Vigabatrin in pediatric patients with refractory epilepsy

New generation antiepileptic medications have improved seizure outcome in patients with intractable epilepsy. We studied the efficacy and side effect profile of vigabatrin (VGB) in pediatric patients with intractable seizure disorder. We reviewed the database of our short-term video-EEG monitoring laboratory to screen patients with intractable epilepsy who were on VGB either alone or in combination for three months or more. We subsequently reviewed the medical records of these patients to abstract clinical information regarding age, sex, seizure type, epilepsy syndrome, efficacy and side effects of VGB. Of 111 patients, 75 (68%) were male and 36 (32%) female. Seizure onset was during the newborn period in 12 patients (11%), during the first year of life beyond the newborn period in 47 patients (42%), between 1-5 years in 23 patients (21%), and above five years in the remaining 29 patients (26%). Fifty-four patients (48.6%) had partial onset seizures with or without secondary generalization; 49 patients (44.1%) had primary generalized seizures; 8 patients (7.2%) had two or more types of seizure. Fifty-three percent of patients had mental retardation, and 35% had abnormal findings on physical/ neurological examination. Of 98 patients, 70 (71.4%) had abnormal magnetic resonance imaging (MRI) findings. Ninety-seven percent of patients had been on polytherapy before VGB was added to treatment. VGB reduced seizure frequency by at least 50% in 33.3% of patients with partial seizures, and in 30.6% of patients with primary generalized seizures. Six of the responders with partial seizures had complete resolution of their seizures. Most common side effects included visual field defects, increased appetite and obesity. Vigabatrin seems to be more effective in partial seizures in childhood intractable epilepsy. Patients should be closely monitored regarding side effects of VGB.     

Altered sleeping arrangements in pediatric patients with epilepsy

Parental fears concerning seizure occurrence may be associated with behavioral changes within the home environment. One possible change involves sleeping arrangements. Questionnaires concerning demographics, medical history, and sleeping arrangements were completed by parents of 179 children with epilepsy and by parents of 155 children with diabetes for comparison purposes. Based on parental response, 40 (22%) children with epilepsy changed to less independent sleeping arrangements. Logistic regression suggested that parental concern over seizure occurrence was highly associated with this change (p=<0.001). In contrast, 13 (8%) of the children with diabetes changed to a less independent sleep pattern. Results suggest changes in sleeping arrangements may alert the pediatrician to possible parental anxiety that may need to be addressed.     

儿童癫痫的精准治疗

癫痫是儿童时期最常见且危害最大的神经系统疾病之一,抗癫痫药是主要的治疗方法,但在过去几十年,人们对癫痫的病因了解不多,因此主要是根据癫痫发作类型进行选药,治标不治本.近年来随着检测工具和手段的进步,越来越多的癫痫患儿能够找到癫痫病因,针对病因进行治疗能达到标本兼治的目的,儿童癫痫的治疗正步入精准治疗时代.文章根据201...     

Psychiatric concerns in pediatric epilepsy

Pediatric epilepsy is a common, chronic, and challenging physical illness for children and their families. This article provides a medical overview and discusses the cognitive functioning and psychosocial adjustment as well as the psychiatric management for children and adolescents with pediatric epilepsy. The management of these children involves establishing a collaborative health care approach, evaluating academic functioning, considering psychotherapy, and managing psychopharmacologic treatment. A thorough understanding of the biopsychosocial concerns in pediatric epilepsy can enable medical providers and mental health clinicians to promote resiliency and adaptation in children and their families facing troubling seizure disorders.     

Developmental language disorders and epilepsy

Abstract: The association of speech and language disorders with epilepsy is well-known in children with acquired epileptic aphasia, involving such entities as Landau—Kleffner syndrome (LKS), continuous spike wave in slow wave sleep (CSWSS) epilepsy and benign partial epilepsy with centra-temporal spikes (BPECTS). The possible association between epilepsy and a subgroup of children with developmental dysphasia is reported less frequently. Lack of controlled prospective studies of sleep electroencephalograms (EEG), and the use of medication, in children with developmental dysphasia, may deny appropriate treatment strategies to children with severe developmental speech and language disorders.     

