J. Kassiri, C. Elliott, Natarie Liu, K. Narvacan, Matt Wheatly, D. Sinclair
Stereoelectroencephalography (SEEG) is the preoperative assessment of choice when the epileptogenic zone (EZ) is unclear in patients requiring surgery for severe, drug-refractory epilepsy. There are relatively little data on the safety and efficacy of SEEG in the pediatric epilepsy population. We, therefore, investigated the insertional complications, rate of successful identification of the EZ, and long-term seizure outcomes following surgery after SEEG in children. This was a retrospective study of drug-resistant pediatric epilepsy patients treated with surgery between 2005 and 2020 and who underwent presurgical SEEG. Rationale for and coverage of SEEG, identification of the EZ, and ultimate seizure outcome following SEEG-tailored resections were collected and analyzed. Thirty patients (15 male, mean age: 12.4 ± 5 years) who underwent SEEG were studied. SEEG-related complications occurred in one case (3%). A total of 190 multicontact electrodes (mean: 7.0 ± 2.5 per patient) were implanted across 30 insertions capturing 440 electrographic seizures (mean: 17.5 ± 27.6 per patient). The most common rationale for SEEG was normal magnetic resonance imaging with surface EEG that failed to identify the EZ (17/30; 57%). SEEG identified a putative EZ in all cases, resulting in SEEG-tailored resections in 25/30 (83%). Freedom from disabling seizures was achieved following resections in 20/25 cases (80%) with 5.9 ± 4.0 years of postoperative follow-up. SEEG is a safe and effective way to identify the EZ in the presurgical evaluation of children with refractory epilepsy and permits effective and long-lasting SEEG-tailored resections.
{"title":"Safety and Efficacy of Stereoelectroencephalography in Pediatric Epilepsy Surgery","authors":"J. Kassiri, C. Elliott, Natarie Liu, K. Narvacan, Matt Wheatly, D. Sinclair","doi":"10.1055/s-0042-1743192","DOIUrl":"https://doi.org/10.1055/s-0042-1743192","url":null,"abstract":"Stereoelectroencephalography (SEEG) is the preoperative assessment of choice when the epileptogenic zone (EZ) is unclear in patients requiring surgery for severe, drug-refractory epilepsy. There are relatively little data on the safety and efficacy of SEEG in the pediatric epilepsy population. We, therefore, investigated the insertional complications, rate of successful identification of the EZ, and long-term seizure outcomes following surgery after SEEG in children. This was a retrospective study of drug-resistant pediatric epilepsy patients treated with surgery between 2005 and 2020 and who underwent presurgical SEEG. Rationale for and coverage of SEEG, identification of the EZ, and ultimate seizure outcome following SEEG-tailored resections were collected and analyzed. Thirty patients (15 male, mean age: 12.4 ± 5 years) who underwent SEEG were studied. SEEG-related complications occurred in one case (3%). A total of 190 multicontact electrodes (mean: 7.0 ± 2.5 per patient) were implanted across 30 insertions capturing 440 electrographic seizures (mean: 17.5 ± 27.6 per patient). The most common rationale for SEEG was normal magnetic resonance imaging with surface EEG that failed to identify the EZ (17/30; 57%). SEEG identified a putative EZ in all cases, resulting in SEEG-tailored resections in 25/30 (83%). Freedom from disabling seizures was achieved following resections in 20/25 cases (80%) with 5.9 ± 4.0 years of postoperative follow-up. SEEG is a safe and effective way to identify the EZ in the presurgical evaluation of children with refractory epilepsy and permits effective and long-lasting SEEG-tailored resections.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":null,"pages":null},"PeriodicalIF":0.2,"publicationDate":"2022-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80694101","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. Almojuela, Q. Xu, Aoife O’Carroll, C. MacDonald, L. Ritchie, D. Serletis
Background A Pediatric Epilepsy Program was instituted in Manitoba in 2016. This report seeks to describe changes in the management of pediatric epilepsy patients in Manitoba since the inception of this Program, to provide an early analysis of local outcomes, and to present a framework for further program development. Methods Data was collected for patients treated both before and after inception of the Program. Caregivers completed questionnaires on quality of life and program satisfaction. An online database was created to capture demographic information, seizure and quality of life outcomes, and caregiver satisfaction ratings. Descriptive statistics were used to summarize the results. Results Prior to commencement of the Program, 16 patients underwent vagal nerve stimulator (VNS) insertion. At last follow-up, 6.25% of patients achieved Engel class I outcome, 75% achieved class III outcome, and 18.75% were classified as class IV. Following inception of the Program, 11 patients underwent resective procedures and 3 underwent VNS insertions. At last follow-up, 78.6% of patients achieved Engel class I outcome, 14.3% achieved class III outcome, and 7.1% were classified as class IV. Since inception of the Program, the average Quality of Life in Childhood Epilepsy Questionnaire-55 score measuring patient quality of life was (59.7 ± 23.2)/100. The average Care-Related Quality of Life-7D score measuring caregiver quality of life was (78.3 ± 18.6)/100. Caregiver satisfaction had an average rating of (9.4 ± 0.8)/10. Conclusion Access to epilepsy surgery has significantly improved for children in Manitoba and has led to favorable, early multidimensional outcomes. Structural organization, funding, and multidisciplinary engagement are necessary for program sustainability and growth.
