the moment of eye opening or closing, and predominantly at a flicker frequency of 15-16 Hz and wavelength of 700nm. Inheritance is autosomal dominant. Luminance studies with sunglasses show variable results in the suppression of PPR. A deep red color is required for induction of PPR. In the prevention of the video game Pokemon effect, blue lenses inhibit the PPR by the short wave length and diminished illumination. Seizures evoked by video games are a manifestation of photosensitive epilepsy. During the PPR 75% patients experience impaired consciousness, opening of eyes or jerking, and pain in the eyes. Persistence of PPR after stimulation is ended is not associated with a higher risk of seizures. Other abnormalities in the EEG, not the PPR are significant in the cause of postPPR seizures. Prognosis is generally good, especially after age 20 years. Valproic acid and levetiracetam are effective in eliminating PPR. (Hughes JR. The photoparoxysmal response: The probable cause of attacks during video games. Clin EEG and Neurosci Jan 2008;39(1): 1-7). (Reprints: John R Hughes, MD PhD, University of Illinois Medical Center (M/C 796), 912 S Wood Street, Chicago, IL 60612).
{"title":"Eyelid myoclonia with absences (Jeavons syndrome): still an overlooked epilepsy syndrome. Comments to Covanis review in this issue of Journal of Epileptology","authors":"S. Striano, P. Striano","doi":"10.1515/JOEPI-2015-0029","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0029","url":null,"abstract":"the moment of eye opening or closing, and predominantly at a flicker frequency of 15-16 Hz and wavelength of 700nm. Inheritance is autosomal dominant. Luminance studies with sunglasses show variable results in the suppression of PPR. A deep red color is required for induction of PPR. In the prevention of the video game Pokemon effect, blue lenses inhibit the PPR by the short wave length and diminished illumination. Seizures evoked by video games are a manifestation of photosensitive epilepsy. During the PPR 75% patients experience impaired consciousness, opening of eyes or jerking, and pain in the eyes. Persistence of PPR after stimulation is ended is not associated with a higher risk of seizures. Other abnormalities in the EEG, not the PPR are significant in the cause of postPPR seizures. Prognosis is generally good, especially after age 20 years. Valproic acid and levetiracetam are effective in eliminating PPR. (Hughes JR. The photoparoxysmal response: The probable cause of attacks during video games. Clin EEG and Neurosci Jan 2008;39(1): 1-7). (Reprints: John R Hughes, MD PhD, University of Illinois Medical Center (M/C 796), 912 S Wood Street, Chicago, IL 60612).","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"51 1","pages":"125 - 127"},"PeriodicalIF":0.0,"publicationDate":"2015-11-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82036974","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}
Pub Date : 2015-10-20DOI: 10.21307/joepi-2015-0032
Tobias Kniess, H. Stefan, Peter Brodisch
Summary Introduction The correct diagnosis of epileptic seizures and non-epileptic attacks has a decisive influence on treatment, counseling and duration of possible work limitations. Diagnostic efforts should aim towards classifying the seizure as precisely as possible. For risk assessments, e.g. at the workplace, a close cooperation and networking of all professionals involved in the epilepsy treatment, care and consultation processes is required. Aim To present guidelines for assessment of occupational capacity of persons with epilepsy and to discuss their value in clinical practice. Method and Material The German employer’s liability insurance association has recently published the new revised BGI 585 Risk Assessment Guidelines (DGUV information 250-001) framework and assistance in epilepsy in view of protection against unfair dismissal. These guidelines provide information on safety and health in the workplace. Throughout all the German federal states, 24 Network teams were established. During the period January 2010 and December 2013, 374 employees with epilepsy were consulted by an expert member of Network Epilepsy and Work (NEA) Team, of which 80 were prospectively included in a study and scientifically evaluated. Guidelines and discussion While conducting the risk assessment, a special medical fact check in accordance with the guidelines was used. In addition to medical aspects, the individual vocational and occupational situation was considered. Based on this assessment an individual recommendation was made relating to continuation of employment. The project NEA established regional teams of physicians, therapists, consultants from social services, employment offices and rehabilitation authorities across Germany in order to link by networks the complex medical and social aspects of reducing the risk of people with epilepsy losing their job. Results It was shown that support and consultation through the NEA team led to an endangered position of employment being maintained in 70% of cases. Conclusion In many cases, loss of employment can be prevented by consequent application of DGUV information 250-001 (recently revised from BGI 585) for risk assessment of epilepsy in employment, together with improved networking between medical professionals, occupational health professionals and social services.