癫痫患儿伴抑郁研究进展

癫痫患者伴发抑郁的发病率较高,儿童及青少年是癫痫的主要人群。由于儿童及青少年年龄阶段身心发育的特殊性,其发病和用药都有自身的特点。文章对儿童及青少年癫痫患者伴发抑郁的影响、原因、特点、应对措施等相关研究进展作一综述。     

Role of surgery in pediatric epilepsy

Twenty five percent of patients with intractable epilepsy have surgically remediable epilepsy syndromes. This article reviews the treatment paradigm for pediatric epilepsy and also the indications, methods, and surgical options for the subgroup of patients with surgically remediable epileptic disorders based on our experience in the management of these children. The article also discusses the rationale for offering surgery and the timing of surgery in these patients. The study of surgically remediable epilepsy can best be divided into focal, sub hemispheric, hemispheric and multifocal epileptic syndromes. These syndromes have both acquired and congenital etiologies and can be treated by resective or disconnective surgery. The surgical management of these conditions (with the exception of multifocal epilepsy) provides Engel's Class 1 outcome(complete seizure freedom) in approximately 80% of children. The consequences of seizure freedom leads to a marked improvement in the quality of life of these children.The benefits to society, of allowing a child to grow to adulthood with normal cognition to earn a livelihood and contribute actively to society, cannot be understated.     

Neurosurgical management of pediatric epilepsy

Pediatric neurosurgeons are often involved early in the evaluation and management of children with epilepsy. Surgery should not be viewed merely as the end stage of multiple diagnostic decisions made by neurologists and pediatricians. Instead, when a reasonable trial of several AEDs has failed to provide adequate seizure control, a pediatrician can refer the patient directly to a pediatric neurosurgeon with a specialty interest in epilepsy. The diagnosis and treatment of focal versus diffuse epilepsy can be discussed in an honest and educated fashion with the families and the caregivers. Although full seizure control may be impossible, improved neurologic development and avoidance of self-injurious drop attacks may be worthy alternative surgical outcomes.     

Add-on lamotrigine in pediatric epilepsy in India

Lamotrigine is a newer antiepileptic drug useful as oral adjunctive therapy in refractory epilepsy. Indian data on use of lamotrigine is limited. This study was conducted to evaluate add-on lamotrigine in Indian children with epilepsy. Twenty children (median age 90 months) receiving lamotrigine as add-on therapy for mean 26.7 (19.1) months, were followed for a median period of 7.9 (6–10) months. Follow-up was done every two weeks. The most common seizures types were either generalized tonic-clonic (6, 30%) or myoclonic (8, 40%). The average dose used was 3.86 mg/kg/day (with concomitant valproate). Good response (>50% reduction) or complete seizure control was seen in 72% patients. Side effects were seen in 27.5% patients and were ‘mild’ in more than half of these. Lamotrigine was stopped in two patients due to adverse reactions, which resolved on stopping the drug. Lamotrigine was observed to be an effective, add-on, broad-spectrum antiepileptic with ‘mild’ side effects in Indian children.     

心内接触与非接触性标测指导导管消融术治疗儿童频发室性早搏

目的比较心内接触与非接触性标测在指导儿童频发室性早搏导管消融术中的优越性、安全性和适应证的选择。方法研究对象为2002年8月至2008年6月广东省人民医院收治的8例无器质性心脏病的频发室性早搏患儿,24h室早总数平均(30000±8465)个。采用Ensite非心内接触性标测系统指导消融4例,年龄10～14岁,右室流出道2例,右室流入道2例;传统心内接触性标测指导消融4例,年龄6～10岁,右室流出道2例,右室流入道1例,左室流出道1例。术前和术后1、3个月记录24h室早总数,记录两种方法的X线曝光时间、并发症和适应证。结果两种方法术后均获即刻成功,无并发症发生,7例术后1、3个月室早总数0～5个/24h,其中1例左室流出道室早复发,先后2次消融成功;X线曝光时间右室流出道:16.5min vs 32.5min,右室流入道:26.5min vs 60min,心内接触性标测消融左室流出道室早:90min。结论EnSite非接触性三维标测较接触性标测下消融有许多优越性,但同样也存在着局限性,心内接触性标测适应性广,是非接触性三维标测的基础和补充,尤其在EnSite非接触性三维标测标测失败时。     

