Surgical Indication for Refractory Childhood Epilepsy

Summary: Recent progress in surgical intervention for medically refractory epilepsy has helped to shed light on more complex epileptogenic problems in children and infants. Surgical treatment increasingly is being used in pediatric patients, but the indications for surgery in this age group have not been well defined. The developing child with a seizure disorder has several problems that are different from adults, such as neural plasticity, deleterious effects of seizures on developmental status, and spontaneous resolution of epilepsy. The critical age for irreversible brain dysfunction and the timing of surgery are the main issues for the treatment of children. Thus, earlier surgical intervention is generally recommended to prevent further deterimental seizure effects, but we still do not know the optimal age. Until the establishment of guidelines for pediatric epilepsy surgery, surgical indications should be determined by the prognosis and the presence of a resectable epileptogenic focus, which in turn are based on the localization of the epileptic focus, seizure frequency, severity, and cognitive function of each case, rather than just the patient's age.     

Treatment and Discontinuation of Antiepileptic Drugs in Childhood Epilepsy

Abstract: The process of discontinuing anticonvulsant therapy was studied clinicoelectroencephalographically on 285 children with epilepsy. The subjects were divided into two groups electroencephalographically: First, 190 cases of the formally discontinued group which had antiepiletic drugs withdrawn after the cessation of clinical seizures and suppressing epileptic discharges for over two years and, second, 95 cases which were informally discontinued. The relapse rate after discontinuation was significantly lower in the former group, 2.1%, than the latter group, 21.1%. Concerning the seizure-free period, among the cases free from clinical seizures for over three years at the time of withdrawal, the relapse rate was significantly lower than the rest of the cases. Moreover, the spike-free period proved to be a more reliable indicator for stopping medication than the seizure-free period. From these findings, the withdrawal of antiepileptic drugs should be started after suppression of epileptic discharges for over two years in childhood epilepsy.     

Initiation of Treatment and Selection of Antiepileptic Drugs in Childhood Epilepsy

Summary:  Purpose: A retrospective study was carried out on 53 cases with childhood epilepsy to evaluate the validity of the initial selection of antiepileptic drug (AED).
Methods: We investigated the AEDs selected at the beginning of the treatment from the medical records of 53 untreated cases. A follow-up study was undertaken to evaluate the effects of the AEDs. In the second study, we investigated the AEDs of 10 cases with atypical benign partial epilepsy (ABPE), to clarify whether the initial AEDs selected for rolandic epilepsy were related to the appearance of ABPE.
Results: The AEDs used at the initial stage consisted of carbamazepine (CBZ), valproic acid (VPA), phenobarbital (PB), and vitamin B₆. The main AEDs were CBZ and VPA for localization-related epilepsy, and VPA for generalized epilepsy. The initial selection of AEDs in 41 (85.4%) of 48 cases treated with AEDs were considered to be correct from the results of follow-up. We could not specify any AEDs that related to the appearance of ABPE.
Conclusions: The selection of AED in this series was considered to be most appropriate. We proposed a criterion to determine whether to begin the AED treatment immediately at the initial seizure.     

Current Advances in Childhood Absence Epilepsy

BackgroundChildhood absence epilepsy is an age-dependent, idiopathic, generalized epilepsy with a characteristic seizure appearance. The disorder is likely to be multifactorial, resulting from interactions between genetic and acquired factors, but the debate is still open. We review recent studies on different aspects of childhood absence epilepsy and also to describe new concepts.MethodsData for this review were identified using Medline and PubMed survey to locate studies dealing with childhood absence epilepsy. Searches included articles published between 1924 and 2013.ResultsThe diagnosis comprises predominant and associated seizure types associated with other clinical and electroencephalographic characteristics. Many studies have challenged the prevailing concepts, particularly with respect to the pathophysiological mechanisms underlying the electroencephalographic seizure discharges. Childhood absence epilepsy fits the definition of system epilepsy as a condition resulting from the persisting susceptibility of the thalamocortical system as a whole to generate seizures. This syndrome, if properly defined using strict diagnostic criteria, has a good prognosis. In some cases, it may affect multiple cognitive functions determining risk for academic and functional difficulties; the detection of children at risk allows tailored interventions. Childhood absence epilepsy is usually treated with ethosuximide, valproate, lamotrigine, or levetiracetam, but the most efficacious and tolerable initial empirical treatment has not been well defined.ConclusionsWe review recent studies and new concepts on the electroclinical features and pathophysiological findings of childhood absence epilepsy in order to highlight areas of consensus as well as areas of uncertainty that indicate directions for future research.     