摘要简介癫痫病发作与非癫痫病发作的正确诊断对治疗、咨询和可能的工作限制的持续时间具有决定性的影响。诊断工作的目标应该是尽可能精确地对癫痫发作进行分类。对于风险评估，例如在工作场所，需要与参与癫痫治疗、护理和咨询过程的所有专业人员密切合作并建立网络。目的提出癫痫患者职业能力评估指南，探讨其在临床实践中的价值。德国雇主责任保险协会最近发布了新修订的BGI 585风险评估指南(DGUV信息250-001)框架和癫痫援助，以防止不公平解雇。这些准则提供了有关工作场所安全和健康的信息。在德国所有联邦州，建立了24个网络小组。2010年1月至2013年12月，对374名癫痫员工进行网络癫痫与工作小组(Network epilepsy and Work, NEA)专家会诊，其中80人前瞻性纳入研究并进行科学评价。指导方针和讨论在进行风险评估时，根据指导方针进行了特殊的医疗事实核查。除了医疗方面，还考虑了个人的职业和职业状况。根据这项评估，提出了关于继续就业的个别建议。NEA项目在德国各地建立了由来自社会服务、就业办公室和康复当局的医生、治疗师、顾问组成的区域小组，以便通过网络将减少癫痫患者失业风险的复杂医疗和社会方面联系起来。结果通过NEA团队的支持和咨询，70%的患者的就业处于危险状态。结论:在许多情况下，通过随后应用DGUV信息250-001(最近从BGI 585修订)进行就业中癫痫风险评估，以及改善医疗专业人员、职业卫生专业人员和社会服务之间的联系，可以预防失业。
{"title":"Diagnosis of epilepsy - consequences for work and professional activities","authors":"Tobias Kniess, H. Stefan, Peter Brodisch","doi":"10.21307/joepi-2015-0032","DOIUrl":"https://doi.org/10.21307/joepi-2015-0032","url":null,"abstract":"Summary Introduction The correct diagnosis of epileptic seizures and non-epileptic attacks has a decisive influence on treatment, counseling and duration of possible work limitations. Diagnostic efforts should aim towards classifying the seizure as precisely as possible. For risk assessments, e.g. at the workplace, a close cooperation and networking of all professionals involved in the epilepsy treatment, care and consultation processes is required. Aim To present guidelines for assessment of occupational capacity of persons with epilepsy and to discuss their value in clinical practice. Method and Material The German employer’s liability insurance association has recently published the new revised BGI 585 Risk Assessment Guidelines (DGUV information 250-001) framework and assistance in epilepsy in view of protection against unfair dismissal. These guidelines provide information on safety and health in the workplace. Throughout all the German federal states, 24 Network teams were established. During the period January 2010 and December 2013, 374 employees with epilepsy were consulted by an expert member of Network Epilepsy and Work (NEA) Team, of which 80 were prospectively included in a study and scientifically evaluated. Guidelines and discussion While conducting the risk assessment, a special medical fact check in accordance with the guidelines was used. In addition to medical aspects, the individual vocational and occupational situation was considered. Based on this assessment an individual recommendation was made relating to continuation of employment. The project NEA established regional teams of physicians, therapists, consultants from social services, employment offices and rehabilitation authorities across Germany in order to link by networks the complex medical and social aspects of reducing the risk of people with epilepsy losing their job. Results It was shown that support and consultation through the NEA team led to an endangered position of employment being maintained in 70% of cases. Conclusion In many cases, loss of employment can be prevented by consequent application of DGUV information 250-001 (recently revised from BGI 585) for risk assessment of epilepsy in employment, together with improved networking between medical professionals, occupational health professionals and social services.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"34 1","pages":"103 - 112"},"PeriodicalIF":0.0,"publicationDate":"2015-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79750459","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}
Summary Introduction Eyelid myoclonia and absences (ELMA) was first described by Jeavons in 1977 as a separate type of photosensitive epilepsy. Aim and method The aim is to consider the updated electro-clinical pathophysiology and to discuss terminology, classification and differential diagnosis. The review includes our own research and relevant papers on the subject of Jeavons syndrome (JS). Review and differential diagnosis Definition eavons syndrome is a generalized idiopathic (genetic) epilepsy syndrome (IGE) characterized by eyelid myoclonia, other seizures (absences, myoclonic and or generalized tonic-clonic) and EEG paroxysms induced by voluntary or on command eye closure, in the light and photosensitivity. Demographical data The prevalence of JS has been reported to vary from 7.3% to 12.9% among idiopathic generalized epilepsies and 2.5% to 2.7% among all patients with epileptic disorders. Etiology JS, as is the case for all idiopathic generalized epilepsies, is genetic and the familial preponderance and concordance is high. Pathophysiology Three factors are important in order for JS to manifest clinically; the genetic predisposition, the voluntary or on command eye closure and the light input. Clinical forms of JS we have identified four forms of JS; early onset (< 4 years), mild form, classical form and an ELMA-JME form. Diagnosis the diagnosis of JS is based on the history, clinical observation and provocation and the confirmation with an EEG. Differential diagnosis is easily made from tics, other idiopathic generalized or focal cryptogenic/symptomatic epilepsies. Conclusion JS is characterized by unique electro-clinical features evoked by voluntary or on command eye closure in the light and photosensitivity.