三维电生理标测系统在儿童心律失常导管消融中的应用

目的 探讨三维标测系统(Carto和Ensite)在指导儿童心律失常导管消融中的安全性、适应证和优越性.方法 ①在Carto指导下房性心动过速(atrial tachycardia,AT)消融8例;年龄(6.2±1.7)岁,体重(18.0±2.0)kg.②在Ensite指导下心律失常消融10例.其中应用Ensite Array系统指导消融8例,包括频发右室室性早搏(premature ventricular contractions,PVCs)6例,右房AT2例,年龄(11.3±1.2)岁,体重(40.0±5.0)kg;应用Ensite NavX系统指导消融2例,为B型预激综合征.结果 ①在Carto指导下8例AT患儿成功消融,其中"切口"性AT6例,左房耳AT和右房AT各1例;1例"切口"AT术后3个月复发,再次消融成功.②在Ensite Array指导下6例频发右PVCs和2例右房AT患儿成功消融,其中PVCs从术前(32 333±4509)个/24 h下降至0～4个/24 h;1例AT患儿1 d后出现另一种周期的AT.在Ensite Navx指导下成功消融2例B型预激综合征.随访6个月无复发.结论 Carto系统适合持续心律失常患儿的检测,而Ensite Array系统适合大于10岁、不持续右心心律失常患儿的检测;Ensite NavX可迅速建模,显示心内解剖结构.     

小儿全身性癫痫与髓鞘碱性蛋白关系的研究

目的　 通过检测脑脊液 (CSF)和血清髓鞘碱性蛋白 (MBP)的含量 ,了解小儿全身性癫痫是否引致髓鞘和血脑屏障(BBB)的破坏。 方法 　采用简易MBPELISA法对 47例小儿全身性癫痫病患儿脑脊液和血清标本作MBP定量测定。 结果 　在一个月内有全身性癫痫发作的患儿其CSF和血清MBP含量均值明显高于正常对照组 (P <0 0 1)。 结论 　小儿全身性癫痫发作可导致CSF和血清MBP升高 ,提示大脑器质性损伤和BBB的破坏。MBP含量的检测可作为全身性癫痫预后评估的一项生化指标。     

Vagal nerve stimulation in refractory epilepsy: the first 100 patients receiving vagal nerve stimulation at a pediatric epilepsy center

OBJECTIVE: To determine the outcome of intermittent left vagal nerve stimulation on the first 100 consecutive patients treated at our pediatric epilepsy center. METHODS: Patients were identified by means of operating room records. Data collected described the patient's epilepsy, previous and subsequent therapies, adverse events, nonepileptic changes, and outcomes. RESULTS: Average age was 10.4 years; years of epilepsy, 8.5; total number of antiepileptic therapies, 8.4; and median monthly seizure frequency, 120. Data on seizure frequency at follow-up were available for 96 of the 100 patients. Forty-five percent of patients achieved greater than 50% reduction; and 18% had had no seizures for the last 6 months. Response was similar in patients with more than 7 years of refractory epilepsy as compared with patients with a shorter history. Magnet-generated, on-demand current reduced seizure intensity in almost half of the patients with available data. Generator infections occurred in 3 patients. Twenty-four patients had their generators removed. Subsequently, 2 of these patients died. CONCLUSIONS: Seizure reduction was the same in patients younger than 12 years and 12 years or older and in patients with shorter and longer histories of refractory epilepsy. Adverse effects were few in this population, particularly in those younger than 12 years. Vagal nerve stimulation appears to be a relatively safe and potentially effective treatment for children with severely intractable epilepsy.