Treatment and long-term prognosis of myoclonic-astatic epilepsy of early childhood

PURPOSE: We retrospectively studied patients with myoclonic-astatic epilepsy of early childhood (MAE) to investigate the most effective treatment and long-term seizure and intellectual prognosis. SUBJECTS: Eighty-one patients with MAE were recruited from among 3600 patients with childhood epilepsy according to the ILAE criteria of MAE. METHODS: We retrospectively investigated the clinical characteristics and ultimate prognosis of the patients with MAE from the medical records. The effects of various antiepileptic drugs, ketogenic diet and ACTH treatments on myoclonic-astatic seizures (MS/AS), apparently a hallmark of this unique epileptic syndrome, were also studied. RESULTS: MS/AS in 89 % of the patients disappeared within 1 to 3 years despite initial resistance, but generalized tonic-clonic or clonic seizures [G(T)CS] tended to continue. The most effective treatment for the MS/AS was ketogenic diet, followed by ACTH and ESM. At the last follow-up, 55 patients or 68 % of all the patients had remission of epilepsy, 11 patients or 14 % experienced a recurrence of GTCS after a long remission period but easily regained control, and the remaining 15 patients or 18 % continued to have seizures and intellectual outcomes were poor. In one half of these patients with poor outcomes, repeated minor epileptic status and nocturnal generalized tonic seizures persisted. A family history of epilepsy and a combination of minor epileptic status are risk factors for poor outcomes. CONCLUSION: MAE is considered to form a clinical spectrum ranging in its main seizure type from myoclonic to atonic, and in seizure and intellectual outcomes from benign to malignant. The overall prognosis, despite initial resistance to treatment, appears to be much better than originally thought when ILAE definitions excluding SME are followed.     

A population-based study of long-term outcomes of cryptogenic focal epilepsy in childhood: cryptogenic epilepsy is probably not symptomatic epilepsy

Purpose: To compare long‐term outcome in a population‐based group of children with cryptogenic versus symptomatic focal epilepsy diagnosed from 1980 to 2004 and to define the course of epilepsy in the cryptogenic group. Methods: We identified all children residing in Olmsted County, MN, 1 month through 17 years, with newly diagnosed, nonidiopathic focal epilepsy from 1980 to 2004. Children with idiopathic partial epilepsy syndromes were excluded. Medical records were reviewed to determine etiology, results of imaging and EEG studies, treatments used, and long‐term outcome. Children were defined as having symptomatic epilepsy if they had a known genetic or structural/metabolic etiology, and as cryptogenic if they did not. Key Findings: Of 359 children with newly diagnosed epilepsy, 215 (60%) had nonidiopathic focal epilepsy. Of these, 206 (96%) were followed for >12 months. Ninety‐five children (46%) were classified as symptomatic. Median follow‐up from diagnosis was similar in both groups, being 157 months (25%, 75%: 89, 233) in the cryptogenic group versus 134 months (25%, 75%: 78, 220) in the symptomatic group (p = 0.26). Of 111 cryptogenic cases, 66% had normal cognition. Long‐term outcome was significantly better in those with cryptogenic versus symptomatic etiology (intractable epilepsy at last follow‐up, 7% vs. 40%, p < 0.001; seizure freedom at last follow‐up, 81% vs. 55%, p < 0.001). Of those who achieved seizure freedom at final follow‐up, 68% of the cryptogenic group versus only 46% of the symptomatic group were off antiepileptic medications (p = 0.01). One‐third of the cryptogenic group had a remarkably benign disorder, with no seizures seen after initiation of medication, or in those who were untreated, after the second afebrile seizure. A further 5% had seizures within the first year but remained seizure‐free thereafter. With the exception of perinatal complications, which predicted against seizure remission, no other factors were found to significantly predict outcome in the cryptogenic group. Significance: More than half of childhood nonidiopathic localization‐related epilepsy is cryptogenic. This group has a significantly better long‐term outcome than those with a symptomatic etiology, and should be distinguished from it.     