{"title":"Jeavons syndrome – updated review","authors":"A. Covanis","doi":"10.1515/JOEPI-2015-0033","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0033","url":null,"abstract":"Summary Introduction Eyelid myoclonia and absences (ELMA) was first described by Jeavons in 1977 as a separate type of photosensitive epilepsy. Aim and method The aim is to consider the updated electro-clinical pathophysiology and to discuss terminology, classification and differential diagnosis. The review includes our own research and relevant papers on the subject of Jeavons syndrome (JS). Review and differential diagnosis Definition eavons syndrome is a generalized idiopathic (genetic) epilepsy syndrome (IGE) characterized by eyelid myoclonia, other seizures (absences, myoclonic and or generalized tonic-clonic) and EEG paroxysms induced by voluntary or on command eye closure, in the light and photosensitivity. Demographical data The prevalence of JS has been reported to vary from 7.3% to 12.9% among idiopathic generalized epilepsies and 2.5% to 2.7% among all patients with epileptic disorders. Etiology JS, as is the case for all idiopathic generalized epilepsies, is genetic and the familial preponderance and concordance is high. Pathophysiology Three factors are important in order for JS to manifest clinically; the genetic predisposition, the voluntary or on command eye closure and the light input. Clinical forms of JS we have identified four forms of JS; early onset (< 4 years), mild form, classical form and an ELMA-JME form. Diagnosis the diagnosis of JS is based on the history, clinical observation and provocation and the confirmation with an EEG. Differential diagnosis is easily made from tics, other idiopathic generalized or focal cryptogenic/symptomatic epilepsies. Conclusion JS is characterized by unique electro-clinical features evoked by voluntary or on command eye closure in the light and photosensitivity.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"42 1","pages":"113 - 123"},"PeriodicalIF":0.0,"publicationDate":"2015-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80603970","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}
D espite the recent discovery of many newer and safer antiepileptic drugs (AEDs) the number of patients with drug resistant epilepsy remains at about 30%. Epilepsy is particularly devastating in the first years of life often causing mental retardation and autistic behavior, usually leading to epileptic encephalopathies. A better understanding of epilepsy development (epileptogenesis) in this group of patients may lead to successful prevention of drug-resistant epilepsy and its comorbidities (Barker-Haliski et al., 2015; Chapman et al., E-pub ahead of print). Scientific basis for possible intervention has been established in recent animal genetic models of epilepsy. In their study, Yan et al. (2005) used an epileptic double mutant rat (SER; zi/zi, tm/tm) which after age 7–8 weeks spontaneously exhibits tonic and absencelike seizures in response to mild sensory stimulation. Three weeks before the expected time of seizure onset, from postnatal weeks 5 to 8, rats received levetiracetam at 80 mg/kg/day (i.p). The seizure assessment has been carried out in weeks 12 and 13 (5 weeks after termination of the administration), by clinical evaluation and conventional EEG recordings. The incidence of both tonic and absence-like seizures was significantly lower in levetiracetam treated group comparing with the control animals. This effect was suggested to be due to an antiepileptogenic rather than an antiseizure effect, as the half-life time of levetiracetam in plasma is short (2–3h in rats) after single and long term administration (Yan et al, 2005). Another group of researchers performed the experiment in a genetic model of human absence epilepsy. Wistar Albino Glaxo/Rij rats, that exhibit spontaneous spike-wake complexes on electroencephalography and seizures at 3 moths of life, were administered oral ethosuximide from day 21 to 5 months of age. On follow-up at 8 months, after several months of discontinuation of the treatment, the significant suppression of discharges and lower number of seizures was still documented (Blumenfeld et al., 2008). Both Yan et al. (2005) and Blumenfeld et al. (2008) studies demonstrated that epilepsy prevention may be possible and that such a strategy could be considered in susceptible human cohorts. Indeed similar studies in humans carried out in selected groups of patients in the first year of life with a high risk of epilepsy, seem to corroborate the results obtained in animals. Ville et al. (2002) identified a group of 16 infants with Sturge-Weber syndrome, the condition characterized by early onset of seizures and poor mental outcome. Preventative treatment of this group with phenobarbitone, the drug frequently used for neonatal seizures, resulted in decreased epilepsy (p < 0.01) and mental retardation (p < 0.05) incidence in comparison to 21 children treated in the standard manner (i.e. after the onset of clinical seizures). The results are concordant with our results obtained in tuberous sclerosis complex (TSC) (Jozwiak
D .尽管最近发现了许多更新和更安全的抗癫痫药物，但耐药癫痫患者的数量仍保持在30%左右。癫痫在生命的头几年尤其具有破坏性，通常会导致智力迟钝和自闭行为，通常会导致癫痫性脑病。更好地了解这组患者的癫痫发展(癫痫发生)可能导致成功预防耐药癫痫及其合并症(Barker-Haliski et al.， 2015;查普曼等人，电子酒吧先于印刷)。最近的癫痫动物遗传模型为可能的干预措施建立了科学基础。在他们的研究中，Yan等人(2005)使用了一只癫痫双突变大鼠(SER;Zi / Zi, tm/tm)， 7-8周后，在轻微的感觉刺激下自发表现出强直性和缺席性癫痫发作。在预计癫痫发作时间前3周，即出生后第5 ~ 8周，给予左乙拉西坦80 mg/kg/天(i.p)。通过临床评估和常规脑电图记录，在第12周和第13周(停药后5周)进行癫痫发作评估。左乙拉西坦治疗组强直性和缺席样癫痫发作的发生率明显低于对照组。这种作用被认为是由于抗癫痫作用而不是抗癫痫作用，因为单次和长期给药后左乙拉西坦在血浆中的半衰期很短(大鼠2 - 3小时)(Yan et al, 2005)。另一组研究人员在人类缺失性癫痫的遗传模型中进行了实验。Wistar白化葛兰素/Rij大鼠在3个月大时脑电图表现出自发的尖峰-尾流复合物和癫痫发作，从第21天至5个月大时口服乙氧亚胺。在8个月的随访中，在停止治疗几个月后，仍然记录到显著抑制出院和减少癫痫发作次数(Blumenfeld et al.， 2008)。Yan等人(2005)和Blumenfeld等人(2008)的研究都表明，预防癫痫是可能的，而且在易感人群中可以考虑采用这种策略。事实上，在一组癫痫高风险的一岁婴儿中进行的类似人类研究似乎证实了在动物身上获得的结果。Ville等人(2002)确定了一组16名患有斯特奇-韦伯综合征的婴儿，这种疾病的特点是癫痫发作早，精神预后差。本组患儿给予新生儿癫痫发作常用药物苯巴比妥预防治疗后，癫痫发生率(p < 0.01)和智力发育迟滞发生率(p < 0.05)较21例患儿标准治疗(即临床癫痫发作后)显著降低。该结果与我们在结节性硬化症(TSC)中获得的结果一致(Jozwiak et al.， 2011)。在一项前瞻性研究中，14名婴儿每4-6周定期接受脑电图评估，直到24个月大，其中10名患者因脑电图发作性多灶活动而接受维加巴特林治疗。如果没有出现临床癫痫发作，则在24个月时停止治疗。尽管服用了药物，10个孩子中有6个还是患上了癫痫。然而，我们证明了较低的耐药发生率