EEG of a Nocturnal Seizure in a Patient with "Benign Epilepsy of Childhood with Rolandic Spikes"

The first EEG recording is reported of a child with "benign epilepsy of childhood with Rolandic spikes" during a partial motor (facial) seizure, which occurred during Phase II of natural sleep.     

Nonconvulsive Status Epilepticus in Childhood Localization-Related Epilepsy

PURPOSE: To report on three children with localization-related epilepsy who exhibited minor seizures (atypical absences, brief atonic, and myoclonic) and nonconvulsive status epilepticus (NCSE) consisting of these minor seizures, and to elucidate their significance. METHODS: We studied the electroclinical characteristics of these children. Ictal electroencephalograms (EEGs) of NCSE were evaluated by using simultaneous video-EEG-electromyogram (EMG) polygraphic recordings. RESULTS: All patients began to have partial seizures between the ages of 6 months and 2 years 7 months, with minor seizures appearing later, between the ages of 1 year 11 months and 6 years 6 months. These minor seizures evolved into NCSE. Complex partial seizures remained after suppression of the minor seizures. Interictal EEGs taken when the minor seizures appeared showed excessive diffuse epileptic discharges in addition to multifocal spike-waves. Before and after suppression of the minor seizures, focal epileptic discharges predominated on the EEGs. On ictal EEGs of brief atonic and myoclonic seizures, diffuse spike-wave and polyspike-wave bursts were detected. Ictal EEGs of the atypical absences revealed diffuse spike-wave bursts mixed with irregular high-voltage slow waves, often interspersed with brief atonic and myoclonic seizures. When atypical absences lasted for a long time, patients manifested NCSE. Polytherapy might be related to the occurrence of minor seizures and NCSE, because all patients were treated with polytherapy at their appearance, and simplification of antiepileptic drug (AED) therapy seemed to be effective. CONCLUSIONS: We concluded that this NCSE is a type of atypical absence status which is an age-dependent, transient, electroclinical condition. The mechanism of occurrence of these minor seizures might be related to secondary bilateral synchrony.     

Complex Partial Seizures in Childhood

Abstract. A clinical and electroencephalographic study was undertaken on 215 children with complex partial seizures as selected on the basis of the International Classification of Epileptic Seizures (1981).

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  Complex partial seizures were noted in 7.8% of the epileptic children.
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  The ictal symptoms of complex partial seizures closely resembled those of psychomotor triad described by Lennox.
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  Interictal EEG revealed seizure discharges from the temporal or frontal focus in 57.2%.
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  There existed a group with automatism as a main symptom having both diffuse slow spike-waves and focal temporal spikes. This type should be interpreted to be one of secondary generalized epilepsies and be a variant of the Lennox syndrome.

