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1.
PurposeEpilepsy with electrical status epilepticus in sleep (ESES) is a devastating disease, and we sought to evaluate the efficacy of levetiracetam (LEV) for the treatment of patients with this epileptic encephalopathy in China.MethodsClinical data from all patients with ESES who received LEV therapy at our pediatric neurology outpatient clinic between 2007 and 2014 (n = 71) were retrospectively analyzed. The LEV dosage was 30–50 mg/kg/day. Electroencephalography recordings and neuropsychological evaluations were performed repeatedly for 3–75 months after the start of LEV therapy.ResultsThirty-five (70%) of 50 patients who had seizures at the start of LEV therapy had a > 50% reduction in seizure frequency. Positive response on EEG was found during the first 3–4 months of LEV therapy in 32 (45%) of 71 patients, with normalization of EEG in 5 patients. Relapse occurred in 8 (25%) of the initial electrical responders. Hence, 47 patients (66%) still suffered from ESES and only 13 patients regained their baseline level of function at the last follow-up. The response to LEV was significantly associated with ESES duration, age at onset of ESES, and etiology of epilepsy. Although fatigue and anorexia were the primary adverse events, LEV was well-tolerated by all patients.ConclusionsLevetiracetam is safe and may be efficient when used to treat ESES syndrome; however, the efficacy EEG neuropsychological outcomes is limited on the whole. 相似文献
2.
Spectrum of epileptic syndromes with electrical status epilepticus during sleep in children 总被引:2,自引:0,他引:2
There has been much debate about the nosologic forms of electrical status epilepticus during sleep (ESES) that can occur in a number of syndromes. The pathogenesis of ESES is unknown, and the natural course is variable. It is debatable whether these age-specific epileptic syndromes belong to the same spectrum of disorders with different severity but a common denominator of sleep-related hypersynchronization of generalized paroxysmal epileptic discharges. This report describes 18 children with medically refractory seizures, gradual deterioration in language skills, fine-motor incoordination, behavioral changes, psychologic and intellectual regression of different degrees, and the ESES phenomenon. Most exhibited clinical and electroencephalographic responses to intravenous or oral benzodiazepines, especially if initiated within the first 2 years of seizure onset. Seizure remission was nearly complete with cessation of seizures and marked improvement in language and fine-motor skills, behavior, and intellectual function in those with an idiopathic etiology. Therapeutic trials with benzodiazepines should be given to all children with the ESES phenomenon. Sleep electroencephalographic monitoring is recommended in all young children with epilepsy and language or psychologic deterioration so that the brain dysfunction can be reversed at a critical and vulnerable period of early life. 相似文献
3.
儿童睡眠中癫(癎)性电持续状态36例临床分析 总被引:1,自引:0,他引:1
目的:总结儿童睡眠中癫(癎)性电持续状态(ESES)的临床、脑电图(EEG)、神经心理障碍的特点及对治疗的反应.方法:对36例ESES患儿进行V-EEG监测,随访观察临床、EEG、神经心理障碍的情况以及治疗效果.结果:36例中27例(75%)经治疗临床发作完全控制或明显减少,EEG上ESES消失.22例使用肾上腺皮质激素甲基泼尼松龙冲击治疗中的17例(77%)在临床发作改善和抑制(癎)样放电方面都有良好的疗效.32例神经心理障碍患儿26例(81%)经治疗后有明显改善.结论:ESES是一种特殊的EEG现象,非快速眼动(NREM)睡眠期持续放电是神经心理障碍的主要原因.抗癫(癎)治疗除控制其临床癫(癎)发作外,还必须及早消除EEG(癎)样放电持续状态. 相似文献
4.