{"title":"Is epileptogenesis a key to treatment of childhood epileptic encephalopathies?","authors":"S. Jóźwiak, K. Kotulska","doi":"10.1515/JOEPI-2015-0027","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0027","url":null,"abstract":"D espite the recent discovery of many newer and safer antiepileptic drugs (AEDs) the number of patients with drug resistant epilepsy remains at about 30%. Epilepsy is particularly devastating in the first years of life often causing mental retardation and autistic behavior, usually leading to epileptic encephalopathies. A better understanding of epilepsy development (epileptogenesis) in this group of patients may lead to successful prevention of drug-resistant epilepsy and its comorbidities (Barker-Haliski et al., 2015; Chapman et al., E-pub ahead of print). Scientific basis for possible intervention has been established in recent animal genetic models of epilepsy. In their study, Yan et al. (2005) used an epileptic double mutant rat (SER; zi/zi, tm/tm) which after age 7–8 weeks spontaneously exhibits tonic and absencelike seizures in response to mild sensory stimulation. Three weeks before the expected time of seizure onset, from postnatal weeks 5 to 8, rats received levetiracetam at 80 mg/kg/day (i.p). The seizure assessment has been carried out in weeks 12 and 13 (5 weeks after termination of the administration), by clinical evaluation and conventional EEG recordings. The incidence of both tonic and absence-like seizures was significantly lower in levetiracetam treated group comparing with the control animals. This effect was suggested to be due to an antiepileptogenic rather than an antiseizure effect, as the half-life time of levetiracetam in plasma is short (2–3h in rats) after single and long term administration (Yan et al, 2005). Another group of researchers performed the experiment in a genetic model of human absence epilepsy. Wistar Albino Glaxo/Rij rats, that exhibit spontaneous spike-wake complexes on electroencephalography and seizures at 3 moths of life, were administered oral ethosuximide from day 21 to 5 months of age. On follow-up at 8 months, after several months of discontinuation of the treatment, the significant suppression of discharges and lower number of seizures was still documented (Blumenfeld et al., 2008). Both Yan et al. (2005) and Blumenfeld et al. (2008) studies demonstrated that epilepsy prevention may be possible and that such a strategy could be considered in susceptible human cohorts. Indeed similar studies in humans carried out in selected groups of patients in the first year of life with a high risk of epilepsy, seem to corroborate the results obtained in animals. Ville et al. (2002) identified a group of 16 infants with Sturge-Weber syndrome, the condition characterized by early onset of seizures and poor mental outcome. Preventative treatment of this group with phenobarbitone, the drug frequently used for neonatal seizures, resulted in decreased epilepsy (p < 0.01) and mental retardation (p < 0.05) incidence in comparison to 21 children treated in the standard manner (i.e. after the onset of clinical seizures). The results are concordant with our results obtained in tuberous sclerosis complex (TSC) (Jozwiak ","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"188 2","pages":"87 - 88"},"PeriodicalIF":0.0,"publicationDate":"2015-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91479990","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}
Summary Background Cerebral palsy (CP) is the most common childhood motor impairment. Epilepsy affects approximately one third of patients with CP. It is characterized by earlier disclosure, it is more severe and shows greater resistance than that of the general epilepsy treatment, associated with necessity for polytherapy. Its presence can result in gradual loss of function, loss of posture in non-ambulant individuals with severe disabilities and cognitive impairment risk, behavioural disorders and reducing probability of walking. Aim The aim of the study was to evaluate the functioning of people with CP with and without epilepsy. Material and Methods The study included 210 patients with a diagnosis of CP, aged 0–18 years. The study was conducted among the patients using the physiotherapy services in centres in southern Poland. The study used the Paediatric Evaluation of Disability Inventory (PEDI) and the classification systems: GMFCS, MACS, and CFCS. Results There were significant differences with regards to social functioning (53.7/67.4; W = 179, p = 0.006) and support in the social functioning (65.4/89.9; W = 185.5, p = 0.007) in patients with diplegia. However, mobility (19.55/29.00; W = 392, p = 0.018) and the social functioning (36.95/44.1; W = 418.5, p = 0.042) were lower in epileptic patients with tetraplegia. In patients with hemiplegia, there were no significant differences, although each domain with epilepsy subgroup had a lower rating than the subgroup without epilepsy. Conclusion The presence of epilepsy is associated with lower levels of social function in patients with cerebral palsy; particularly, with regard to mobility and selfservice. Assessment of epilepsy impact on the level of social functioning of people with CP (diplegia, tetraplegia, hemiplegia) is difficult because ambiguous relationship with mental retardation. The assessment should be undertaken separately for each group of spastic CP.
