Abstract Children with chronic care or complex medical needs, such as epilepsy, depend on advocates to ensure their safety and well-being, a role typically filled by parents or extended family. Close participation to provide vital history and details is quintessential to help guide appropriate management decisions to ensure optimal neurodevelopmental outcome. However, when these vulnerable children are placed in the foster care system, their need for an advocate becomes even more paramount. Unfortunately, this can be lacking and can lead to a breakdown in their medical care. Their complex medical conditions often can be intimidating to most potential foster families and may result in placement in long-term care facilities. This, in turn, presents additional obstacles that can hinder optimal care such as lack of consistent, dedicated caretakers. This void not only impacts their outcome and medical care but also leads to excessive use of limited healthcare resources as well as unwanted adverse reactions that can be prevented if a reliable source of history is available. As a result, there is a need for dedicated resources to help provide devoted advocates for these vulnerable children.
{"title":"Quality of Life Dilemmas in Pediatric Patients with Epilepsy Residing in Long-Term Care Facilities","authors":"L. Tran","doi":"10.1055/s-0040-1714356","DOIUrl":"https://doi.org/10.1055/s-0040-1714356","url":null,"abstract":"Abstract Children with chronic care or complex medical needs, such as epilepsy, depend on advocates to ensure their safety and well-being, a role typically filled by parents or extended family. Close participation to provide vital history and details is quintessential to help guide appropriate management decisions to ensure optimal neurodevelopmental outcome. However, when these vulnerable children are placed in the foster care system, their need for an advocate becomes even more paramount. Unfortunately, this can be lacking and can lead to a breakdown in their medical care. Their complex medical conditions often can be intimidating to most potential foster families and may result in placement in long-term care facilities. This, in turn, presents additional obstacles that can hinder optimal care such as lack of consistent, dedicated caretakers. This void not only impacts their outcome and medical care but also leads to excessive use of limited healthcare resources as well as unwanted adverse reactions that can be prevented if a reliable source of history is available. As a result, there is a need for dedicated resources to help provide devoted advocates for these vulnerable children.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":"1 1","pages":"083 - 084"},"PeriodicalIF":0.2,"publicationDate":"2020-07-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90868554","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 Abnormal movements are not uncommon in children. Most of these abnormal movements can be defined by the available criteria. Abnormal movements are easily diagnosed in adults. Most of these abnormal movements are well categorized and diagnosed by the defined characteristics. Sometimes it is difficult to diagnose an unusual movement disorder in children, particularly if it is rare and there are no investigations to prove the diagnosis. Repetitive arm and hand movements (complex motor stereotypy) is one such disorder which is rare and difficult to diagnose unless seen before. In this case report, we described a 6-year-old boy with this disorder, in whom it took 6 years to diagnose.
{"title":"Repetitive Arm and Hand Movements (Complex Motor Stereotypy) in a Six-Year Boy","authors":"R. Koul, R. Hora","doi":"10.1055/s-0040-1712162","DOIUrl":"https://doi.org/10.1055/s-0040-1712162","url":null,"abstract":"Abstract Abnormal movements are not uncommon in children. Most of these abnormal movements can be defined by the available criteria. Abnormal movements are easily diagnosed in adults. Most of these abnormal movements are well categorized and diagnosed by the defined characteristics. Sometimes it is difficult to diagnose an unusual movement disorder in children, particularly if it is rare and there are no investigations to prove the diagnosis. Repetitive arm and hand movements (complex motor stereotypy) is one such disorder which is rare and difficult to diagnose unless seen before. In this case report, we described a 6-year-old boy with this disorder, in whom it took 6 years to diagnose.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":"3 1","pages":"035 - 036"},"PeriodicalIF":0.2,"publicationDate":"2020-07-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81298023","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 is not uncommon for pediatric neurologists to encounter ethical dilemmas in clinical practice. These dilemmas not only require an exploration of the basic bioethical principles, but also considering that neurological disease can have impact on a person's self-identity. Patients with neurological disease are particularly vulnerable and at risk of having long-term sequelae of their nervous system and their individual selves. These important considerations can then raise the question of futility of treatment in a setting of uncertainty of outcome. In these complex situations, a pediatric neurologist plays an important role in shared decision making with both families and multidisciplinary teams. In this paper, we explore case-based scenarios that serve to demonstrate the unique character of ethics in neurology and the role of pediatric neurologists in finding solutions that are in a patient's best interest, have a minimal chance of harm, respect a patient's autonomy, and are just and promote justice.