Bahi-Buisson N Savini R Eisermann M Bulteau C Dulac O Hertz-Pannier L Chiron C 《Pediatric neurology》2006,34(2):146-150
Electrical status epilepticus during sleep syndrome and its variants are age-dependent epileptic encephalopathies associated with a sleep-related electroencephalographic pattern of continuous spike-waves, combined with motor or cognitive impairment. These epileptic encephalopathies are usually not responsive to conventional antiepileptic drugs. This report describes two children in whom clonazepam had no effect on cognitive and motor disorders but controlled spike activity, preventing a proper diagnosis. Withdrawal of clonazepam was accompanied by the recurrence of continuous spike-waves in slow sleep, permitting the diagnosis of electrical status epilepticus during sleep syndrome and appropriate therapeutic decisions. These two cases of the misleading effect of clonazepam in electrical status epilepticus during sleep syndrome illustrate the puzzling situation that can occur when therapeutic options only consider the electroencephalographic features without prior syndromic diagnosis. 相似文献
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Kevelam SH Jansen FE Binsbergen Ev Braun KP Verbeek NE Lindhout D Poot M Brilstra EH 《Journal of child neurology》2012,27(2):178-182
Electrical status epilepticus in sleep syndrome is the association of the electroencephalographic pattern and deficits in language or global cognitive function and behavioral problems. The etiology is often unknown, but genetic risk factors have been implicated. Array-based comparative genomic hybridization was used to identify copy number variations in 13 children with electrical status epilepticus in sleep syndrome to identify possible underlying risk factors. Seven copy number variations were detected in 4 of the 13 patients, which consisted of 6 novel gains and 1 loss, the recurrent 15q13.3 microdeletion. Two patients carried a probable pathogenic copy number variation containing a gene involved in the cholinergic pathway. Genetic aberrations in patients with electrical status epilepticus in sleep syndrome can provide an entry in the investigation of the etiology of electrical status epilepticus in sleep. However, further studies are needed to confirm our findings. 相似文献
6.
Clinical spectrum and medical treatment of children with electrical status epilepticus in sleep (ESES) 总被引:1,自引:0,他引:1
Uri Kramer Liora Sagi †Hadassa Goldberg-Stern ‡Nathanel Zelnik §Andreea Nissenkorn §Bruria Ben-Zeev 《Epilepsia》2009,50(6):1517-1524
Purposes: To describe the clinical spectrum and to evaluate the efficacy of different therapeutic agents in children with electrical status epilepticus in sleep (ESES).
Methods: Clinical data of all patients with ESES (not including patients with Landau-Kleffner syndrome) in four pediatric neurology outpatient clinics were analyzed. Thirty patients with ESES had been treated between 1994 and 2007.
Results: Eleven (37%) children had benign partial epilepsies of childhood, five (17%) had cerebral palsy, five (17%) had hydrocephalus, one (3%) had schizencephaly, one (3%) had prenatal parenchymal bleeding, and the etiology was unclear in seven (23%). The duration of ESES ranged between 2 and 60 months. The antiepileptic drugs that were found to be efficacious were: levetiracetam (41%), clobazam (31%), and sulthiame (17%). Valproic acid, lamotrigine, topiramate, and ethosuximide showed no efficacy. Steroids were efficacious in 65%; immunoglobulins were efficacious in 33%. High-dose diazepam was efficacious in 37%, but all the children had temporary response. Seventeen patients (57%) had cognitive deterioration, whereas the rest presented with regression in attention, speech, communication, and behavior. Fourteen children had permanent cognitive deficit. There was a significant correlation (p = 0.029) between the duration of ESES and residual intellectual deficit at follow-up.
Conclusions: ESES reflects an evolution of benign partial epilepsy of childhood in more than one-third of the patients, whereas there is an underlying structural brain anomaly in another one-third. The most efficacious antiepileptic drugs (AEDs) are levetiracetam and clobazam. The duration of ESES correlated significantly with residual intellectual deficit at follow-up. 相似文献
Methods: Clinical data of all patients with ESES (not including patients with Landau-Kleffner syndrome) in four pediatric neurology outpatient clinics were analyzed. Thirty patients with ESES had been treated between 1994 and 2007.