背景脑瘫是儿童最常见的运动障碍。癫痫影响约三分之一的CP患者。其特点是较早发现，比一般癫痫治疗更严重，并表现出更大的耐药性，与综合治疗的必要性有关。它的存在可导致功能逐渐丧失，严重残疾、认知障碍风险、行为障碍和行走可能性降低的不能行走的个体失去姿势。目的本研究的目的是评估伴有和不伴有癫痫的CP患者的功能。材料与方法本研究纳入210例诊断为CP的患者，年龄0 ~ 18岁。该研究是在波兰南部中心使用物理治疗服务的患者中进行的。该研究采用儿科残疾评估量表(PEDI)和分类系统:GMFCS、MACS和CFCS。结果两组在社会功能方面差异有统计学意义(53.7/67.4;W = 179, p = 0.006)和社会功能支持(65.4/89.9;W = 185.5, p = 0.007)。但是，流动性(19.55/29.00;W = 392, p = 0.018)和社会功能(36.95/44.1;W = 418.5, p = 0.042)。在偏瘫患者中，虽然有癫痫亚组的每个域的评分低于没有癫痫的亚组，但没有显著差异。结论癫痫的存在与脑瘫患者社交功能水平降低有关;特别是在流动性和自助服务方面。评估癫痫对CP(双瘫、四肢瘫、偏瘫)患者社会功能水平的影响是困难的，因为癫痫与智力迟钝的关系不明确。每组痉挛性脑瘫应分别进行评估。
{"title":"The effect of seizures on functional status of people with spastic forms of cerebral palsy","authors":"A. Michalska, J. Wendorff","doi":"10.1515/JOEPI-2015-0031","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0031","url":null,"abstract":"Summary Background Cerebral palsy (CP) is the most common childhood motor impairment. Epilepsy affects approximately one third of patients with CP. It is characterized by earlier disclosure, it is more severe and shows greater resistance than that of the general epilepsy treatment, associated with necessity for polytherapy. Its presence can result in gradual loss of function, loss of posture in non-ambulant individuals with severe disabilities and cognitive impairment risk, behavioural disorders and reducing probability of walking. Aim The aim of the study was to evaluate the functioning of people with CP with and without epilepsy. Material and Methods The study included 210 patients with a diagnosis of CP, aged 0–18 years. The study was conducted among the patients using the physiotherapy services in centres in southern Poland. The study used the Paediatric Evaluation of Disability Inventory (PEDI) and the classification systems: GMFCS, MACS, and CFCS. Results There were significant differences with regards to social functioning (53.7/67.4; W = 179, p = 0.006) and support in the social functioning (65.4/89.9; W = 185.5, p = 0.007) in patients with diplegia. However, mobility (19.55/29.00; W = 392, p = 0.018) and the social functioning (36.95/44.1; W = 418.5, p = 0.042) were lower in epileptic patients with tetraplegia. In patients with hemiplegia, there were no significant differences, although each domain with epilepsy subgroup had a lower rating than the subgroup without epilepsy. Conclusion The presence of epilepsy is associated with lower levels of social function in patients with cerebral palsy; particularly, with regard to mobility and selfservice. Assessment of epilepsy impact on the level of social functioning of people with CP (diplegia, tetraplegia, hemiplegia) is difficult because ambiguous relationship with mental retardation. The assessment should be undertaken separately for each group of spastic CP.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"69 1","pages":"91 - 102"},"PeriodicalIF":0.0,"publicationDate":"2015-06-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74060834","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}
A. Rohracher, J. Dobesberger, C. Granbichler, J. Höfler, G. Kuchukhidze, M. Ortler, I. Unterberger, G. Walser, Aljoscha Thomschewski, E. Trinka
SUMMARY Background. Early identification of potential epilepsy surgery candidates is essential to the treatment process. Aim. To evaluate the clinical applicability of the ILAE definition of drug resistant epilepsy and its potential in identifying surgical candidates earlier compared to three established "older" definitions of drug resistant epi- lepsy. Material and Methods. Retrospective analysis of 174 patients who underwent epilepsy surgery between 1998 and 2009. Clinical factors and course of disease were extracted from patientscharts. Drug resistant epilepsy was classified according to four definitions and the time until fulfillment of criteria compared. Results. Mean time to fulfillment of criteria of drug resistant epilepsy ranged from 11.8 (standard deviation (SD) 9.8) to 15.6 years (SD 11.3). Time to drug resistance was significantly longer applying the only definition, requiring failure of three antiepileptic drugs (AEDs) (Canada definition), whereas time to fulfillment of all other definitions did not differ. Fifty percent of all patients experienced a seizure free period of ≥1 year prior to being classified as drug resistant, 13% entered another 1-year remission after fulfilling any criteria for drug resistance. Conclusion. We conclude that the ILAE definition identifies drug resistant epilepsy, with similar latency like two of three formerly used definitions. It is an easy applicable tool to minimize the delay of referral to a specialized center. Intermittent remissions delay assessment of drug resistance for all definitions and 13% of patients enter a remission despite established drug resistance.