{"title":"Development of a Pediatric Epilepsy Program: Analysis of Early Multidimensional Outcomes","authors":"A. Almojuela, Q. Xu, Aoife O’Carroll, C. MacDonald, L. Ritchie, D. Serletis","doi":"10.1055/s-0042-1742607","DOIUrl":"https://doi.org/10.1055/s-0042-1742607","url":null,"abstract":"\u0000 Background A Pediatric Epilepsy Program was instituted in Manitoba in 2016. This report seeks to describe changes in the management of pediatric epilepsy patients in Manitoba since the inception of this Program, to provide an early analysis of local outcomes, and to present a framework for further program development.\u0000 Methods Data was collected for patients treated both before and after inception of the Program. Caregivers completed questionnaires on quality of life and program satisfaction. An online database was created to capture demographic information, seizure and quality of life outcomes, and caregiver satisfaction ratings. Descriptive statistics were used to summarize the results.\u0000 Results Prior to commencement of the Program, 16 patients underwent vagal nerve stimulator (VNS) insertion. At last follow-up, 6.25% of patients achieved Engel class I outcome, 75% achieved class III outcome, and 18.75% were classified as class IV. Following inception of the Program, 11 patients underwent resective procedures and 3 underwent VNS insertions. At last follow-up, 78.6% of patients achieved Engel class I outcome, 14.3% achieved class III outcome, and 7.1% were classified as class IV. Since inception of the Program, the average Quality of Life in Childhood Epilepsy Questionnaire-55 score measuring patient quality of life was (59.7 ± 23.2)/100. The average Care-Related Quality of Life-7D score measuring caregiver quality of life was (78.3 ± 18.6)/100. Caregiver satisfaction had an average rating of (9.4 ± 0.8)/10.\u0000 Conclusion Access to epilepsy surgery has significantly improved for children in Manitoba and has led to favorable, early multidimensional outcomes. Structural organization, funding, and multidisciplinary engagement are necessary for program sustainability and growth.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":null,"pages":null},"PeriodicalIF":0.2,"publicationDate":"2022-02-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85274021","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}
Fabiana Di Stasio, Martha Caterina Faraguna, Santo Di Marco, Viola Crescitelli, M. Iascone, Santa Florio, C. Peruzzi, S. Gasperini
Asparagine synthetase (ASNS) deficiency is a rare inborn error of metabolism caused by a defect in ASNS—a gene encoding asparagine synthetase. It has mainly been described as a neurological phenotype manifesting as severe developmental delay, congenital microcephaly, spasticity, and refractory seizures; it is not associated with any specific dysmorphisms. ASNS deficiency leads to the inability to synthesize a nonessential amino acid in the brain, this explains why the symptoms are primarily neurological. The accumulation of aspartate/glutamate causes increased neuronal apoptosis leading to brain atrophy and increased neuronal excitability leading to seizures. Asparagine levels in plasma and cerebrospinal fluid are not reliable biomarkers for this disorder, therefore diagnosis is mainly obtained by molecular genetics. This disorder is associated with a poor prognosis and there is no treatment except supportive therapy. Prenatal diagnosis is possible. We report a case of a later onset form, c.146G > A (p.Arg49Gln) variant in the ASNS gene detected by molecular analysis using next-generation sequencing; the patient's clinical presentation included microcephaly, regression of developmental milestones, epilepsy, and hyperthermia.
