to differentiate from other encephalopathies. This case report suggests that analysis of urinary organic acids should be performed in all patients at risk to allow early diagnosis. DNA analysis with the WES technique can confirm the diagnosis.
琥珀酸半醛脱氢酶缺乏症(SSADH),又称4-羟基丁酸尿症(OMIM #271980, 610045),是一种罕见的常染色体隐性遗传的神经代谢疾病。其特征通常是在生命的头两年出现相对非进行性脑病,伴张力低下和发育迟缓,伴有轻度共济失调和反射减退,以及语言和言语发育迟缓。病例报告我们报告了一个四岁的女孩与SSADH缺乏症谁提出,在11个月大的时候,明显的张力低下,整体神经发育迟缓和癫痫。由于ALDH5A1基因纯合子突变导致4-羟基丁酸和3,4-二羟基丁酸水平明显升高,经WES技术鉴定为“琥珀酸半醛脱氢酶缺乏症”。目前,她四岁,有严重的全身性精神运动迟缓、过度张力过低、过度伸展和共济失调,无癫痫发作。结论在临床表现早期,本病难以与其他脑病鉴别。本病例报告建议,所有有风险的患者都应进行尿有机酸分析,以便早期诊断。DNA分析与WES技术可以证实诊断。
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引用次数: 0
Epilepsy knowledge, attitudes, behaviors, and associated factors among primary, post-primary, and secondary school teachers in Ouagadougou (Burkina Faso) 瓦加杜古(布基纳法索)小学、小学后和中学教师的癫痫知识、态度、行为及相关因素
Pub Date : 2021-10-01 DOI: 10.21307/jepil-2021-003
A. A. Dabilgou, A. Dravé, J. Kyelem, Naobar Meda, C. Napon, K. Karfo, J. Kaboré
SUMMARY Background. Epilepsy is one of the most common pediatric neurological disorders. The knowledge and attitude of teachers toward epileptic students can be crucial. Patients and methods. This cross-sectional study was carried on schoolteachers in the city of Ouagadougou during the period from March 02 to July 20, 2020. The schools were chosen randomly among a list of recognized public or private establishments. Results. Two hundred and twenty teachers were included in the study. Among them, 35.45% were post-primary teachers. Most of them had already heard of epilepsy (98.6%). For the majority of school teachers, epilepsy was not a contagious disease (74%). Regarding the causes of epilepsy, the majority of participants had listed brain disease (65%) and genetic disorders (18.20%). The majority of schoolteachers (70.9%) believed that students with epilepsy usually had associated mental retardation. For the majority of teachers (73.20%), epilepsy was a stigmatizing disease, and students with epilepsy should benefit from personalized supervision (65%). The majority of schoolteachers (75.9%) had a good knowledge of epilepsy, and 43.6% had good attitudes toward epilepsy. The factor associated with teachers’ knowledge was having witnessed an epileptic seizure (p < 0.05). The factors related to schoolteacher practice was gender (p < 0.05) and having already witnessed an epileptic seizure (p < 0.05) Conclusion. Our study found that teachers had a good knowledge of epilepsy, but attitudes and practices were inadequate.
摘要背景。癫痫是最常见的儿童神经系统疾病之一。教师对癫痫学生的认识和态度是至关重要的。患者和方法。本横断面研究于2020年3月2日至7月20日期间对瓦加杜古市的学校教师进行。这些学校是从一系列公认的公立或私立学校中随机挑选出来的。结果。共有220名教师参与了这项研究。其中小学后教师占35.45%。大多数患者(98.6%)已经听说过癫痫。对大多数学校教师来说,癫痫不是一种传染性疾病(74%)。关于癫痫的病因,大多数参与者列出了脑部疾病(65%)和遗传性疾病(18.20%)。大多数学校教师(70.9%)认为癫痫学生通常伴有智力低下。对于大多数教师(73.20%)来说,癫痫是一种耻辱性疾病,患有癫痫的学生应该受益于个性化监督(65%)。大多数教师(75.9%)对癫痫有良好的认识,43.6%的教师对癫痫有良好的态度。与教师知识相关的因素是曾目睹过癫痫发作(p < 0.05)。与教师实习相关的因素为性别(p < 0.05)和曾发生过癫痫发作(p < 0.05)。我们的研究发现,教师对癫痫有很好的认识,但态度和做法不充分。
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引用次数: 0
Epilepsy and autism: How does age at seizure onset factor in? 癫痫和自闭症:癫痫发作的年龄是如何影响的?