{"title":"Exploring the Role of Pediatric Neurology in Determinations of Futility, Identifying Vulnerability, and Participating in Shared Decision Making through Case-Based Analysis","authors":"Julio Quezada, J. Lantos","doi":"10.1055/s-0040-1714354","DOIUrl":"https://doi.org/10.1055/s-0040-1714354","url":null,"abstract":"Abstract It is not uncommon for pediatric neurologists to encounter ethical dilemmas in clinical practice. These dilemmas not only require an exploration of the basic bioethical principles, but also considering that neurological disease can have impact on a person's self-identity. Patients with neurological disease are particularly vulnerable and at risk of having long-term sequelae of their nervous system and their individual selves. These important considerations can then raise the question of futility of treatment in a setting of uncertainty of outcome. In these complex situations, a pediatric neurologist plays an important role in shared decision making with both families and multidisciplinary teams. In this paper, we explore case-based scenarios that serve to demonstrate the unique character of ethics in neurology and the role of pediatric neurologists in finding solutions that are in a patient's best interest, have a minimal chance of harm, respect a patient's autonomy, and are just and promote justice.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":"11 1","pages":"067 - 072"},"PeriodicalIF":0.2,"publicationDate":"2020-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78985788","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 The ketogenic diet (KD) is a powerful therapeutic tool that can reduce seizure activity in patients with refractory epilepsy. However, dietary implementation can be difficult for patients and families due to the time and resource intensive nature of the diet. These challenges are particularly pronounced in economically disadvantaged or socially unstable patient populations. The first case of this series describes the successful implementation of KD in a low-income, homeless patient through the use of innovative KD recipes and access to social programs offered through Boston Medical Center. The second case illustrates the importance of consistent parental support to the success of KD and describes mechanisms used to improve familial support within a nonunified household. Collectively, these cases demonstrate ways by which health care providers can make this powerful dietary treatment accessible to low-resource patients.
{"title":"Social and Economic Challenges to Implementing the Ketogenic Diet: A Case Series","authors":"Sloan Lynch, Catherine N. Barry, L. Douglass","doi":"10.1055/s-0040-1713908","DOIUrl":"https://doi.org/10.1055/s-0040-1713908","url":null,"abstract":"Abstract The ketogenic diet (KD) is a powerful therapeutic tool that can reduce seizure activity in patients with refractory epilepsy. However, dietary implementation can be difficult for patients and families due to the time and resource intensive nature of the diet. These challenges are particularly pronounced in economically disadvantaged or socially unstable patient populations. The first case of this series describes the successful implementation of KD in a low-income, homeless patient through the use of innovative KD recipes and access to social programs offered through Boston Medical Center. The second case illustrates the importance of consistent parental support to the success of KD and describes mechanisms used to improve familial support within a nonunified household. Collectively, these cases demonstrate ways by which health care providers can make this powerful dietary treatment accessible to low-resource patients.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":"32 1","pages":"037 - 042"},"PeriodicalIF":0.2,"publicationDate":"2020-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83654983","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 Epilepsy is a chronic neurological disorder with a significant impact on sociocultural and economic aspects. The management of epilepsy in low-middle income countries (LMICs) is influenced by many factors such as disease burden, poverty, educational status, the reflection of the disease as a social stigma, diverse religious beliefs, and treatment expense. Despite the improvement in educational and social parameters, the stigmatization of the disease is still evident in many LMICs. The associated comorbidities and neurodevelopmental disorders further add to the cost and stigmatization. The pediatric neurologists/epileptologists in LMICs are encountered by distinctive ethical, social, and economic dilemmas during the patient care and management of epilepsy. This article discusses the various ethical dilemmas in a child with Dravet syndrome in a developing country.