天冬酰胺合成酶(ASNS)缺乏症是由编码天冬酰胺合成酶的基因ASNS缺陷引起的一种罕见的先天性代谢错误。它主要被描述为一种神经表型，表现为严重的发育迟缓、先天性小头畸形、痉挛和难治性癫痫发作;它不与任何特定的畸形相关联。ASNS缺陷导致大脑无法合成一种非必需氨基酸，这就解释了为什么症状主要是神经系统的。天冬氨酸/谷氨酸的积累导致神经元凋亡增加，导致脑萎缩和神经元兴奋性增加，导致癫痫发作。血浆和脑脊液中的天冬酰胺水平不是这种疾病的可靠生物标志物，因此诊断主要通过分子遗传学获得。这种疾病与预后不良有关，除了支持治疗外没有其他治疗方法。产前诊断是可能的。我们报告了一例发病较晚的ASNS基因c.146G > a (p.a g49gln)变异，通过使用下一代测序进行分子分析检测;患者的临床表现包括小头畸形、发育里程碑倒退、癫痫和高热。
{"title":"A Neuro-metabolic Syndrome that Needs to Be Discovered: A Child with Late Onset Asparagine Synthetase Deficiency","authors":"Fabiana Di Stasio, Martha Caterina Faraguna, Santo Di Marco, Viola Crescitelli, M. Iascone, Santa Florio, C. Peruzzi, S. Gasperini","doi":"10.1055/s-0041-1739488","DOIUrl":"https://doi.org/10.1055/s-0041-1739488","url":null,"abstract":"Asparagine synthetase (ASNS) deficiency is a rare inborn error of metabolism caused by a defect in ASNS—a gene encoding asparagine synthetase. It has mainly been described as a neurological phenotype manifesting as severe developmental delay, congenital microcephaly, spasticity, and refractory seizures; it is not associated with any specific dysmorphisms. ASNS deficiency leads to the inability to synthesize a nonessential amino acid in the brain, this explains why the symptoms are primarily neurological. The accumulation of aspartate/glutamate causes increased neuronal apoptosis leading to brain atrophy and increased neuronal excitability leading to seizures. Asparagine levels in plasma and cerebrospinal fluid are not reliable biomarkers for this disorder, therefore diagnosis is mainly obtained by molecular genetics. This disorder is associated with a poor prognosis and there is no treatment except supportive therapy. Prenatal diagnosis is possible. We report a case of a later onset form, c.146G > A (p.Arg49Gln) variant in the ASNS gene detected by molecular analysis using next-generation sequencing; the patient's clinical presentation included microcephaly, regression of developmental milestones, epilepsy, and hyperthermia.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":null,"pages":null},"PeriodicalIF":0.2,"publicationDate":"2021-12-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89097447","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}
B. Mehra, R. Sabharwal, A. Sachdev, Praveen Kumar, R. Mehta, Neeraj Gupta
Abstract The treatment of super-refractory status epilepticus (SRSE) and prolonged SRSE rests on urgent seizure control to minimize excitotoxic cerebral damage, other forms of neurologic damage, and multiple medical complications. To date no randomized controlled trials or clear-cut guidelines are available for the management of SRSE. We reported the case of a 10-year-old previously healthy male child patient who presented with a febrile illness and new onset prolonged SRSE that became refractory to multiple antiseizure medications (ASMs). Coma induction with anesthetic agents, 14 ASMs, ketogenic diet, immunotherapy failed to completely control the SRSE in our patient. On day 22, clinical and electroencephalographic seizure control was achieved with isoflurane inhalation anesthesia, which was continued for 3 weeks but was unable to be weaned. From day 57 onwards, electroconvulsive therapy was administered (total 14 sessions that resulted in complete control of seizures). He was discharged on the 80th day.