A prospective long-term study of external trigeminal nerve stimulation for drug-resistant epilepsy

BackgroundExternal trigeminal nerve stimulation (eTNS) is an emerging noninvasive therapy for drug-resistant epilepsy (DRE). We report the long-term safety and efficacy of eTNS after completion of a phase II randomized controlled clinical trial for drug-resistant epilepsy.MethodsThis was a prospective open-label long-term study. Subjects who completed the phase II randomized controlled trial of eTNS for DRE were offered long-term follow-up for 1 year. Subjects who were originally randomized to control settings were crossed over to effective device parameters (30 s on, 30 s off, pulse duration of 250 s, frequency of 120 Hz). Efficacy was assessed using last observation carried forward or parametric imputation methods for missing data points. Outcomes included change in median seizure frequency, RRATIO, and 50% responder rate.ResultsThirty-five of 50 subjects from the acute double-blind randomized controlled study continued in the long-term study. External trigeminal nerve stimulation was well tolerated. No serious device-related adverse events occurred through 12 months of long-term treatment. At six and twelve months, the median seizure frequency for the original treatment group decreased by -2.39 seizures per month at 6 months (-27.4%) and -3.03 seizures per month at 12 months (-34.8%), respectively, from the initial baseline (p < 0.05, signed-rank test). The 50% responder rates at three, six, and twelve months were 36.8% for the treatment group and 30.6% for all subjects.ConclusionThe results provide long-term evidence that external trigeminal nerve stimulation is a safe and promising long-term treatment for drug-resistant epilepsy.     

伽玛刀治疗颞叶内侧癫的长期随访研究

目的探讨伽玛刀治疗颞叶内侧癫的长期疗效。方法回顾性分析应用伽玛刀治疗的46例顽固性颞叶内侧癫病人的临床资料。伽玛刀治疗范围包括杏仁核、海马及部分海马旁回,周边剂量17～20 Gy。结果随访46例,时间6～10年。术后5年按Engel分级:Ⅰ~Ⅱ级31例,Ⅲ级8例,Ⅳ级7例,有效率为67.4%。术后出现一过性头痛、记忆力下降20例,视野缺损12例,均在3~6个月内逐渐缓解。结论伽玛刀治疗相比传统开颅手术具有损伤轻、并发症少、长期有效率高等优点,是治疗颞叶内侧癫的方法之一。     

Prospective study on long-term treatment with oxcarbazepine in pediatric epilepsy

Following a previous preliminary report on a group of children suffering from partial epilepsies, we present the final considerations on the same group in order to evaluate the long-term efficacy, tolerability and safety of oxcarbazepine (OXC). We enrolled 36 patients (mean age 8.5), between January 2003 and December 2004, with new diagnosis of partial epilepsy: 25 patients were affected by idiopathic partial epilepsy, eight by symptomatic epilepsy and three by cryptogenic epilepsy. Each patient was scheduled to attend the center four times after the initial examination: 3 months (T1), 12 months (T2), 24 (T3) months and 36 (T4) months after the beginning of OXC-monotherapy (T0). At the end of our study, 20 patients were seizure free (SF): nine stopped OXC because of SF for at least 2 years, 11 were still on therapy. One patient showed a reduction of seizure frequency ≥50%, three were non responders (but still on therapy), nine stopped OXC due to a non-responder condition during follow-up before T4 and one because of adverse effects. At the end of the study no EEG focal abnormalities became generalized because of treatment. Normalization of EEG was observed in ten patients. Our preliminary findings have been confirmed. OXC can be considered an effective and well tolerated first line drug for long-term monotherapy in children with epilepsy, both for idiopathic and symptomatic/cryptogenic forms.     

The prognosis of epilepsy.