{"title":"Ethical, Social, and Economic Challenges in Managing a Child with Dravet Syndrome in a Developing Country","authors":"S. Gulati, R. Sinha, Priyanka Madaan","doi":"10.1055/s-0040-1714065","DOIUrl":"https://doi.org/10.1055/s-0040-1714065","url":null,"abstract":"Abstract Epilepsy is a chronic neurological disorder with a significant impact on sociocultural and economic aspects. The management of epilepsy in low-middle income countries (LMICs) is influenced by many factors such as disease burden, poverty, educational status, the reflection of the disease as a social stigma, diverse religious beliefs, and treatment expense. Despite the improvement in educational and social parameters, the stigmatization of the disease is still evident in many LMICs. The associated comorbidities and neurodevelopmental disorders further add to the cost and stigmatization. The pediatric neurologists/epileptologists in LMICs are encountered by distinctive ethical, social, and economic dilemmas during the patient care and management of epilepsy. This article discusses the various ethical dilemmas in a child with Dravet syndrome in a developing country.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":"28 1","pages":"094 - 096"},"PeriodicalIF":0.2,"publicationDate":"2020-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81572688","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 Epilepsy and febrile convulsion are the most common neurological diseases with significant effect on cardiac functions. This study aimed to evaluate electrocardiography parameters alterations in epilepsy and febrile convulsion children compared with controls. In this comparison study, 270 children analyzed who shared equally in epilepsy, febrile convulsion, and healthy that aged from 0.5 to 5 years. The participants were collected from Ali ibn Abi Talib Hospital of Zahedan, Iran. Epilepsy confirmed based on definition of having at least two unprovoked seizures in 24 hours. Febrile convulsion was confirmed based on its definition by the International League against Epilepsy. Healthy children selected from those referred to the hospital with fever and without any underline diseases. Electrocardiography was performed by a pediatric cardiologist. Data were analyzed using SPSS 19 with p < 0.05 significant level. Heart rate was higher in epilepsy (129.64 ± 27.63) compared with control (108.78 ± 26.01) and febrile convulsion (125.79 ± 25.38; X 2 = 28.701, p < 0.001). S wave in lead V1 was higher in controls (0.72 ± 0.36) compared with epilepsy (0.58 ± 0.45) and febrile convulsion (0.58 ± 0.36). QT dispersion and QTc dispersion levels were higher in epilepsy than febrile convulsion children that both were higher than controls. Concluded that R in aVL, LV mass (LVM), QT dispersion, and QTc dispersion were higher significantly in epilepsy compared with febrile convulsion children. To maintain a good strategic treatment in patients with epilepsy and febrile convulsion, there is a need to assess alternations in ECG parameters, especially QT interval changes that lead to better comprehensive autonomic changes.
癫痫和热性惊厥是最常见的神经系统疾病,对心功能有显著影响。本研究旨在评估癫痫和热性惊厥患儿与对照组相比的心电图参数改变。在这项比较研究中,分析了270名年龄在0.5至5岁之间的儿童,他们在癫痫、热性惊厥和健康方面具有相同的特征。参与者来自伊朗扎黑丹的Ali ibn Abi Talib医院。根据24小时内至少有两次非诱发性癫痫发作的定义确诊为癫痫。热性惊厥是根据国际抗癫痫联盟的定义确定的。从那些转诊到医院的有发烧和没有任何突出疾病的健康儿童中挑选。由儿科心脏病专家进行心电图检查。数据采用SPSS 19分析,p < 0.05显著水平。癫痫组心率(129.64±27.63)高于对照组(108.78±26.01)和热惊厥组(125.79±25.38);x2 = 28.701, p < 0.001)。对照组V1导联S波(0.72±0.36)高于癫痫组(0.58±0.45)和热惊厥组(0.58±0.36)。癫痫患儿QT离散度和QTc离散度水平高于热惊厥患儿,两者均高于对照组。结论癫痫病患儿aVL R、左室质量(LVM)、QT离散度、QTc离散度明显高于热惊厥患儿。为了对癫痫和热性惊厥患者保持良好的治疗策略,有必要评估心电图参数的改变,特别是QT间期的改变是否能导致更好的全面自主神经变化。
{"title":"Electrocardiography Parameters Changes in Epilepsy and Febrile Convulsion Children Compared with Controls","authors":"N. Noori, Alireza Teimouri, A. Khajeh","doi":"10.1055/s-0040-1713907","DOIUrl":"https://doi.org/10.1055/s-0040-1713907","url":null,"abstract":"Abstract Epilepsy and febrile convulsion are the most common neurological diseases with significant effect on cardiac functions. This study aimed to evaluate electrocardiography parameters alterations in epilepsy and febrile convulsion children compared with controls. In this comparison study, 270 children analyzed who shared equally in epilepsy, febrile convulsion, and healthy that aged from 0.5 to 5 years. The participants were collected from Ali ibn Abi Talib Hospital of Zahedan, Iran. Epilepsy confirmed based on definition of having at least two unprovoked seizures in 24 hours. Febrile convulsion was confirmed based on its definition by the International League against Epilepsy. Healthy children selected from those referred to the hospital with fever and without any underline diseases. Electrocardiography was performed by a pediatric cardiologist. Data were analyzed using SPSS 19 with p < 0.05 significant level. Heart rate was higher in epilepsy (129.64 ± 27.63) compared with control (108.78 ± 26.01) and febrile convulsion (125.79 ± 25.38; X 2 = 28.701, p < 0.001). S wave in lead V1 was higher in controls (0.72 ± 0.36) compared with epilepsy (0.58 ± 0.45) and febrile convulsion (0.58 ± 0.36). QT dispersion and QTc dispersion levels were higher in epilepsy than febrile convulsion children that both were higher than controls. Concluded that R in aVL, LV mass (LVM), QT dispersion, and QTc dispersion were higher significantly in epilepsy compared with febrile convulsion children. To maintain a good strategic treatment in patients with epilepsy and febrile convulsion, there is a need to assess alternations in ECG parameters, especially QT interval changes that lead to better comprehensive autonomic changes.