{"title":"Successful Use of Inhalational Anesthesia and Electroconvulsive Therapy in a Child with New Onset Prolonged Super-Refractory Status Epilepticus","authors":"B. Mehra, R. Sabharwal, A. Sachdev, Praveen Kumar, R. Mehta, Neeraj Gupta","doi":"10.1055/s-0041-1740112","DOIUrl":"https://doi.org/10.1055/s-0041-1740112","url":null,"abstract":"Abstract The treatment of super-refractory status epilepticus (SRSE) and prolonged SRSE rests on urgent seizure control to minimize excitotoxic cerebral damage, other forms of neurologic damage, and multiple medical complications. To date no randomized controlled trials or clear-cut guidelines are available for the management of SRSE. We reported the case of a 10-year-old previously healthy male child patient who presented with a febrile illness and new onset prolonged SRSE that became refractory to multiple antiseizure medications (ASMs). Coma induction with anesthetic agents, 14 ASMs, ketogenic diet, immunotherapy failed to completely control the SRSE in our patient. On day 22, clinical and electroencephalographic seizure control was achieved with isoflurane inhalation anesthesia, which was continued for 3 weeks but was unable to be weaned. From day 57 onwards, electroconvulsive therapy was administered (total 14 sessions that resulted in complete control of seizures). He was discharged on the 80th day.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":null,"pages":null},"PeriodicalIF":0.2,"publicationDate":"2021-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86164046","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}
Abstract We aimed to evaluate the contribution of simultaneous electrocardiography (ECG) recording during routine interictal electroencephalography (EEG) recording in patients with seizures or epilepsy and therefore to provide evidence-based data on this subject. Patients with interictal cardiac arrhythmia on routine EEG-ECG recordings were determined and evaluated based on cardiologic and neurologic findings. Out of 1,078 patients aged between 5 and 16 years (mean: 10.2 ± 3.2), 9 (0.08%) patients were found to have an arrhythmia. Six patients had both epilepsy and cardiac arrhythmia (premature ventricular contractions [PVCs] in 5; Wolff-Parkinson-White [WPW] in 1 patient) and the remaining three patients had nonepileptic paroxysmal events (NPEs) and arrhythmia (PVC in 2; WPW in 1). Three patients had other diseases (neurofibromatosis type 1, tuberous sclerosis, and congenital heart disease status postsurgery). Cardiac arrhythmia required radiofrequency ablation or antiarrhythmic drug treatment in two patients with epilepsy and also two patients with NPE; however, it improved with no specific treatment in the remaining five patients. NPE was not related to arrhythmia in one of three patients with NPE. Our study suggests that routine interictal EEG-ECG recording provides a valuable and feasible opportunity to reveal unnoticed or new-onset cardiac arrhythmias. Therefore, ECG should be recorded simultaneously during routine interictal EEG recordings. Cardiac arrhythmias detected by routine interictal EEG-ECG recordings would require arrhythmia treatment in nearly half of the patients.
{"title":"Routine Interictal EEG Recording Should be Performed Together with Simultaneous Two-Lead ECG Recording","authors":"K. Yılmaz, S. Işıkay, Sibel Yavuz, O. Başpınar","doi":"10.1055/s-0042-1751247","DOIUrl":"https://doi.org/10.1055/s-0042-1751247","url":null,"abstract":"Abstract We aimed to evaluate the contribution of simultaneous electrocardiography (ECG) recording during routine interictal electroencephalography (EEG) recording in patients with seizures or epilepsy and therefore to provide evidence-based data on this subject. Patients with interictal cardiac arrhythmia on routine EEG-ECG recordings were determined and evaluated based on cardiologic and neurologic findings. Out of 1,078 patients aged between 5 and 16 years (mean: 10.2 ± 3.2), 9 (0.08%) patients were found to have an arrhythmia. Six patients had both epilepsy and cardiac arrhythmia (premature ventricular contractions [PVCs] in 5; Wolff-Parkinson-White [WPW] in 1 patient) and the remaining three patients had nonepileptic paroxysmal events (NPEs) and arrhythmia (PVC in 2; WPW in 1). Three patients had other diseases (neurofibromatosis type 1, tuberous sclerosis, and congenital heart disease status postsurgery). Cardiac arrhythmia required radiofrequency ablation or antiarrhythmic drug treatment in two patients with epilepsy and also two patients with NPE; however, it improved with no specific treatment in the remaining five patients. NPE was not related to arrhythmia in one of three patients with NPE. Our study suggests that routine interictal EEG-ECG recording provides a valuable and feasible opportunity to reveal unnoticed or new-onset cardiac arrhythmias. Therefore, ECG should be recorded simultaneously during routine interictal EEG recordings. Cardiac arrhythmias detected by routine interictal EEG-ECG recordings would require arrhythmia treatment in nearly half of the patients.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":null,"pages":null},"PeriodicalIF":0.2,"publicationDate":"2021-11-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85189660","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}
Goichiro Tamura, W. Lo, I. Yau, Kerry A. Vaughan, C. Go, W. Singleton, David Hazon, Han Yan, H. Otsubo, E. Donner, J. Rutka, G. Ibrahim