Results: Eleven (37%) children had benign partial epilepsies of childhood, five (17%) had cerebral palsy, five (17%) had hydrocephalus, one (3%) had schizencephaly, one (3%) had prenatal parenchymal bleeding, and the etiology was unclear in seven (23%). The duration of ESES ranged between 2 and 60 months. The antiepileptic drugs that were found to be efficacious were: levetiracetam (41%), clobazam (31%), and sulthiame (17%). Valproic acid, lamotrigine, topiramate, and ethosuximide showed no efficacy. Steroids were efficacious in 65%; immunoglobulins were efficacious in 33%. High-dose diazepam was efficacious in 37%, but all the children had temporary response. Seventeen patients (57%) had cognitive deterioration, whereas the rest presented with regression in attention, speech, communication, and behavior. Fourteen children had permanent cognitive deficit. There was a significant correlation (p = 0.029) between the duration of ESES and residual intellectual deficit at follow-up.
Conclusions: ESES reflects an evolution of benign partial epilepsy of childhood in more than one-third of the patients, whereas there is an underlying structural brain anomaly in another one-third. The most efficacious antiepileptic drugs (AEDs) are levetiracetam and clobazam. The duration of ESES correlated significantly with residual intellectual deficit at follow-up. 相似文献
7.
Steven H. Mott Richard P. Morse Scott A. Burroughs Ashura W. Buckley Cristan A. Farmer Audrey E. Thurm Susan E. Swedo Amara L. Krag Gregory L. Holmes 《Epileptic Disord》2019,21(1):55-64
Aims. Electrical status epilepticus in sleep (ESES) is an age‐related, self‐limited epileptic encephalopathy. The syndrome is characterized by cognitive and behavioral abnormalities and a specific EEG pattern of continuous spikes and waves during slow‐wave sleep. While spikes and sharp waves are known to result in transient cognitive impairment during learning and memory tasks performed during the waking state, the effect of epileptiform discharges during sleep on cognition and behavior is unclear. There is increasing evidence that abnormalities of coherence, a measure of the consistency of the phase difference between two EEG signals when compared over time, is an important feature of brain oscillations and plays a role in cognition and behavior. The objective of this study was to determine whether coherence of EEG activity is altered during slow‐wave sleep in children with ESES when compared to typically developing children. Methods. We examined coherence during epochs of ESES versus epochs when ESES was not present. In addition, we compared coherence during slow‐wave sleep between typically developing children and children with ESES. Results. ESES was associated with remarkably high coherences at all bandwidths and most electrode pairs. While the high coherence was largely attributed to the spikes and spike‐and‐wave discharge, activity between spikes and spike‐and‐wave discharge also demonstrated high coherence. Conclusions. This study indicates that EEG coherence during ESES is relatively high. Whether these increases in coherence correlate with the cognitive and behavioral abnormalities seen in children with this EEG pattern remains to be determined. 相似文献
8.
The results of long-term follow-up of 10 children with global or specific cognitive deterioration and, on the electroencephalogram, electrical status epilepticus during sleep (ESES) are described. They were referred because of cognitive deterioration and underwent repeated neurological and neuropsychological examinations and all-night electroencephalography. A previous cognitive level was known or could be estimated in all. Seven children had a continuous spikes and waves during sleep (CSWS) syndrome, with global cognitive deterioration in four and more specific cognitive decline in three, and another three children had Landau-Kleffner syndrome (LKS). Of the last three, two children never had seizures, while the other had localization-related epilepsy. No children experienced aggravation of clinical seizures. However, therapy was disappointing. Cognitive dysfunction did not respond to valproate and/or benzodiazepines in 9 of the 10 children. A frontal epileptic focus was found in 5 of 7 children with CSWS, and a left temporal focus in 2 of 3 children with LKS. The ESES persisted in CSWS for 5-9 years and in LKS for 1-5 years, and disappeared at puberty. Good cognitive recovery after disappearance of ESES occurred in only one child, and partial recovery in four. An unfavorable prognosis of cognitive deterioration seems to be related to long-duration ESES and/or early onset epileptic activity. The authors are of the opinion that cognitive deterioration in children, with or without manifest epileptic seizures, should mandate electroencephalographic investigation during sleep. 相似文献
9.