Pub Date : 2021-10-01 DOI: 10.21307/jepil-2021-002
C. Braun, Jonathan Elie-Fortier
SUMMARY Introduction. Three neuropathological conditions and two neurosurgical situations have been reported to present significantly earlier seizure onset in cases with autism than without. These are tuberous sclerosis, Angelman syndrome, the PCDH19 mutation, vagal nerve stimulation and epilepsy surgery. Method. We reviewed the case-report literature to determine the extension of this autism-specific early seizure onset effect across all relevant neuropathological conditions. Published clinical cases were collected fulfilling two inclusion criteria: age at seizure onset stated and presence (N = 1885 cases) or absence (N=4907 cases) of autism. We also documented the type and tractability/intractability of the epilepsy, genetic abnormality, neurologic syndrome, structural brain imaging findings and presence of intellectual disability when available. Results. Cases with autism presented significantly earlier seizure onset than cases without autism in 38 neuropathological conditions out of 162, including the previously established five. These 38 neuropathological conditions typically involved intractable epilepsy caused by focal cortical dysplasia located in the social brain, with the ictal or interictal electrical focus also located in the social brain. Within these 38 neuropathological conditions, in the cases with autism, the median seizure onset occurred between 50 days and 24 months after birth. Conclusion. Onset of severe seizure disorder during an early critical post-natal interval, caused by brain damage specifically located in the social brain, strongly associates with subsequent autism.
总结介绍。据报道,三种神经病理条件和两种神经外科情况下,自闭症患者的癫痫发作明显早于非自闭症患者。这些是结节硬化,天使综合征,PCDH19突变,迷走神经刺激和癫痫手术。方法。我们回顾了病例报告文献,以确定这种自闭症特异性早期癫痫发作效应在所有相关神经病理条件下的延伸。已发表的临床病例收集符合两项纳入标准:癫痫发作时陈述的年龄和存在(N= 1885例)或不存在(N=4907例)自闭症。我们还记录了癫痫的类型和易感性/难治性,遗传异常,神经系统综合征,脑结构成像结果和智力残疾的存在。结果。在162个神经病理条件中,有38个自闭症患者比非自闭症患者癫痫发作明显早,包括之前确定的5个。这38种神经病理情况通常涉及由位于社交脑的局灶性皮质发育不良引起的难治性癫痫,其临界或间歇电灶也位于社交脑。在这38种神经病理情况中,在自闭症病例中,癫痫发作的中位数发生在出生后50天和24个月之间。结论。在早期关键的产后间隔期间,由位于社会性脑的脑损伤引起的严重癫痫发作,与随后的自闭症密切相关。
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引用次数: 0
Levetiracetam as second-line treatment of status epilepticus – which dose should be applied? 左乙拉西坦作为癫痫持续状态的二线治疗-应使用何种剂量?
Pub Date : 2021-07-01 DOI: 10.21307/jepil-2021-001
J. Rösche, Bernd Schade
SUMMARY Introduction Since 2004 many treatment episodes in different stages of status epilepticus (SE) have been reported. Nowadays the use of levetiracetam (LEV) is recommended as a second-line treatment of SE, when the use of a benzodiazepine was not successful. Aim The evidence based on randomized controlled trials for the application of a particular weight-based dose of LEV as a second-line treatment in benzodiazepine refractory SE is investigated. Methods Pubmedsearches were undertaken using the terms “Levetiracetam-status-epilepticus-trials” and “Levetiracetam-status-randomized” on May 8th 2021. We identified 17 studies reporting treatment with LEV as second line treatment and reporting dosages in mg/kg body weight. We grouped the studies according to the reported dosages (i.e. 20–25 mg/kg; 30 mg/kg; 40 mg/kg, 60 mg/kg). For each group we calculated the mean efficacy rate and the standard deviation of the efficacy rate weighted for the number of cases in the different studies. Twelve studies compared LEV with 20 mg/kg phenytoin (PHT). In these studies, we analysed the relative efficacy rate in comparison to PHT with the same procedure. Results Seven studies used LEV 20–25 mg/kg, two studies 30 mg/kg, six studies 40 mg/kg and one study 60 mg/kg. Efficacy rate was highest in the group given 30 mg/kg (95% CI 87.5–90.1%). The relative efficacy rate with this weight-based dose was 1.12. This is just above the upper range of the 95% CI of the relative efficacy rate in studies using 40 mg/kg LEV (i.e. 1.11). The relative efficacy rates in the two other groups were considerably lower. Conclusion According to the randomized controlled trials published so far a weight-based dose of 30–40 mg/kg LEV may be appropriate for the treatment of benzodiazepine-refractory SE.