The cardinal question for a person developing seizures is ‘What is the likelihood that they will go away?’ ‘Prognosis’ refers to the possible outcomes of a disease and the frequency at which they can be expected to occur. Prognostic factors may include demographic features, disease-specific indicators (e.g. seizure frequency, aetiology of epilepsy) or co-morbidity. Such factors do not necessarily cause the outcome, but they are associated strongly with the outcome measured. They are distinct from risk factors—which are associated with the initial development of the disorder. Ideas about the outcome for epilepsy have been altered radically in the past century by study of its epidemiology. The prognosis for epilepsy comprises a number of measurable end-points: the prediction of recurrence after a single unprovoked seizure, the chance of remission after the diagnosis of epilepsy and the risk of premature death.     

A long-term follow-up study of schizophrenia: psychiatric course of illness and prognosis.

A systematic psychiatric follow-up study of 502 schizophrenics was carried out using the same well-defined criteria to evaluate the patients throughout the investigation. After an average course of disease of 22.4 years, 22.1% of the patients showed complete psychopathological remission, 43.2% had non-characteristic types of remission and 34.7% suffered from characteristic schizophrenic deficiency syndromes. At the time of the last follow-up investigation, 86.7% of the patients were living at home, while 13.3% were permanently hospitalized. Of the entire sample, 55.9% were found to be "socially recovered". Higher education, psychoreactive provocation, depressive traits, perception of delusions, catatonic agitation, non-characteristic thought disorders and symptoms of depersonalization at the onset of the illness tended to carry with them a favorable prognosis. On the other hand, low intelligence, abnormal primary personality, premorbid disturbances in social behavior, broken homes, prolonged prodromal stages, pneumoence-phalographically measurable atrophic or dysplastic changes in the brain ventricles as well as somatic and auditory hallucinations and predominance of hebephrenic symptoms at the onset of the illness tended to lead to an unfavorable prognosis. The principle of the basic reversibility of typical schizophrenic symptoms and the extensive irreversibility of the non-characteristic defect is important for the psychopathological and social long-term prognosis.     

The attention networks in benign epilepsy with centrotemporal spikes: A long-term follow-up study

PurposeTo evaluate the long-term prognosis of attention deficit in children with newly diagnosed benign childhood epilepsy with centrotemporal spikes (BECTS).MethodsAttention network test (ANT) was performed over a period of 7 years on 42 patients who were newly diagnosed with BECTS, in the Department of Neurology of Anhui Provincial Children's Hospital.ResultsIn the patients’ group, the accuracy of ANT was lower (P = 0.000), the total response time was longer (P = 0.000), and the efficiency of orienting (P = 0.000) and alerting (P = 0.041) networks was lower than that of the control group. Accuracy was positively correlated with age of onset (b = 1.184) and negatively correlated with number of seizures (b = −1.321). After 7 years, there was no significant difference in the accuracy (P = 0.385); total response time (P = 0.661); and alerting (P = 0.797), orienting (P = 0.709), and executive control (P = 0.806) network efficiencies between the patients and controls. Accuracy was positively correlated with age of onset (b = 0.8583) and negatively correlated with number of seizures (b = −1.017) and duration of antiepileptic drugs therapy (b = −3.203).ConclusionsIn our study, the newly diagnosed BECTS patients had impaired attention network, mainly in the alerting and orienting domains. Age of onset, number of seizures, and time of antiepileptic treatment may affect the attention networks. With the remission of BECTS, the attention network dysfunction was reversed.     

A clinical study on frontal lobe epilepsy

Of 30 patients whose epileptogenic focus was presumed to be located in the frontal lobe on the basis of a clinico-electrographic correlation, the principal seizure manifestation consisted of peculiar automatisms in about 40% of the cases. However, the nature of the automatisms was of a short duration lasting about one minute but in most cases it was accompanied by extremely violent movements, but this was not true in all cases. Of the 30 cases, only 5 cases showed marked improvement one year after being discharged from the hospital so that this type of seizure event is extremely intractable.     