Praline J Barthez MA Castelnau P Debiais S Lucas B Billard C Piller AG de Becque B de Toffol B Autret A Hommet C 《Journal of the neurological sciences》2006,249(2):166-171
We report the case of a young girl who presented severe learning disabilities in oral and written language related to a continuous spike-waves during slow sleep (CSWS) syndrome. A sleep EEG recording obtained in her younger brother, who presented a clinical pattern suggesting developmental dysphasia, also showed a CSWS syndrome. These two clinical cases underscore the need to look for this syndrome in the siblings of an affected child when learning difficulties appear in a child who previously had normal psychomotor development. 相似文献
10.
Non-linear EEG analysis in children with epilepsy and electrical status epilepticus during slow-wave sleep (ESES) 总被引:2,自引:0,他引:2
Raffaele Ferri Maurizio Elia Sebastiano A. Musumeci Cornelis J. Stam 《Clinical neurophysiology》2001,112(12):247-2280
OBJECTIVE: The objective of this work was to study the non-linear aspects of electroencephalography (EEG) in children with epilepsy and electrical status epilepticus during slow-wave sleep (ESES). METHODS: In this study, we recorded the sleep EEG in 5 subjects with ESES (4 males and one female, aged 6.5-10 years) who were also mentally retarded and affected by cerebral palsy (3 subjects) and hydrocephalus (two subjects). The signals were sampled at 128Hz and stored on hard disk. All the subsequent computational steps were performed on EEG epochs (4096 data points) selected from wakefulness and non-rapid eye movement (non-REM) (with ESES) or REM sleep. The dynamic properties of the EEG were assessed by means of the non-linear cross prediction (NLCP) test which uses 3 different 'model' time series in order to predict non-linearly the original data set (Pred, Ama and Tir). Pred is a measure of the predictability of the time series and Ama and Tir are measures of asymmetry, indicating non-linear structure. Moreover, the correlation dimension (D2) was estimated by means of the algorithm by for the epochs showing non-linear nature. RESULTS: The NLCP test provided evidence of significant non-linear dynamics in all epochs of non-REM sleep, when ESES was evident. Only during this stage, the possible presence of low-dimensional chaos could also be suspected (average D2=4.02; range 3.16-6.21). EEG without ESES could not be distinguished from linearly filtered noise. CONCLUSIONS: The results of the present study seem to indicate that subjects with ESES show a profound modification of their EEG dynamics with the occurrence, during sleep, of long periods characterized by non-linear dynamics and, probably, low-dimensional chaotic structure able to modify in a substantial way their brain functioning during sleep. 相似文献
11.
Serum inflammatory mediators correlate with disease activity in electrical status epilepticus in sleep (ESES) syndrome
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Kees P. J. Braun H. Myrthe Boss Michèl A. Willemsen Annet van Royen‐Kerkhof Floor E. Jansen 《Epilepsia》2016,57(2):e45-e50
We aimed to study serum cytokine levels in 11 electrical status epilepticus in sleep (ESES) patients and 20 healthy control children. Patients showed significantly higher levels of interleukin (IL)‐1α, IL‐6, IL‐10, chemokine (C‐C motif) ligand (CCL)2 and chemokine (C‐X‐C motif) ligand (CXCL)8/IL‐8 than controls, while macrophage migration inhibitory factor (MIF) and CCL3 were significantly lower. Follow‐up analyses in five patients revealed a significant decrease of IL‐6 levels after immunomodulating treatment. IL‐6 changes were accompanied by clear improvement of electroencephalography (EEG) patterns and neuropsychological evaluation. We hypothesize that IL‐6 correlates with disease activity and immunomodulating treatment efficacy. 相似文献
12.