Abstract Clinical responses to vagus nerve stimulation (VNS) therapy for intractable epilepsy can be unpredictable, and factors that predict response to therapy are elusive. Minority of children undergoing VNS achieve seizure freedom. The current study aimed to characterize this exceptional patient population, defined as “super-responders” (SRs). Retrospective data were collected from 150 children who underwent VNS at a single pediatric institution. The patients' mean age at VNS device implantation was 12.0 years (range, 3.09–17.9 years). Ten SRs (6.7%) were identified who achieved and maintained seizure freedom for longer than 1 year following implantation. The interval between epilepsy onset and VNS device implantation was significantly shorter in SRs than in the other children (mean epilepsy duration 5.72 vs. 8.44 years, respectively; p = 0.032). SRs also had a significantly shorter proportion of life with epilepsy compared with the other children (mean ratio of epilepsy duration to age at implantation 0.52 vs. 0.71, respectively; p = 0.023). SRs reported their seizure freedom relatively early (six patients within 6 months and all patients within 12 months after implantation) at relatively low device settings (mean output current 0.81 mA at their last follow-up). Compared with conventional models, responsive VNS models with autostimulation features did not increase the ratio of SRs. No other clinical or imaging characteristic difference between SRs and the other children was found in this cohort. The current study showed a significant association between shorter epilepsy duration and shorter proportion of life with epilepsy and seizure freedom after VNS.
{"title":"Patient Characteristics Associated with Seizure Freedom after Vagus Nerve Stimulation in Pediatric Intractable Epilepsy: An Analysis of “Super-Responders”","authors":"Goichiro Tamura, W. Lo, I. Yau, Kerry A. Vaughan, C. Go, W. Singleton, David Hazon, Han Yan, H. Otsubo, E. Donner, J. Rutka, G. Ibrahim","doi":"10.1055/s-0041-1739489","DOIUrl":"https://doi.org/10.1055/s-0041-1739489","url":null,"abstract":"Abstract Clinical responses to vagus nerve stimulation (VNS) therapy for intractable epilepsy can be unpredictable, and factors that predict response to therapy are elusive. Minority of children undergoing VNS achieve seizure freedom. The current study aimed to characterize this exceptional patient population, defined as “super-responders” (SRs). Retrospective data were collected from 150 children who underwent VNS at a single pediatric institution. The patients' mean age at VNS device implantation was 12.0 years (range, 3.09–17.9 years). Ten SRs (6.7%) were identified who achieved and maintained seizure freedom for longer than 1 year following implantation. The interval between epilepsy onset and VNS device implantation was significantly shorter in SRs than in the other children (mean epilepsy duration 5.72 vs. 8.44 years, respectively; p = 0.032). SRs also had a significantly shorter proportion of life with epilepsy compared with the other children (mean ratio of epilepsy duration to age at implantation 0.52 vs. 0.71, respectively; p = 0.023). SRs reported their seizure freedom relatively early (six patients within 6 months and all patients within 12 months after implantation) at relatively low device settings (mean output current 0.81 mA at their last follow-up). Compared with conventional models, responsive VNS models with autostimulation features did not increase the ratio of SRs. No other clinical or imaging characteristic difference between SRs and the other children was found in this cohort. The current study showed a significant association between shorter epilepsy duration and shorter proportion of life with epilepsy and seizure freedom after VNS.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":null,"pages":null},"PeriodicalIF":0.2,"publicationDate":"2021-11-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80889213","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}
Abstract It has been known for several decades that epilepsy and autism spectrum disorders (ASD) are related to each other. Epilepsy frequently accompanies ASD. The purpose of this study was to investigate relationship between clinical and electroencephalogram (EEG) findings in ASD patients and to identify EEG characteristics that may create a disposition to epilepsy in ASD by examining differences in clinical and EEG findings between patients diagnosed with ASD without epilepsy and ASD with epilepsy. A total of 102 patients aged 2 to 18 years and diagnosed with ASD based on Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) diagnostic criteria between January 2017 and June 2019 were included in the study. Patients were assigned into two groups: (1) ASD with epilepsy and (2) ASD without epilepsy. Clinical findings were retrieved from patients' files, and EEG findings from first EEG records in the EEG laboratory at the time of diagnosis. EEG findings were defined as central, parietal, frontal, temporal, or generalized, depending on the location of rhythmic discharges. The incidence of epilepsy in our ASD patients was 33.7% and that of febrile convulsion was 4%. Generalized motor seizures were the most common seizure type. Epileptic discharges most commonly derived from the central and frontal regions. These abnormalities, especially frontal and central rhythmic discharges, may represent a precursor for the development of epilepsy in ASD patients.