{"title":"The ILAE definition of drug resistant epilepsy and its clinical applicability compared with \"older\" established definitions","authors":"A. Rohracher, J. Dobesberger, C. Granbichler, J. Höfler, G. Kuchukhidze, M. Ortler, I. Unterberger, G. Walser, Aljoscha Thomschewski, E. Trinka","doi":"10.1515/JOEPI-2015-0025","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0025","url":null,"abstract":"SUMMARY Background. Early identification of potential epilepsy surgery candidates is essential to the treatment process. Aim. To evaluate the clinical applicability of the ILAE definition of drug resistant epilepsy and its potential in identifying surgical candidates earlier compared to three established \"older\" definitions of drug resistant epi- lepsy. Material and Methods. Retrospective analysis of 174 patients who underwent epilepsy surgery between 1998 and 2009. Clinical factors and course of disease were extracted from patientscharts. Drug resistant epilepsy was classified according to four definitions and the time until fulfillment of criteria compared. Results. Mean time to fulfillment of criteria of drug resistant epilepsy ranged from 11.8 (standard deviation (SD) 9.8) to 15.6 years (SD 11.3). Time to drug resistance was significantly longer applying the only definition, requiring failure of three antiepileptic drugs (AEDs) (Canada definition), whereas time to fulfillment of all other definitions did not differ. Fifty percent of all patients experienced a seizure free period of ≥1 year prior to being classified as drug resistant, 13% entered another 1-year remission after fulfilling any criteria for drug resistance. Conclusion. We conclude that the ILAE definition identifies drug resistant epilepsy, with similar latency like two of three formerly used definitions. It is an easy applicable tool to minimize the delay of referral to a specialized center. Intermittent remissions delay assessment of drug resistance for all definitions and 13% of patients enter a remission despite established drug resistance.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"278 1","pages":"39-44"},"PeriodicalIF":0.0,"publicationDate":"2015-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77109078","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}
Pub Date : 2015-04-02DOI: 10.21307/joepi-2015-0025
A. Rohracher, J. Dobesberger, C. Granbichler, J. Höfler, G. Kuchukhidze, M. Ortler, I. Unterberger, G. Walser, Aljoscha Thomschewski, E. Trinka
Summary Background Early identification of potential epilepsy surgery candidates is essential to the treatment process. Aim To evaluate the clinical applicability of the ILAE definition of drug resistant epilepsy and its potential in identifying surgical candidates earlier compared to three established “older” definitions of drug resistant epilepsy. Material and Methods Retrospective analysis of 174 patients who underwent epilepsy surgery between 1998 and 2009. Clinical factors and course of disease were extracted from patients’ charts. Drug resistant epilepsy was classified according to four definitions and the time until fulfillment of criteria compared. Results Mean time to fulfillment of criteria of drug resistant epilepsy ranged from 11.8 (standard deviation (SD) 9.8) to 15.6 years (SD 11.3). Time to drug resistance was significantly longer applying the only definition, requiring failure of three antiepileptic drugs (AEDs) (Canada definition), whereas time to fulfillment of all other definitions did not differ. Fifty percent of all patients experienced a seizure free period of ≥1 year prior to being classified as drug resistant, 13% entered another 1-year remission after fulfilling any criteria for drug resistance. Conclusion We conclude that the ILAE definition identifies drug resistant epilepsy, with similar latency like two of three formerly used definitions. It is an easy applicable tool to minimize the delay of referral to a specialized center. Intermittent remissions delay assessment of drug resistance for all definitions and 13% of patients enter a remission despite established drug resistance.
{"title":"The ILAE definition of drug resistant epilepsy and its clinical applicability compared with “older” established definitions","authors":"A. Rohracher, J. Dobesberger, C. Granbichler, J. Höfler, G. Kuchukhidze, M. Ortler, I. Unterberger, G. Walser, Aljoscha Thomschewski, E. Trinka","doi":"10.21307/joepi-2015-0025","DOIUrl":"https://doi.org/10.21307/joepi-2015-0025","url":null,"abstract":"Summary Background Early identification of potential epilepsy surgery candidates is essential to the treatment process. Aim To evaluate the clinical applicability of the ILAE definition of drug resistant epilepsy and its potential in identifying surgical candidates earlier compared to three established “older” definitions of drug resistant epilepsy. Material and Methods Retrospective analysis of 174 patients who underwent epilepsy surgery between 1998 and 2009. Clinical factors and course of disease were extracted from patients’ charts. Drug resistant epilepsy was classified according to four definitions and the time until fulfillment of criteria compared. Results Mean time to fulfillment of criteria of drug resistant epilepsy ranged from 11.8 (standard deviation (SD) 9.8) to 15.6 years (SD 11.3). Time to drug resistance was significantly longer applying the only definition, requiring failure of three antiepileptic drugs (AEDs) (Canada definition), whereas time to fulfillment of all other definitions did not differ. Fifty percent of all patients experienced a seizure free period of ≥1 year prior to being classified as drug resistant, 13% entered another 1-year remission after fulfilling any criteria for drug resistance. Conclusion We conclude that the ILAE definition identifies drug resistant epilepsy, with similar latency like two of three formerly used definitions. It is an easy applicable tool to minimize the delay of referral to a specialized center. Intermittent remissions delay assessment of drug resistance for all definitions and 13% of patients enter a remission despite established drug resistance.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"51 1","pages":"39 - 44"},"PeriodicalIF":0.0,"publicationDate":"2015-04-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91302157","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}
Summary Introduction Neurostimulation and neuromodulation are techniques that may be able to affect the course of epilepsy. In the last 20 years, since the approval of VNS, we have observed a surge of studies assessing the potential of other devices and techniques for the treatment of pharmacoresistant epilepsies including deep brain stimulation (DBS), responsive neurostimulation (RNS), trigeminal nerve stimulation (TNS), transcranial direct current stimulation (tDCS), and repetitive transcranial magnetic stimulation (rTMS). Are these devices and techniques simply another treatment option that can be offered to patients with epilepsy or do they offer specific advantages when compared to the standard antiepileptic drugs (AEDs)? Aim The aim of this review is to present the neurostimulation and neuromodulation devices and techniques that are now in use, or at least available for testing and to discuss the science behind them, their applications, efficacy, potential risks vs. benefits and, above all, how to navigate the choices so clinicians are able to provide their patients with the best possible option for the treatment of epilepsy. Material and methods We analyzed PubMed and MEDLINE databases to select the most salient and recent (up to November 2014) publications on each treatment device. In addition to these searches bibliographies of selected articles were hand-searched for possible sources. Discussion and conclusions Great progress in neurostimulation and neuromodulation has been made over the last two decades with 2 devices (VNS, RNS) approved for the treatment of epilepsy in the US and three (DBS in addition to VNS and RNS) in Europe. The future of neuromodulation/neurostimulation is exciting – various studies and efforts are underway and will provide us with more data in the future. There appears to be one clear advantage of these treatments/devices over the AEDs that is consistently noted – routinely observed is continuous improvement in seizure control over time. This is something that the AEDs have thus far failed to deliver.