自2004年以来,许多癫痫持续状态(SE)不同阶段的治疗事件被报道。目前,当苯二氮卓类药物使用不成功时,推荐使用左乙拉西坦(LEV)作为SE的二线治疗。目的:通过随机对照试验,探讨一定体重剂量的LEV作为苯二氮卓类药物难治性SE的二线治疗的证据。方法于2021年5月8日在pubmed检索“左乙拉西坦-癫痫状态-试验”和“左乙拉西坦-状态-随机化”。我们确定了17项报告将LEV作为二线治疗的研究,并报告了以mg/kg体重为单位的剂量。我们根据报道的剂量(即20-25 mg/kg;30毫克/公斤;40 mg/kg, 60 mg/kg)。对于每一组,我们计算了不同研究中病例数加权后的平均有效率和有效率的标准差。12项研究比较了LEV与20 mg/kg苯妥英(PHT)。在这些研究中,我们分析了与PHT相比,采用相同程序的相对有效率。结果7项研究使用LEV 20 ~ 25 mg/kg, 2项研究使用LEV 30 mg/kg, 6项研究使用LEV 40 mg/kg, 1项研究使用LEV 60 mg/kg。30 mg/kg组有效率最高(95% CI 87.5-90.1%)。以体重为基础的剂量的相对有效率为1.12。这刚好高于使用40 mg/kg LEV的研究中相对有效率的95% CI上限(即1.11)。其他两组的相对有效率明显较低。结论根据目前发表的随机对照试验,以体重为基础的30-40 mg/kg LEV可能是治疗苯二氮卓类难治性SE的合适剂量。
{"title":"Levetiracetam as second-line treatment of status epilepticus – which dose should be applied?","authors":"J. Rösche, Bernd Schade","doi":"10.21307/jepil-2021-001","DOIUrl":"https://doi.org/10.21307/jepil-2021-001","url":null,"abstract":"SUMMARY Introduction Since 2004 many treatment episodes in different stages of status epilepticus (SE) have been reported. Nowadays the use of levetiracetam (LEV) is recommended as a second-line treatment of SE, when the use of a benzodiazepine was not successful. Aim The evidence based on randomized controlled trials for the application of a particular weight-based dose of LEV as a second-line treatment in benzodiazepine refractory SE is investigated. Methods Pubmedsearches were undertaken using the terms “Levetiracetam-status-epilepticus-trials” and “Levetiracetam-status-randomized” on May 8th 2021. We identified 17 studies reporting treatment with LEV as second line treatment and reporting dosages in mg/kg body weight. We grouped the studies according to the reported dosages (i.e. 20–25 mg/kg; 30 mg/kg; 40 mg/kg, 60 mg/kg). For each group we calculated the mean efficacy rate and the standard deviation of the efficacy rate weighted for the number of cases in the different studies. Twelve studies compared LEV with 20 mg/kg phenytoin (PHT). In these studies, we analysed the relative efficacy rate in comparison to PHT with the same procedure. Results Seven studies used LEV 20–25 mg/kg, two studies 30 mg/kg, six studies 40 mg/kg and one study 60 mg/kg. Efficacy rate was highest in the group given 30 mg/kg (95% CI 87.5–90.1%). The relative efficacy rate with this weight-based dose was 1.12. This is just above the upper range of the 95% CI of the relative efficacy rate in studies using 40 mg/kg LEV (i.e. 1.11). The relative efficacy rates in the two other groups were considerably lower. Conclusion According to the randomized controlled trials published so far a weight-based dose of 30–40 mg/kg LEV may be appropriate for the treatment of benzodiazepine-refractory SE.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"1 1","pages":"7 - 12"},"PeriodicalIF":0.0,"publicationDate":"2021-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78504455","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical characteristics of forced normalization and alternative psychosis with special consideration of the new anticonvulsants 强迫正常化和替代精神病的临床特点,特别考虑新的抗惊厥药
Pub Date : 2020-12-01 DOI: 10.21307/jepil-2020-001
W. Fröscher, V. Faust, T. Steinert