Peltola ME Liukkonen E Granström ML Paetau R Kantola-Sorsa E Valanne L Falck B Blomstedt G Gaily E 《Epilepsia》2011,52(3):602-609
Purpose: We analyzed clinical and electroencephalography (EEG) outcomes of 13 patients with pharmacoresistant encephalopathy with electrical status epilepticus during sleep (ESES) following epilepsy surgery. Methods: All patients had symptomatic etiology of ESES and preoperative neuropsychological deterioration. Ten patients had daily atypical absences. Clinical outcome was assessed at 6 months and at 2 years after surgery. Clinical and EEG data were reviewed retrospectively. The spike propagation pattern and area and source strength in source montage were analyzed from preoperative and postoperative EEG studies. Key Findings: Preoperative sleep EEG showed electrical status epilepticus during sleep (SES) with one‐way interhemispheric propagation in nine patients and with two‐way interhemispheric propagation in four. The age of the patients at the time of surgery ranged from 3.6–9.9 years. Focal resection (two patients) or hemispherotomy (one patient with postoperative EEG) either terminated SES or restricted the discharge to one region. Either reduced SES propagation area or source strength was found in four of eight callosotomy patients with postoperative EEG. Of patients who had seizures preoperatively, Engel class I–II seizure outcome was observed in two of three children after focal resection or hemispherotomy and in two of eight children after callosotomy. None of these patients with Engel class I–II outcome had SES with two‐way interhemispheric propagation on preoperative EEG. Cognitive deterioration was halted postoperatively in all except one patient. Cognitive catch‐up of more than 10 IQ points was seen in three patients, all of whom had shown a first measured IQ of >75. Significance: Patients with pharmacoresistant ESES based on symptomatic etiology may benefit from resective surgery or corpus callosotomy regarding both seizure outcome and cognitive prognosis. 相似文献
13.
A 4-year-old female patient with epilepsy with continuous spike-and-waves during slow-wave sleep not classified as Landau-Klefner syndrome, refractory to antiepileptic drugs including valproate, benzodiazepines, and lamotrigine, was treated successfully with high-dose intravenous methylprednisolone therapy. Valproate, clobazam, and lamotrigine were continued at the same dose during and after high-dose intravenous corticosteroid therapy. During corticosteroid therapy, awake and sleep electroencephalogram was recorded every day. On day 7, a dramatic clinical and electroencephalographic response was observed. After high-dose intravenous methylprednisolone, prednisolone was administered orally (2 mg/kg daily) for 2 months, then gradually withdrawn. After the withdrawal of corticosteroid therapy, the patient maintained the clinical improvement in behavior, and no continuous spike-and-wave electrical status epilepticus during slow-wave sleep occurred on routine monthly sleep electroencephalogram performed for the last 6 months. In the present case, an add-on high-dose intravenous corticosteroid seems to be effective in the treatment of patients with electrical status epilepticus during slow-wave sleep syndrome, especially when antiepileptic drugs fail. 相似文献
14.
The spectrum of neuropsychiatric abnormalities associated with electrical status epilepticus in sleep 总被引:7,自引:0,他引:7
Electrical status epilepticus in sleep (ESES) is an electrographic pattern consisting of an almost continuous presence of spike-wave discharges in slow wave sleep. ESES is frequently encountered in pediatric syndromes associated with epilepsy or cognitive and language dysfunction. It can be present in various evolutionary stages of a spectrum of diseases, the prototypes of which are the 'continuous spikes and waves during slow wave sleep' syndrome (CSWS), the Landau-Kleffner syndrome (LKS), as well as in patients initially presenting as benign childhood epilepsy with centrotemporal spikes (BECTS). The purpose of this article is to review the literature data on the semiology, electrographic findings, prognosis, therapeutic options, as well as the current theories on the pathophysiology of these disorders. The frequent overlap of CSWS, LKS, and BECTS urges an increased level of awareness for the occasional transition from benign conditions such as BECTS to more devastating syndromes such as LKS and CSWS. Identification of atypical signs and symptoms, such as high discharge rates, prolonged duration of ESES, neuropsychiatric and cognitive dysfunction, lack of responsiveness to medications, and pre-existing neurologic conditions is of paramount importance in order to initiate the appropriate diagnostic measures. Prolonged and if needed repetitive sleep electroencephalographs (EEGs) are warranted for proper diagnosis. 相似文献