{"title":"Neurostimulation, neuromodulation, and the treatment of epilepsies","authors":"Lauren B. Bolden, S. Pati, J. Szaflarski","doi":"10.1515/JOEPI-2015-0022","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0022","url":null,"abstract":"Summary Introduction Neurostimulation and neuromodulation are techniques that may be able to affect the course of epilepsy. In the last 20 years, since the approval of VNS, we have observed a surge of studies assessing the potential of other devices and techniques for the treatment of pharmacoresistant epilepsies including deep brain stimulation (DBS), responsive neurostimulation (RNS), trigeminal nerve stimulation (TNS), transcranial direct current stimulation (tDCS), and repetitive transcranial magnetic stimulation (rTMS). Are these devices and techniques simply another treatment option that can be offered to patients with epilepsy or do they offer specific advantages when compared to the standard antiepileptic drugs (AEDs)? Aim The aim of this review is to present the neurostimulation and neuromodulation devices and techniques that are now in use, or at least available for testing and to discuss the science behind them, their applications, efficacy, potential risks vs. benefits and, above all, how to navigate the choices so clinicians are able to provide their patients with the best possible option for the treatment of epilepsy. Material and methods We analyzed PubMed and MEDLINE databases to select the most salient and recent (up to November 2014) publications on each treatment device. In addition to these searches bibliographies of selected articles were hand-searched for possible sources. Discussion and conclusions Great progress in neurostimulation and neuromodulation has been made over the last two decades with 2 devices (VNS, RNS) approved for the treatment of epilepsy in the US and three (DBS in addition to VNS and RNS) in Europe. The future of neuromodulation/neurostimulation is exciting – various studies and efforts are underway and will provide us with more data in the future. There appears to be one clear advantage of these treatments/devices over the AEDs that is consistently noted – routinely observed is continuous improvement in seizure control over time. This is something that the AEDs have thus far failed to deliver.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"27 1","pages":"45 - 59"},"PeriodicalIF":0.0,"publicationDate":"2015-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72976312","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}
Summary Introduction The traditional view of cognition in idiopathic or genetic generalized epilepsy (GGE) is that “one size fits all” i.e. only very mild generalized impairment might be detected, if any. This paper describes four case studies of cognitive functioning in GGE patients with photosensitivity and reflexive seizures. Aim The aim was to discover whether each individual’s set of cognitive deficits varied in accordance with his/her other clinical phenomena such as photosensitivity and kinds of reflexive seizures. Method Neurological and cognitive performance was assessed by comprehensive evaluation of each patient based on interviews, neurologist’s EEG reports and neuropsychological tests. Assessment of cognitive domains included estimated pre-morbid I.Q. based on reading ability and demographic norms, current I.Q., attention factors, verbal memory, visual memory and executive functions. Results Clinical signs and investigative studies indicated that two cases typically began reflexive seizure episodes with facial myoclonia which evolved into tonic-clonic convulsions or generalized myoclonic seizures. These patients had widespread attention and working memory deficits, some severe, together with lowered intelligence scores. In contrast, two other cases (with no history of myoclonus) had generalized reflexive seizures originating in the occipital lobes, very mild localized visual dysfunction and high intelligence. Conclusions The systematic variation in extent and nature of cognitive dysfunction illustrated in these cases with reflexive seizures (preceded by myoclonia or visual phenomena) would be explained by a more recent conceptualization of GGE as encompassing regional differences in variable hyperexcitability located at cortical levels or functional neural networks.