SUMMARY Background In the case of an alternative psychosis (AP) or forced normalization (FN), the patient alternates between periods of clinically manifest seizures and normal behavior, and other periods of seizure freedom or significant seizure reduction accompanied by psychosis or behavioral disturbances. In clinical practice and in the literature, the terms AP and FN are mostly used synonymously despite small differences. FN of the EEG is not only common to interictal mental disturbances but may also occur in the case of pre-ictal and postictal mental disturbances. Aim To update the 2007 review on “Alternative Psychoses of Epilepsy” in this journal with special consideration of the new anticonvulsants. Material and Methods We conducted a literature research from 1987 (in this year a psychosis, triggered by the first “new “anticonvulsant vigabatrin in a patient with epilepsy was reported for the first time) up to September 2019. Discussion AP/FN are rare events; only 10% of epileptic psychosis are AP/FN. AP/FN respectively occur with both generalized and focal epilepsy; in recent years, patients with focal epilepsy predominate. AP/FN generally present with behavioral disturbances of acute or subacute onset associated with thought disorder, delusions, hallucinations, significant mood change, or anxiety with depersonalization and derealization symptoms. The reports on EEG findings in patients with AP are inconsistent. In the case of FN, the EEG is by definition normal or substantially improved. The most prominent risk factor for the development of an AP/FN is the anticonvulsant medication. The following anticonvulsants have not been observed until now as triggers of an AP/FN in the literature reviewed by us: Acetazolamide and sulthiame (“old” anticonvulsants) and the “new” anticonvulsants brivaracetam, eslicarbazepine, pregabalin, retigabine, rufinamide, stiripentol. The treatment is based on 3 strategies: Reduction or complete cessation of anticonvulsants, change of anticonvulsants and administration of antipsychotic drugs. Conclusion The risk of an AP/FN is probably different for the individual dugs. At the current level of experience, gabapentin, pregabalin, oxcarbazepine or eslicarbazepine can be the first alternative if an AP/FN was triggered by another anticonvulsant in a patient with focal epilepsy. In generalized epilepsy, especially in patients with absences, valproic acid remains the first alternative.
背景:在选择性精神病(AP)或强迫正常化(FN)的病例中,患者在临床表现为癫痫发作和行为正常的时期和其他癫痫发作自由或显著癫痫发作减少的时期之间交替,并伴有精神病或行为障碍。在临床实践和文献中,尽管AP和FN有微小的差异,但它们大多是同义使用的。脑电图FN不仅常见于精神障碍间期,也可出现在精神障碍前后。目的更新本刊2007年关于“癫痫的替代精神病”的综述,特别考虑新的抗惊厥药。我们进行了一项从1987年到2019年9月的文献研究(今年首次报道了癫痫患者中第一种“新型”抗惊痫药维加巴特林引发的精神病)。AP/FN是罕见事件;只有10%的癫痫性精神病是AP/FN。AP/FN分别发生于全身性和局灶性癫痫;近年来以局灶性癫痫为主。AP/FN通常表现为急性或亚急性发作的行为障碍,伴有思维障碍、妄想、幻觉、明显的情绪变化或焦虑,伴有人格解体和现实感丧失症状。有关AP患者脑电图结果的报道不一致。在FN的情况下,脑电图正常或明显改善。发生AP/FN最重要的危险因素是抗惊厥药物。在我们回顾的文献中,以下抗惊厥药物尚未被观察到作为AP/FN的触发因素:乙酰唑胺和磺胺(“旧”抗惊厥药物)和“新”抗惊厥药物布瓦西坦、埃斯卡巴西平、普瑞巴林、瑞加滨、鲁非那胺、斯立哌酮。治疗基于3种策略:减少或完全停用抗惊厥药物,改变抗惊厥药物和给予抗精神病药物。结论不同药物发生AP/FN的风险可能不同。在目前的经验水平下,如果局灶性癫痫患者的AP/FN由另一种抗惊厥药引发,则加巴喷丁、普瑞巴林、奥卡西平或埃斯利卡巴西平可作为第一选择。在全身性癫痫中,特别是在不存在癫痫的患者中,丙戊酸仍然是第一选择。
{"title":"Clinical characteristics of forced normalization and alternative psychosis with special consideration of the new anticonvulsants","authors":"W. Fröscher, V. Faust, T. Steinert","doi":"10.21307/jepil-2020-001","DOIUrl":"https://doi.org/10.21307/jepil-2020-001","url":null,"abstract":"SUMMARY Background In the case of an alternative psychosis (AP) or forced normalization (FN), the patient alternates between periods of clinically manifest seizures and normal behavior, and other periods of seizure freedom or significant seizure reduction accompanied by psychosis or behavioral disturbances. In clinical practice and in the literature, the terms AP and FN are mostly used synonymously despite small differences. FN of the EEG is not only common to interictal mental disturbances but may also occur in the case of pre-ictal and postictal mental disturbances. Aim To update the 