{"title":"Electroencephalogram Abnormalities and Epilepsy in Autism Spectrum Disorders: Clinical and Electroencephalogram Findings","authors":"Fatma Hancı, Sevim Türay, Y. Öztürk, N. Kabakuş","doi":"10.1055/s-0041-1736557","DOIUrl":"https://doi.org/10.1055/s-0041-1736557","url":null,"abstract":"Abstract It has been known for several decades that epilepsy and autism spectrum disorders (ASD) are related to each other. Epilepsy frequently accompanies ASD. The purpose of this study was to investigate relationship between clinical and electroencephalogram (EEG) findings in ASD patients and to identify EEG characteristics that may create a disposition to epilepsy in ASD by examining differences in clinical and EEG findings between patients diagnosed with ASD without epilepsy and ASD with epilepsy. A total of 102 patients aged 2 to 18 years and diagnosed with ASD based on Diagnostic and Statistical Manual of Mental Disorders, fifth edition (DSM-5) diagnostic criteria between January 2017 and June 2019 were included in the study. Patients were assigned into two groups: (1) ASD with epilepsy and (2) ASD without epilepsy. Clinical findings were retrieved from patients' files, and EEG findings from first EEG records in the EEG laboratory at the time of diagnosis. EEG findings were defined as central, parietal, frontal, temporal, or generalized, depending on the location of rhythmic discharges. The incidence of epilepsy in our ASD patients was 33.7% and that of febrile convulsion was 4%. Generalized motor seizures were the most common seizure type. Epileptic discharges most commonly derived from the central and frontal regions. These abnormalities, especially frontal and central rhythmic discharges, may represent a precursor for the development of epilepsy in ASD patients.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":null,"pages":null},"PeriodicalIF":0.2,"publicationDate":"2021-10-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72448293","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}
K. Kchaou, I. Kammoun, S. Chakroun, A. Haddar, K. Masmoudi
Abstract The objective of this study was to identify clinical parameters predicting either a pathological EEG or a subsequent epileptic seizure (SES), based on the relation between paroxysmal EEG abnormalities and clinical features in children who presented at least one febrile seizure (FS). We collected data of children who presented to our department during the period 2013 to 2018 for EEG recording as part of their febrile seizure assessment. Only children aged between 1 month to 5 years were included. Both the clinical and EEG data were retrospectively collected and statistically studied. We performed a detailed analysis of the EEG recordings. SES was identified for patients with sufficient follow-up. A total of 120 children were included in the study, of whom 48% had EEG abnormalities. Psychomotor retardation (p = 0.002), completion of an EEG within 7 days of the last FS (p = 0.046), and late age (> 3 years) of the first FS onset (p = 0.021) were significantly associated with a pathological EEG. In multivariate analysis, performing early EEG (< 7 days from the last FS) (odds ratio [OR]: 2.35; p = 0.043; confidence interval [CI]: 1.028–5.375) and psychomotor retardation (OR: 4.19; p = 0.008; CI: 1.46–12) were independent predictors of a pathological EEG. Of 120 patients, 45 had a follow-up. However, only 10 (22.22%) had SES. Children with SES tended more to have a psychomotor delay, compared with children without SES (50% vs. 14.28%, p = 0.029). Moreover, the percentage of initial abnormal EEG in patients with SES was significantly higher than those without SES (70% vs. 34.28%, p = 0.05). Even though some FS characteristics predict EEG abnormalities, they are not always associated with SES. We highlight the importance of performing an EEG in the group of children who had both FS and psychomotor retardation. This is most likely the group at the highest risk of developing epilepsy.