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":"11 1","pages":"110 - 116"},"PeriodicalIF":0.2,"publicationDate":"2020-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81687799","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 At least 20% of patients referred to pediatric epilepsy centers with the suspicion of epileptic seizures actually have other conditions. Neuroglobin is a new globin member which is highly expressed in the central and peripheral nervous systems. In this article, we aimed to evaluate usefulness of neuroglobin to differentiate between epilepsy and other conditions that mimic epilepsy. Our study was conducted on 90 children divided into three groups: 30 patients with epileptic seizures, 35 children with nonepileptic paroxysmal disorder, and 25 apparently healthy, age and sex-matched children as a normal control. Complete blood count, blood chemistries including random blood glucose, calcium, sodium, in addition to serum prolactin, and neuroglobin were performed for all children. This study showed a significant increase of both serum neuroglobin and prolactin levels in epileptic group compared with nonepileptic paroxysmal disorder and control groups (p < 0.01). Serum neuroglobin showed 95% sensitivity and 95.7% specificity in the diagnosis of generalized seizures. Serum neuroglobin may be a promising novel marker to differentiate epileptic versus nonepileptic disorders in children in the emergency setting, when history and clinical presentation are equivocal.
{"title":"Neuroglobin and Prolactin As Potential Biomarkers for Differentiating Epileptic versus Nonepileptic Paroxysmal Disorders in Children","authors":"M. Mahgoob, M. Moussa","doi":"10.1055/s-0040-1713153","DOIUrl":"https://doi.org/10.1055/s-0040-1713153","url":null,"abstract":"Abstract At least 20% of patients referred to pediatric epilepsy centers with the suspicion of epileptic seizures actually have other conditions. Neuroglobin is a new globin member which is highly expressed in the central and peripheral nervous systems. In this article, we aimed to evaluate usefulness of neuroglobin to differentiate between epilepsy and other conditions that mimic epilepsy. Our study was conducted on 90 children divided into three groups: 30 patients with epileptic seizures, 35 children with nonepileptic paroxysmal disorder, and 25 apparently healthy, age and sex-matched children as a normal control. Complete blood count, blood chemistries including random blood glucose, calcium, sodium, in addition to serum prolactin, and neuroglobin were performed for all children. This study showed a significant increase of both serum neuroglobin and prolactin levels in epileptic group compared with nonepileptic paroxysmal disorder and control groups (p < 0.01). Serum neuroglobin showed 95% sensitivity and 95.7% specificity in the diagnosis of generalized seizures. Serum neuroglobin may be a promising novel marker to differentiate epileptic versus nonepileptic disorders in children in the emergency setting, when history and clinical presentation are equivocal.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":"111 1","pages":"104 - 109"},"PeriodicalIF":0.2,"publicationDate":"2020-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80777664","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 Separate pediatric studies for antiepileptic drugs (AEDs) emerged with general separate drug approval in children and were defined by the U.S. Food and Drug Administration (FDA) as <17 years and by the European Union (EU) as <18 years. These administrative age limits are necessary in pediatrics, but they correspond variably with the physiological maturity of young patients and are not helpful for therapeutic decisions or as study inclusion criteria. AEDs are often effective for partial onset seizures (POS) in 2 to 17-year-olds as well as in ≥18-year-olds, if dosed correctly. Separate pediatric AED studies assume no difference between the legal and the physiological meaning of the word “child.” While the FDA now accepts efficacy of AEDs in POS in children ≥2 years, the EU still requires separate “pediatric” studies. For retigabine it waived all pediatric studies after having required 20 such studies over several years. We feel the current regulation creates a situation where many studies in children are done unnecessarily; we question the ethics of such an approach, which in our view, is morally wrong. Critical publications contributed to the FDA's shift of opinion for AEDs in POS but did not address the blur of different meanings of the word “child.”