内侧额叶癫痫发作的临床特征分析

目的 分析内侧额叶癫痫发作的临床特征。方法 回顾性分析10例经手术证实的内侧额叶起源的癫痫患者129次临床发作症状，脑电图表现，致痫灶定位及手术结果等临床资料。结果 内侧额叶癫痫发作的常见形式包括：过度运动发作、姿势性强直发作、额叶失神发作等，多在夜间发作，持续时间较短暂。10例均应用颅内电极进行长程记录定位致痫灶。致痫灶位于辅助运动区2例，内侧额回4例，额极1例，眶额区1例，扣带回1例，扣带回及额上回1例。按照Engel术后效果进行分级，Ⅰ级3例，Ⅱ级2例，Ⅲ级3例，2例随访时间未满1年。结论 内侧额叶癫痫发作具有一定的特征性。普通头皮脑电图定位致痫灶常较困难，需要应用颅内电极。手术切除致痫灶效果较满意。     

The prognosis of idiopathic generalized epilepsy

Prognosis describes the trajectory and long‐term outcome of a condition. Most studies indicate a better prognosis in idiopathic generalized epilepsy (IGE) in comparison with other epilepsy syndromes. Studies looking at the long‐term outcome of different IGE syndromes are relatively scant. Childhood absence epilepsy appears to have a higher rate of remission compared to juvenile absence epilepsy. In absence epilepsies, development of myoclonus and generalized tonic–clonic seizures predicts lower likelihood of remission. Although most patients with juvenile myoclonic epilepsy (JME) achieve remission on antiepileptic drug therapy, <20% appear to remain in remission without treatment. Data on the prognosis of other IGE syndromes are scarce. There are contradictory findings reported on the value of electroencephalography as a predictor of prognosis. Comparisons are made difficult by study heterogeneity, particularly in methodology and diagnostic criteria.     

Lifetime prognosis of juvenile myoclonic epilepsy

Juvenile myoclonic epilepsy (JME) is among the most common types of genetic epilepsies, displaying a good prognosis when treated with appropriate drugs, but with a well-known tendency to relapse after withdrawal. The majority of patients with JME have continuing seizures after a follow-up of two decades. However, 17% are able to discontinue medication and remain seizure-free thereafter. Clinicians should remember that there is a small but still considerable subgroup of JME patients whose seizures are difficult to treat before informing patients with newly-diagnosed JME about their “benign” prognosis. This resistant course is not fully explained, though there are many suggested factors. The dominating myoclonic seizures disappear or diminish in severity in the fourth decade of life. Despite the favorable seizure outcome in most of the cases, 3/4 of patients with JME have at least one major unfavorable social outcome. The possible subsyndromes of JME, its genetic background, and its pathophysiological and neuroimaging correlates should be further investigated.This article is part of a supplemental special issue entitled Juvenile Myoclonic Epilepsy: What is it Really?     

Update on the epidemiology and prognosis of pediatric epilepsy

Epilepsy is among the most common serious neurologic disorders in childhood. Epidemiologic studies over the past few decades have greatly increased current knowledge of the incidence and prognosis of seizures. Newer epidemiologic studies have used population- or community-based cohorts, and careful attention has been given to etiology and specific epilepsy syndromes, the two most important factors affecting prognosis. Risk of epilepsy is highest in patients with an associated serious neurologic abnormality, such as mental retardation or cerebral palsy. More than two thirds of patients with childhood-onset epilepsy ultimately achieve remission. Of those attaining remission on medications, approximately 70% remain seizure free when medications are discontinued. Mortality is increased in patients with epilepsy, but the increased mortality risk in childhood-onset epilepsy is primarily seen in patients with neurologic abnormalities or intractable epilepsy. Although long-term seizure outcomes are generally favorable, childhood-onset epilepsy is associated with adverse long-term psychosocial outcomes, even in patients attaining remission. This review summarizes recent data on the epidemiology and prognosis of pediatric epilepsy.