摘要:本研究的目的是根据至少出现一次发热性癫痫发作(FS)的儿童阵发性脑电图异常与临床特征之间的关系，确定预测病理性脑电图或随后癫痫发作(SES)的临床参数。我们收集了2013年至2018年期间到我科就诊的儿童的数据，用于脑电图记录，作为其热性癫痫发作评估的一部分。仅包括1个月至5岁的儿童。回顾性收集临床和脑电图资料并进行统计学分析。我们对脑电图记录进行了详细分析。对随访充分的患者进行SES鉴定。本研究共纳入120名儿童，其中48%存在脑电图异常。精神运动迟缓(p = 0.002)、最后一次FS发生后7天内完成脑电图(p = 0.046)和首次FS发病的年龄较晚(p = 0.021)与病理性脑电图显著相关。在多变量分析中，早期EEG(距离最后一次FS < 7天)(优势比[OR]: 2.35;p = 0.043;置信区间[CI]: 1.028-5.375)和精神运动迟缓(OR: 4.19;p = 0.008;CI: 1.46-12)是病理性脑电图的独立预测因子。在120名患者中，有45人进行了随访。然而，只有10人(22.22%)患有SES。与非SES儿童相比，SES儿童更倾向于出现精神运动延迟(50% vs. 14.28%， p = 0.029)。有SES的患者初始脑电图异常比例显著高于无SES的患者(70% vs. 34.28%， p = 0.05)。尽管一些FS特征可以预测脑电图异常，但它们并不总是与SES相关。我们强调在患有FS和精神运动迟缓的儿童中进行脑电图的重要性。这很可能是患癫痫风险最高的人群。
{"title":"Clinical Predictive Factors of Pathological EEG in Children with Febrile Seizures and Their Association with Subsequent Epileptic Seizures","authors":"K. Kchaou, I. Kammoun, S. Chakroun, A. Haddar, K. Masmoudi","doi":"10.1055/s-0041-1736214","DOIUrl":"https://doi.org/10.1055/s-0041-1736214","url":null,"abstract":"Abstract The objective of this study was to identify clinical parameters predicting either a pathological EEG or a subsequent epileptic seizure (SES), based on the relation between paroxysmal EEG abnormalities and clinical features in children who presented at least one febrile seizure (FS). We collected data of children who presented to our department during the period 2013 to 2018 for EEG recording as part of their febrile seizure assessment. Only children aged between 1 month to 5 years were included. Both the clinical and EEG data were retrospectively collected and statistically studied. We performed a detailed analysis of the EEG recordings. SES was identified for patients with sufficient follow-up. A total of 120 children were included in the study, of whom 48% had EEG abnormalities. Psychomotor retardation (p = 0.002), completion of an EEG within 7 days of the last FS (p = 0.046), and late age (> 3 years) of the first FS onset (p = 0.021) were significantly associated with a pathological EEG. In multivariate analysis, performing early EEG (< 7 days from the last FS) (odds ratio [OR]: 2.35; p = 0.043; confidence interval [CI]: 1.028–5.375) and psychomotor retardation (OR: 4.19; p = 0.008; CI: 1.46–12) were independent predictors of a pathological EEG. Of 120 patients, 45 had a follow-up. However, only 10 (22.22%) had SES. Children with SES tended more to have a psychomotor delay, compared with children without SES (50% vs. 14.28%, p = 0.029). Moreover, the percentage of initial abnormal EEG in patients with SES was significantly higher than those without SES (70% vs. 34.28%, p = 0.05). Even though some FS characteristics predict EEG abnormalities, they are not always associated with SES. We highlight the importance of performing an EEG in the group of children who had both FS and psychomotor retardation. This is most likely the group at the highest risk of developing epilepsy.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":null,"pages":null},"PeriodicalIF":0.2,"publicationDate":"2021-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"76945787","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}
Abstract Febrile seizure (FS) is the most common convulsive disorder in children with FS prevalence in Indonesia reaching 2 to 4% in 2008. Although this entity has a good prognosis, it often brings panic, fear, and anxiety to the parents. This seemingly benign condition might lead to changes in family structures resulting in adverse effects on the family's daily lives and affect their overall quality of life (QoL). This study evaluates the QoL of parents whose children have FS. A cross-sectional study done in 47 parents whose children had a FS between ages 1 and 4 years from January 2020 to May 2020 and who were evaluated at the Siloam General Hospital, Lippo Village. Parents were asked to fill in Pediatric Quality of Life Questionnaire parent proxy. Data normality was analyzed using the Shapiro–Wilk's test and the significant impact of parents' QoL using the chi-square and independent t-tests. From a total of 47 parents, 30 (63.8%) parents had children with simple FS and 17 (36.2%) parents had children with complex FS. Parents whose children were in the age group of 1 year to 1 year 11 months had the best mean score of 79.64 (12.17) compared with other age groups. In the subset of 3 to 4 years old, the daily activities domain was significantly affected (p-value = 0.3). Parents with a lower educational level had a higher mean score of 76.53 (14.42) than parents who had a higher educational level, with a total mean of 79.88 (11.85), particularly with the highest mean score of 100 in the communication domain. The occurrence of FSs in children affected their parents' QoL in almost all domains in the Pediatric Quality of Life Inventory questionnaire.