{"title":"Ethical Issues in Pediatric Regulatory Studies Involving Placebo Treatment","authors":"K. Rose, D. Neubauer, J. Grant-Kels","doi":"10.1055/s-0040-1712147","DOIUrl":"https://doi.org/10.1055/s-0040-1712147","url":null,"abstract":"Abstract Separate pediatric studies for antiepileptic drugs (AEDs) emerged with general separate drug approval in children and were defined by the U.S. Food and Drug Administration (FDA) as <17 years and by the European Union (EU) as <18 years. These administrative age limits are necessary in pediatrics, but they correspond variably with the physiological maturity of young patients and are not helpful for therapeutic decisions or as study inclusion criteria. AEDs are often effective for partial onset seizures (POS) in 2 to 17-year-olds as well as in ≥18-year-olds, if dosed correctly. Separate pediatric AED studies assume no difference between the legal and the physiological meaning of the word “child.” While the FDA now accepts efficacy of AEDs in POS in children ≥2 years, the EU still requires separate “pediatric” studies. For retigabine it waived all pediatric studies after having required 20 such studies over several years. We feel the current regulation creates a situation where many studies in children are done unnecessarily; we question the ethics of such an approach, which in our view, is morally wrong. Critical publications contributed to the FDA's shift of opinion for AEDs in POS but did not address the blur of different meanings of the word “child.”","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":"46 1","pages":"073 - 079"},"PeriodicalIF":0.2,"publicationDate":"2020-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"83662222","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 Convulsive status epilepticus (CSE) is one of the commonest and life threatening pediatric neurological emergencies. Only few studies on pediatric status epilepticus (SE) are available from the Indian subcontinent. The aim was to study the etiology and immediate outcome of CSE in children getting admitted in pediatric emergency during the study period and to study association of various clinical presentations with immediate outcome. A prospective observational study was conducted in tertiary care institute of Northern India. Children aged between 1 month and 18 years with CSE were enrolled and followed up till discharge. Primary outcome was kept as hospital survival/mortality; those who survived were further graded on the basis of disability at discharge. A total of 81 patients with CSE were assessed for primary outcome. The mortality rate was found to be 15% (12/81); 83% deaths were due to acute central nervous system (CNS) infection. Ten percent of children (8/81) had disability at discharge, five had mild, and three had moderate disability. The neurocysticercosis was the most common etiology seen in 23% (19/81) of the children followed by febrile SE in 20% (13/81) of the study participants. CSE responded to first-line antiepileptic drugs (AED) in 15% children (12/81). Refractory status was seen in 13.5% (11/81) cases. Prehospital treatment was received only in 15% patients. Young age, low Glasgow coma scale score at admission, and requirement of critical care support were the factors found to be significantly associated with mortality. CSE has a high mortality especially in young children and acute CNS infections are the most common cause of it.