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Anthony L. Fine Elaine C. Wirrell Lily C. Wong‐Kisiel Katherine C. Nickels 《Epilepsia》2015,56(9):e134-e138
Electrical status epilepticus in slow‐wave sleep (ESES) is characterized by nearly continuous spike–wave discharges during non–rapid eye movement (REM) sleep. ESES is present in Landau‐Kleffner syndrome (LKS) and continuous spike and wave in slow‐wave sleep (CSWS). Sulthiame has demonstrated reduction in spike–wave index (SWI) in ESES, but is not available in the United States. Acetazolamide (AZM) is readily available and has similar pharmacologic properties. Our aims were to assess the effect of AZM on SWI and clinical response in children with LKS and CSWS. Children with LKS or CSWS treated with AZM at our institution were identified retrospectively. Pre‐ and posttherapy electroencephalography (EEG) studies were evaluated for SWI. Parental and teacher report of clinical improvement was recorded. Six children met criteria for inclusion. Three children (50%) demonstrated complete resolution or SWI <5% after AZM. All children had improvement in clinical seizures and subjective improvement in communication skills and school performance. Five of six children had subjective improvement in hyperactivity and attention. AZM is a potentially effective therapy for children with LKS and CSWS. This study lends to the knowledge of potential therapies that can be used for these disorders, which can be challenging for families and providers. 相似文献
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Landau-Kleffner syndrome (LKS): long-term follow-up and links with electrical status epilepticus during sleep (ESES) 总被引:4,自引:0,他引:4
We describe 11 patients affected by Landau-Kleffner syndrome (LKS) with a mean follow-up of 9 years and 8 months. EEG recordings during wakefulness, NREM and REM sleep showed a bitemporal electrical status epilepticus during sleep (BTESES) in all cases; four of them presented a shift from a BTESES towards an 'intercalated electrical status epilepticus during sleep' (IESES) accompanied by a global regression of cognitive and behavioural functions in 3/4 of cases. At the last observation, only 18.2% of cases presented a complete language recovery and mental retardation was evident in 63.6%. The prognosis of LKS in our cases may depend on the interaction of different negative factors such as onset of aphasia before 4 years, its duration for longer than 1 year, long-lasting duration and continuity without fluctuations of BTESES/IESES, probably preexisting mild speech delay. It is important for the prognosis to utilize antiepileptic treatment and possibly neurosurgical techniques to eliminate EEG paroxysmal abnormalities. At present, no similar cases with clinical-EEG evolution from LKS to electrical status epilepticus during sleep (ESES) have ever been described. Our observation demonstrates that LKS and ESES classified as different clinical-EEG syndromes represent two aspects of the same brain dysfunction and they may exist separately or pass one into the other with a change in the clinical-EEG picture. The common origin of the two syndromes is confirmed by recent functional brain imaging, neurophysiological and neurosurgical techniques. 相似文献
20.
Treatment of epilepsy with electrical status epilepticus during slow sleep and its related disorders
To elucidate an effective therapeutic strategy for 'ESES syndrome', epilepsy with electrical status epilepticus during slow sleep (ESES) and its related epileptic disorders, we studied the effect of treatment on the EEG pattern of continuous spike-waves during slow wave sleep (CSWS) in 15 afflicted patients. Basically performed in the following order, the employed therapies included (1) high-dose valproate (VPA) therapy (serum level >100 microg/ml); (2) a combination therapy of VPA and ethosuximide (ESM); (3) short cycles of high-dose diazepam (oral or intrarectal DZP, 0.5-1 mg/kg per day for 6-7 days); and (4) intramuscular synthetic ACTH-Z therapy (0.01-0.04 mg/kg per day for 11-43 days). Regarding the initial EEG effect, a remission of CSWS was achieved by high-dose VPA therapy in 7 of 15 trials (47%), by the combination therapy of VPA and ESM in 3/7 trials (43%), by short cycles of high-dose DZP in 2/4 trials (50%), and by ACTH-Z therapy in 2/5 trials (40%). A permanent remission of ESES syndrome was achieved by high-dose VPA therapy and/or combination therapy of VPA and ESM in 10 patients (67%). The effects of short cycles of high-dose DZP and ACTH-Z therapy were at best temporary. Our strategy for the treatment of ESES syndrome is therefore considered valid. 相似文献