2007 review on “Alternative Psychoses of Epilepsy” in this journal with special consideration of the new anticonvulsants. Material and Methods We conducted a literature research from 1987 (in this year a psychosis, triggered by the first “new “anticonvulsant vigabatrin in a patient with epilepsy was reported for the first time) up to September 2019. Discussion AP/FN are rare events; only 10% of epileptic psychosis are AP/FN. AP/FN respectively occur with both generalized and focal epilepsy; in recent years, patients with focal epilepsy predominate. AP/FN generally present with behavioral disturbances of acute or subacute onset associated with thought disorder, delusions, hallucinations, significant mood change, or anxiety with depersonalization and derealization symptoms. The reports on EEG findings in patients with AP are inconsistent. In the case of FN, the EEG is by definition normal or substantially improved. The most prominent risk factor for the development of an AP/FN is the anticonvulsant medication. The following anticonvulsants have not been observed until now as triggers of an AP/FN in the literature reviewed by us: Acetazolamide and sulthiame (“old” anticonvulsants) and the “new” anticonvulsants brivaracetam, eslicarbazepine, pregabalin, retigabine, rufinamide, stiripentol. The treatment is based on 3 strategies: Reduction or complete cessation of anticonvulsants, change of anticonvulsants and administration of antipsychotic drugs. Conclusion The risk of an AP/FN is probably different for the individual dugs. At the current level of experience, gabapentin, pregabalin, oxcarbazepine or eslicarbazepine can be the first alternative if an AP/FN was triggered by another anticonvulsant in a patient with focal epilepsy. In generalized epilepsy, especially in patients with absences, valproic acid remains the first alternative.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"13 1","pages":"35 - 41"},"PeriodicalIF":0.0,"publicationDate":"2020-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79589883","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
mHealth tools in the management of epilepsy 癫痫管理中的移动健康工具
Pub Date : 2020-12-01 DOI: 10.21307/jepil-2020-007
Natasza Blek, P. Zwolinski
SUMMARY Introduction. Epilepsy is a persistent neurological condition characterized by frequent seizures that are not triggered by an environmental or reversible stimulus. Although not yet widely used, mobile health (mHealth) innovations have enhanced epilepsy care and prevention and are expected to play an increasing role in the ownership of smartphones, wearable devices and innovation in medical technology. Aim. The present review paper aims to summarize the current state of knowledge regarding the use of mHealth tools in epilepsy management. Discussion and Conclusions. In this paper, we review available mHealth tools that influence key epilepsy management elements. These components include patient education, self-management directly affecting seizure control, diagnosis and therapy, and managing medical data. mHealth solutions are a promising approach to epilepsy self-management; further work is needed to explore their effectiveness.
总结介绍。癫痫是一种持续的神经系统疾病,其特征是频繁发作,不是由环境刺激或可逆刺激引发的。虽然尚未广泛使用,但移动健康(mHealth)创新已经加强了癫痫的护理和预防,并有望在智能手机、可穿戴设备和医疗技术创新方面发挥越来越大的作用。的目标。本综述旨在总结关于在癫痫管理中使用移动健康工具的知识现状。讨论和结论。在本文中,我们回顾了影响关键癫痫管理元素的可用移动健康工具。这些组成部分包括患者教育、直接影响癫痫控制、诊断和治疗的自我管理,以及管理医疗数据。移动医疗解决方案是一种很有前途的癫痫自我管理方法;需要进一步的工作来探索其有效性。
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Journal of Epileptology
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