{"title":"Assessment of Parental Quality of Life in Febrile Seizures Using Pediatric Quality of Live Inventory Parental Report","authors":"G. Octavius, Cindy Virliani, A. Juliansen","doi":"10.1055/s-0041-1733954","DOIUrl":"https://doi.org/10.1055/s-0041-1733954","url":null,"abstract":"Abstract Febrile seizure (FS) is the most common convulsive disorder in children with FS prevalence in Indonesia reaching 2 to 4% in 2008. Although this entity has a good prognosis, it often brings panic, fear, and anxiety to the parents. This seemingly benign condition might lead to changes in family structures resulting in adverse effects on the family's daily lives and affect their overall quality of life (QoL). This study evaluates the QoL of parents whose children have FS. A cross-sectional study done in 47 parents whose children had a FS between ages 1 and 4 years from January 2020 to May 2020 and who were evaluated at the Siloam General Hospital, Lippo Village. Parents were asked to fill in Pediatric Quality of Life Questionnaire parent proxy. Data normality was analyzed using the Shapiro–Wilk's test and the significant impact of parents' QoL using the chi-square and independent t-tests. From a total of 47 parents, 30 (63.8%) parents had children with simple FS and 17 (36.2%) parents had children with complex FS. Parents whose children were in the age group of 1 year to 1 year 11 months had the best mean score of 79.64 (12.17) compared with other age groups. In the subset of 3 to 4 years old, the daily activities domain was significantly affected (p-value = 0.3). Parents with a lower educational level had a higher mean score of 76.53 (14.42) than parents who had a higher educational level, with a total mean of 79.88 (11.85), particularly with the highest mean score of 100 in the communication domain. The occurrence of FSs in children affected their parents' QoL in almost all domains in the Pediatric Quality of Life Inventory questionnaire.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":null,"pages":null},"PeriodicalIF":0.2,"publicationDate":"2021-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90271117","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}
Abstract Although clinical judgement and sedation scales are primarily used in intensive care units (ICUs) to manage sedation, adjunctive data are needed to direct therapy with sedative and hypnotic agents to prevent side effects and long-term sequelae. In this report, we describe three cases where we used quantitative electroencephalogram (qEEG) data in a pediatric ICU (PICU); to manage these specific clinical situations and to identify the limitations of the qEEG data, two patients were admitted for post–cardiac arrest care and the third was admitted for status epilepticus. In post–cardiac arrest patients, qEEG was mainly used for monitoring depth of sedation and drug titration. Unnecessary use of high-drug doses was prevented, and monitoring also helped to guide clinical intervention for the management of seizure activity. In the patient with status epilepticus, qEEG data on burst suppression and depth of sedation were used. In this report, we describe three different cases where we used qEEG data in a PICU, to give insight on the use of data in specific clinical situations and to describe the limitations of the qEEG data monitoring system.
{"title":"Quantitative Electroencephalogram in Pediatric Intensive Care Unit in Three Different Clinical Scenarios","authors":"A. Yetimakman, E. Kıral","doi":"10.1055/s-0041-1733858","DOIUrl":"https://doi.org/10.1055/s-0041-1733858","url":null,"abstract":"Abstract Although clinical judgement and sedation scales are primarily used in intensive care units (ICUs) to manage sedation, adjunctive data are needed to direct therapy with sedative and hypnotic agents to prevent side effects and long-term sequelae. In this report, we describe three cases where we used quantitative electroencephalogram (qEEG) data in a pediatric ICU (PICU); to manage these specific clinical situations and to identify the limitations of the qEEG data, two patients were admitted for post–cardiac arrest care and the third was admitted for status epilepticus. In post–cardiac arrest patients, qEEG was mainly used for monitoring depth of sedation and drug titration. Unnecessary use of high-drug doses was prevented, and monitoring also helped to guide clinical intervention for the management of seizure activity. In the patient with status epilepticus, qEEG data on burst suppression and depth of sedation were used. In this report, we describe three different cases where we used qEEG data in a PICU, to give insight on the use of data in specific clinical situations and to describe the limitations of the qEEG data monitoring system.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":null,"pages":null},"PeriodicalIF":0.2,"publicationDate":"2021-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74969195","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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