     

Intravenous Methylprednisolone for Intractable Childhood Epilepsy

BackgroundSteroids have been used for the treatment of certain epilepsy types, such as infantile spasms; however, the use in the treatment of other intractable epilepsies has received limited study. We report our experience with intravenous methylprednisolone in children with epilepsy refractory to multiple antiepileptic drugs.MethodsA series of consecutive children were analyzed retrospectively. Patients with infantile spasms, progressive degenerative, or metabolic disorders were excluded.ResultsSeventeen children aged 2-14 (mean 5.3) years were included. Associated cognitive and motor deficits were recognized in 82%. Most children (88%) had daily seizures and 13 (76%) were admitted previously with status epilepticus. The epilepsy was cryptogenic (unknown etiology) in 47% and the seizures were mixed in 41%. Intravenous methylprednisolone was given at 15 mg/kg per day followed by a weaning dose of oral prednisolone for 2-8 weeks (mean 3 weeks). Children were followed for 6-24 months (mean 18). Six (35%) children became completely seizure free; however, three of them later developed recurrent seizures. At 6 months posttreatment, improved seizure control was noted in 10 (59%) children. Children with mixed seizures were more likely to have a favorable response than those with one seizure type (49% vs 31%, P = 0.02). No major side effects were noted, and 35% of the parents reported improvements in their child's alertness and appetite.ConclusionAdd-on steroid treatment for children with intractable epilepsy is safe and may be effective in some children when used in a short course.     

Refractory Childhood Epilepsy and Factors Related to Refractoriness

Summary: To clarify the characteristics of refractory childhood epilepsy, we compared recent refractory cases and those of approximately 15 years ago, all of which were seen at the Okayama University Hospital. We also analyzed predictive factors related to refractoriness in the recent refractory cases. Among the recent refractory cases, the proportion of localization-related epilepsies increased, and the proportion of generalized epilepsies decreased compared to historical cases. In generalized epilepsies, the proportion of cases with Lennox-Gastaut syndrome decreased to less than half. In localization-related epilepsies, the proportion of cases with frontal lobe epilepsy increased. The proportion of cases with unknown causes decreased to less than half. Of the cases with known causes, postencephalitis/postencephalopathy and focal cortical malformation, including tuberous sclerosis, accounted for most of the cases. More of the recent refractory cases were treated with high-dose AED monotherapy, compared to more poly-therapy in the cases of 15 years earlier. The following factors were related to future refractoriness: less than 1 year of age at onset of seizures, the presence of underlying pathology, status epilepticus, changes in type of epilepsy during the clinical course, and neonatal seizures. Regarding EEG findings of cases that had localization-related epilepsies at the end of follow-up, focal spike-waves associated with diffuse spike-waves on the first EEGs indicated future refractoriness.     

儿童良性癫Xian的随访研究

目的:探讨儿童良性癫癎的预后。方法:对１８３例儿童良性癫癎进行了２~１０年的随访观察。结果:１５６例(８５．２５%)脑电图恢复正常,其中１４３例经控制发作后已停药２~８年,均无临床发作。结论:良性癫癎患儿可无家族史。脑电图睡眠诱发试验可提高本病确诊率。经抗癫癎治疗控制发作１~２年后,脑电图恢复正常者可以停药。及早诊断及治疗者,预后较好。本病患儿可无明显心理障碍。     

Factors That Preclude the Successful Use of Monopharmacy in the Medical Treatment of Patients with Epilepsy

Abstract: A neurologist conducted research efforts for more than 12 years as a step toward the establishment of monotherapy for epilepsy. Of 406 patients with epilepsy, seizures could be controlled for more than one year in 72% and for more than 3 years in 54%. Monotherapy was given to 57% of all the patients with a success rate of 54%. Factors that were found likely to interfere with a reduction in antiepileptic drug therapy to one drug modality included: symptomatic etiology, prolonged duration of illness, low age at onset and secondary generalized epilepsy with a large number of seizures in combination, for generalized epilepsies; symptomatic etiology, prolonged duration of illness, low age at onset, other than occipital lobe origin, complex partial seizure with secondary generalization, temporal lobe epilepsy with associated automatisms, elementary sensorimotor seizure and high frequency of seizures, for partial epilepsies. In addition to these factors relevant to the nature of epilepsies, other factors apparently unrelated to the disease process, e.g., liability of polypharmacy to produce side effects precluding dosage elevation, patient's rejection to reduce drug and the inability of a physician to treat the patient properly for want of information, were also recognized to exist.     