{"title":"Convulsive Status Epilepticus in Children: A Prospective Observational Study from India","authors":"Adhi Arya, C. Azad, V. Mahajan, V. Guglani","doi":"10.1055/s-0040-1712544","DOIUrl":"https://doi.org/10.1055/s-0040-1712544","url":null,"abstract":"Abstract Convulsive status epilepticus (CSE) is one of the commonest and life threatening pediatric neurological emergencies. Only few studies on pediatric status epilepticus (SE) are available from the Indian subcontinent. The aim was to study the etiology and immediate outcome of CSE in children getting admitted in pediatric emergency during the study period and to study association of various clinical presentations with immediate outcome. A prospective observational study was conducted in tertiary care institute of Northern India. Children aged between 1 month and 18 years with CSE were enrolled and followed up till discharge. Primary outcome was kept as hospital survival/mortality; those who survived were further graded on the basis of disability at discharge. A total of 81 patients with CSE were assessed for primary outcome. The mortality rate was found to be 15% (12/81); 83% deaths were due to acute central nervous system (CNS) infection. Ten percent of children (8/81) had disability at discharge, five had mild, and three had moderate disability. The neurocysticercosis was the most common etiology seen in 23% (19/81) of the children followed by febrile SE in 20% (13/81) of the study participants. CSE responded to first-line antiepileptic drugs (AED) in 15% children (12/81). Refractory status was seen in 13.5% (11/81) cases. Prehospital treatment was received only in 15% patients. Young age, low Glasgow coma scale score at admission, and requirement of critical care support were the factors found to be significantly associated with mortality. CSE has a high mortality especially in young children and acute CNS infections are the most common cause of it.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":"4 1","pages":"027 - 032"},"PeriodicalIF":0.2,"publicationDate":"2020-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89433423","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 Epilepsy represents a burdensome neurological disorder with higher incidence before the age of 18 years. The treatment is medical and involves long-term administration of antiepileptic drugs (AED). There are known high-resistant syndromes with onset in infancy and childhood, Dravet syndrome, being one of them. It is a well-known fact that early seizures treatment prevents associated comorbidities, cognitive and motor disabilities, and improve long-term prognosis. There are several AEDs available but not all of them are approved for use in infants. This is due to the need for additional toxicology studies at this age and for development of suitable formulations. A 14-month-old girl with Dravet syndrome was presented here. Prompt diagnosis was made based on clinical features and confirmed by the genetic tests. She partially responded to valproate and clobazam but continued to have prolonged febrile seizures. We added stiripentol after consulting reports of studies in infants younger than 2 years and after obtaining family consent. She responded well with decrease in episodes of status epilepticus and improvement in psychomotor development and stiripentol was tolerated well. Off label use of certain AEDs can benefit infants when there are no major pharmacokinetic differences in comparison to older children.
{"title":"Should Off Label Antiepileptic Drugs Be Used for Treatment of Infancy and Childhood Epilepsy? Discussions Based on a Dravet Syndrome Case Report","authors":"M. Vințan","doi":"10.1055/s-0040-1712498","DOIUrl":"https://doi.org/10.1055/s-0040-1712498","url":null,"abstract":"Abstract Epilepsy represents a burdensome neurological disorder with higher incidence before the age of 18 years. The treatment is medical and involves long-term administration of antiepileptic drugs (AED). There are known high-resistant syndromes with onset in infancy and childhood, Dravet syndrome, being one of them. It is a well-known fact that early seizures treatment prevents associated comorbidities, cognitive and motor disabilities, and improve long-term prognosis. There are several AEDs available but not all of them are approved for use in infants. This is due to the need for additional toxicology studies at this age and for development of suitable formulations. A 14-month-old girl with Dravet syndrome was presented here. Prompt diagnosis was made based on clinical features and confirmed by the genetic tests. She partially responded to valproate and clobazam but continued to have prolonged febrile seizures. We added stiripentol after consulting reports of studies in infants younger than 2 years and after obtaining family consent. She responded well with decrease in episodes of status epilepticus and improvement in psychomotor development and stiripentol was tolerated well. Off label use of certain AEDs can benefit infants when there are no major pharmacokinetic differences in comparison to older children.","PeriodicalId":42559,"journal":{"name":"Journal of Pediatric Epilepsy","volume":"24 1","pages":"080 - 082"},"PeriodicalIF":0.2,"publicationDate":"2020-05-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81822452","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}