{"title":"Variations in cognitive functioning in genetic generalized epilepsy: four case studies","authors":"E. Ballini, E. Helmes, B. Schefft","doi":"10.1515/JOEPI-2015-0024","DOIUrl":"https://doi.org/10.1515/JOEPI-2015-0024","url":null,"abstract":"Summary Introduction The traditional view of cognition in idiopathic or genetic generalized epilepsy (GGE) is that “one size fits all” i.e. only very mild generalized impairment might be detected, if any. This paper describes four case studies of cognitive functioning in GGE patients with photosensitivity and reflexive seizures. Aim The aim was to discover whether each individual’s set of cognitive deficits varied in accordance with his/her other clinical phenomena such as photosensitivity and kinds of reflexive seizures. Method Neurological and cognitive performance was assessed by comprehensive evaluation of each patient based on interviews, neurologist’s EEG reports and neuropsychological tests. Assessment of cognitive domains included estimated pre-morbid I.Q. based on reading ability and demographic norms, current I.Q., attention factors, verbal memory, visual memory and executive functions. Results Clinical signs and investigative studies indicated that two cases typically began reflexive seizure episodes with facial myoclonia which evolved into tonic-clonic convulsions or generalized myoclonic seizures. These patients had widespread attention and working memory deficits, some severe, together with lowered intelligence scores. In contrast, two other cases (with no history of myoclonus) had generalized reflexive seizures originating in the occipital lobes, very mild localized visual dysfunction and high intelligence. Conclusions The systematic variation in extent and nature of cognitive dysfunction illustrated in these cases with reflexive seizures (preceded by myoclonia or visual phenomena) would be explained by a more recent conceptualization of GGE as encompassing regional differences in variable hyperexcitability located at cortical levels or functional neural networks.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"71 1","pages":"25 - 37"},"PeriodicalIF":0.0,"publicationDate":"2015-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81817436","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}
Pub Date : 2015-02-27DOI: 10.21307/joepi-2015-0023
A. Balogh, P. Halász, Dániel Fabó, L. Erőss
Summary Introduction The seizure propagation phenomenon by inducing remote symptoms brings several difficulties in finding the seizure onset and delineating the epileptic network which should be taken into consideration in epilepsy surgery. By demonstrating a difficult (MRI negative) epilepsy surgery case explored with invasive presurgical evaluation we highlight the importance to recognise the secondary sensory area and to explore the the parieto-opercular-insular-medial frontal network in certain cases. A further conclusion is the consideration of the redistributory role of the insula as a special structure in the cerebral connectome, having a role in epileptic network organisation. Aims To support the role of the insula in the organisation of an opercular – medial frontal epileptic network and to confirm Penfield’s the “second somatic sensory leg area” by way of a case report. We try to give an up to date exploration of our patient’s remote epileptic seizures by way of a connectome. Methods The epileptic disorder was studied with intensive video EEG monitoring and two times 3T MRI. Interictal FDG (fluorodeoxyglucose) PET was also undertaken. Beside the scalp EEG and computerized frequency analysis, the evaluation was performed by invasive EEG with 2 grids and 2 strips and an insular deep electrode in addition. Electrical cortical stimulation and cortical mapping were also undertaken. Results The video-EEG study revealed the complex seizure semiology. The left sided global somatosesensory aura in the leg, followed supplementary motor area manifestations represented a remote seizure. The seizure onset zone and the symptomatogenic zone were localised by the invasive electrophysiology. With the insular deep electrode we succeeded to explore the propagation of ictal activity to the insula and later to frontal medial surface. The PET, the negative 3T MRI results and the postprocessing morphometry confirmed the lesional origin and localised the epileptogenic area to the second somato-sensory field where a dysgenesis was located. Conclusions By preoperative invasive video-EEG evaluation, the second somato-sensory leg area was delineated as the seizure onset zone. The resection of this area by IIb type cortical dysgenesis, resulted in a complete relief of the seizures. The invasive video-EEG revealed the peculiar role of the insula in the propagation of the epileptic seizure from the second sensory leg area to the ipsilateral fronto-medial supplemetary motor area. Our results, confirm, that the insula has a relay or node function on the parietal opercular-fronto-medial epileptic network. The connectome of the insula is a further additive of the scale-free features of the remote epileptic networks.
{"title":"Epileptic seizure propagation from the second somatic sensory area to the fronto-medial region, by insular redistribution. A case report and a connectome description","authors":"A. Balogh, P. Halász, Dániel Fabó, L. Erőss","doi":"10.21307/joepi-2015-0023","DOIUrl":"https://doi.org/10.21307/joepi-2015-0023","url":null,"abstract":"Summary Introduction The seizure propagation phenomenon by inducing remote symptoms brings several difficulties in finding the seizure onset and delineating the epileptic network which should be taken into consideration in epilepsy surgery. By demonstrating a difficult (MRI negative) epilepsy surgery case explored with invasive presurgical evaluation we highlight the importance to recognise the secondary sensory area and to explore the the parieto-opercular-insular-medial frontal network in certain cases. A further conclusion is the consideration of the redistributory role of the insula as a special structure in the cerebral connectome, having a role in epileptic network organisation. Aims To support the role of the insula in the organisation of an opercular – medial frontal epileptic network and to confirm Penfield’s the “second somatic sensory leg area” by way of a case report. We try to give an up to date exploration of our patient’s remote epileptic seizures by way of a connectome. Methods The epileptic disorder was studied with intensive video EEG monitoring and two times 3T MRI. Interictal FDG (fluorodeoxyglucose) PET was also undertaken. Beside the scalp EEG and computerized frequency analysis, the evaluation was performed by invasive EEG with 2 grids and 2 strips and an insular deep electrode in addition. Electrical cortical stimulation and cortical mapping were also undertaken. Results The video-EEG study revealed the complex seizure semiology. The left sided global somatosesensory aura in the leg, followed supplementary motor area manifestations represented a remote seizure. The seizure onset zone and the symptomatogenic zone were localised by the invasive electrophysiology. With the insular deep electrode we succeeded to explore the propagation of ictal activity to the insula and later to frontal medial surface. The PET, the negative 3T MRI results and the postprocessing morphometry confirmed the lesional origin and localised the epileptogenic area to the second somato-sensory field where a dysgenesis was located. Conclusions By preoperative invasive video-EEG evaluation, the second somato-sensory leg area was delineated as the seizure onset zone. The resection of this area by IIb type cortical dysgenesis, resulted in a complete relief of the seizures. The invasive video-EEG revealed the peculiar role of the insula in the propagation of the epileptic seizure from the second sensory leg area to the ipsilateral fronto-medial supplemetary motor area. Our results, confirm, that the insula has a relay or node function on the parietal opercular-fronto-medial epileptic network. The connectome of the insula is a further additive of the scale-free features of the remote epileptic networks.","PeriodicalId":15683,"journal":{"name":"Journal of Epileptology","volume":"26 1","pages":"61 - 67"},"PeriodicalIF":0.0,"publicationDate":"2015-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73698989","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}
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