左乙拉西坦添加治疗难治性癫癎的疗效和安全性分析

目的:探讨左乙拉西坦添加治疗难治性癫痫的疗效和安全性。方法:回顾性分析了35例左乙拉西坦添加治疗的难治性癫痫患者,随访3～18个月,观察其疗效及不良反应。结果:左乙拉西坦添加治疗难治性癫痫总有效率为48.6%,对部分性发作和全面性发作的疗效无显著差异。不良反应发生率为31.5%,无严重不良反应。结论:左乙拉西坦是一种安全有效的难治性癫痫治疗药物,可以用于难治性癫痫的添加治疗。     

左乙拉西坦单药治疗各类癫痈的疗效

左乙拉西坦是一种新型作用机制的抗癫痫药物,其抗痢机制可能是通过影响突触囊泡蛋白SV2A来实现。本文就该药物单一治疗各类癫痫（新诊断癫痫、部分性发作、全面性发作及手术后癫痫等）的疗效和耐受性研究做介绍。     

High-Dose Phenytoin in the Treatment of Refractory Epilepsy

Refractory, long-standing, high-frequency epilepsy was successfully managed with high-dose phenytoin treatment. Side effects were observed in only three patients and were easily corrected. Most of the patients had partial seizures, but many also had a myoclonic component. Emphasis was placed on monotherapy whenever possible. Results, interpretation of serum levels, and toxic reactions to phenytoin are discussed.     

Frequency of Automatic Stimulations in Responsive Vagal Nerve Stimulation in Patients With Refractory Epilepsy

BackgroundIn vagal nerve stimulation (VNS) therapy, the release of VNS model 106 (AspireSR) allowed for responsive VNS (rVNS). rVNS utilizes a cardiac-based seizure detection algorithm to detect seizure-induced tachycardia to trigger additional stimulation. There are some studies suggesting clinical benefits of rVNS over traditional VNS, but the performance and significance of autostimulation mode in clinical practice are poorly understood.ObjectivesTo assess the effect of initiation of rVNS therapy and altered stimulation settings on the number of daily stimulations and energy consumption in VNS therapy and to compare autostimulation performance in different epilepsy types.Materials and MethodsRetrospective follow-up of 30 patients with drug-resistant epilepsy treated with rVNS including 17 new implantations and 13 battery replaces at a single center in Finland. Our data consist of 208 different stimulation periods, that is, episodes with defined stimulation settings and both autostimulation and total stimulation performance-related data along with clinical follow-up.ResultsThe variation in autostimulation frequency was highly dependent on the duration of the OFF-time and autostimulation threshold (p < 0.05). There was a large additional effect of autostimulation mode on therapy time and energy consumption with longer OFF-times, but a minor effect with shorter OFF-times. Significantly more autostimulations were triggered in the temporal lobe and multifocal epilepsies than in extratemporal lobe epilepsies.ConclusionsThe initiation of autostimulation mode in VNS therapy increased the total number of stimulations. Shortening the OFF-time leads to a decreased number and share of automatic activations. Epilepsy type may affect autostimulation activity.     

Anterior Temporal Lobectomy and Medically Refractory Temporal Lobe Epilepsy of Childhood

The evaluation and outcome of 22 patients who had onset of complex partial seizures (CPS) of temporal lobe origin in childhood and subsequently underwent anterior temporal lobectomy are described. All patients showed improved seizure control; 81.8% had a reduction greater than or equal to 95% in seizure frequency. However, many patients had difficulty adjusting to a seizure-free life. Psychosocial, behavioral, and educational problems occurred more frequently in patients whose surgery was delayed until adult life. We conclude that attempts should be made early in the course of CPS of childhood to determine whether the seizures are truly intractable to medical management so that surgical intervention can be expedited.