A Multicenter Study of Self-Limited Epilepsy With Centrotemporal Spikes: Effectiveness of Antiseizure Medication With Respect to Spike-Wave Index.

BACKGROUND: There is no certain validated electroencephalographic (EEG) parameters for outcome prediction in children with self-limited epilepsy with centrotemporal spikes. To assess the effectiveness of antiseizure medication (ASM) for seizure outcome with respect to the spike-wave index (SWI) on serial EEG recordings. METHODS: In this multicenter study, the study cohort consisted of 604 children with self-limited epilepsy with centrotemporal spikes. A data set of epilepsy centers follow-up between 2010 and 2022. The cohort was divided into 4 groups as those receiving 3 different monotherapy (carbamazepine [CBZ]/valproic acid [VPA]/levetiracetam [LEV]) and dual therapy. SWI analysis was performed with the percent of spikes in the 2-minute epoch in the 5th 6th minutes of the nonrapid eye movement sleep EEG record. The study group were also categorized according to seizure burden with seizure frequency (I) >2 seizures and (II) >5 seizures. Seizure outcome was evaluated based on the reduction in seizure frequency over 6-month periods: (1) 50% reduction and (2) seizure-free (complete response). RESULTS: ASM monotherapy was achieved in 74.5% children with VPA, CBZ, and LEV with similar rates of 85.8%, 85.7%, and 77.9%. Dual therapy was need in the 25.5% of children with SeLECT. More dual therapy was administered in children aged below 5 years with a rate of 46.2%. Earlier seizure-free achievement time was seen in children with LEV monotherapy with more complete-response rate (86.7%) compared the VPA and CBZ. CONCLUSIONS: We also determined that the children on dual therapy had more SWI clearance in the subsequent EEG recordings. The ROC curve analyses were performed to predict initial drug selection with using the SWI% might be used for the prediction of ASM type and drug selection in children.

The prospective study of 54 children with electrical status epilepticus during sleep: How to simplify the electroencephalogram diagnosis and guide the treatment.

OBJECTIVE: To simplify the electroencephalogram (EEG) diagnosis and guide the treatment of electrical status epilepticus during sleep (ESES). METHODS: We recruited 54 children with ESES from December 2019 to December 2020 and compared various spike-wave index (SWI) calculation methods. Time-frequency analysis assessed the correlation between high-frequency oscillations energy and the SWI. We divided 42 children into responder and non-responder treatment groups based on the observations made during a 12-month follow-up period and evaluate different treatment and the independent risk factors of refractory ESES. RESULTS: The SWI of 5 min before the second sleep cycle of non-rapid eye movement (NREM; long method II) and that of all NREM sleep (total method) were not significantly different (p = .06). The average energy of γ (r = .288, p = .002) and ripple (r = .203, p = .04) oscillations were correlated with the SWI. Multivariable logistic regression analysis showed that encephalomalacia was an independent risk factor for refractory ESES (OR: 10.48, 95% CI: 1.62-67.63). The clinical seizure improvement rates of anti-seizure medications (ASMs), ASMs with benzodiazepines, and ASMs with benzodiazepines and steroids after 12 months were 9.3%, 42.8%, and 53.8%, EEG improvement rate were 5.5%, 30.9% and 37%, respectively. The intelligence of the children in the responder treatment group has improved during the 1-year follow-up. SIGNIFICANCE: These findings demonstrate EEG and clinical features of ESES and may provide basis for simplifying diagnosis and guiding the treatment of children with ESES.

Is sleep captured during a standard daytime EEG sufficient to diagnose Electrical Status Epilepticus in Sleep.

Electrical Status epilepticus of sleep (SES) is an EEG pattern where there is significant activation of epileptiform activity in NREM sleep. A spike wave index (SWI) of > 80-85% is often labelled as typical SES. We aimed to explore if sleep during a standard daytime-EEG, as compared an overnight-EEG, was adequate to diagnose ESES. Ten children with daytime and overnight studies suggestive of SES were audited. SWI and Spike Wave Density (SWD) were calculated for 5-minute epochs of wake in the daytime and overnight study, as well daytime-EEG sleep and first and last NREM cycle in the overnight-EEG. SWI in daytime NREM was not significantly different from SWI in the first sleep cycle of the overnight study. SWI in the last sleep cycle was significantly lower than the first sleep cycle in the overnight-EEG. SWD was significantly higher in the first sleep cycle in the overnight-EEG than the daytime sleep and the last NREM cycle. SES may be diagnosed in NREM sleep from a daytime-EEG study. Larger studies are needed to explore the significance of the disparity between SWI and SWD in the first and last NREM cycles in the overnight study.

Effects of altered excitation-inhibition imbalance by repetitive transcranial magnetic stimulation for self-limited epilepsy with centrotemporal spikes.

Objectives: Patients with self-limited epilepsy with centrotemporal spikes (SeLECTS) with electrical status epilepticus in sleep (ESES) have generalized cognitive impairment, yet treatment options are limited. Our study aimed to examine the therapeutic effects of repetitive transcranial magnetic stimulation (rTMS) on SeLECTS with ESES. In addition, we applied electroencephalography (EEG) aperiodic components (offset and slope) to investigate the improvement of rTMS on the excitation-inhibition imbalance (E-I imbalance) in the brain of this group of children. Methods: Eight SeLECTS patients with ESES were included in this study. Low-frequency rTMS (≤1 Hz) was applied for 10 weekdays in each patient. To assess the clinical efficacy and changes in E-I imbalance, EEG recordings were performed both before and after rTMS. Seizure-reduction rate and spike-wave index (SWI) were measured to investigate the clinical effects of rTMS. The aperiodic offset and slope were calculated to explore the effect of rTMS on E-I imbalance. Results: Five of the eight patients (62.5%) were seizure-free within 3 months after stimulation, with treatment effects decreasing with longer follow-ups. The SWI decreased significantly at 3 and 6 months after rTMS compared with the baseline (P = 0.0157 and P = 0.0060, respectively). The offset and slope were compared before rTMS and within 3 months after stimulation. The results showed a significant reduction in the offset after stimulation (P < 0.0001). There was a remarkable increase in slope after the stimulation (P < 0.0001). Conclusion: Patients achieved favorable outcomes in the first 3 months after rTMS. The ameliorative effect of rTMS on SWI may last up to 6 months. Low-frequency rTMS could reduce firing rates in neuronal populations throughout the brain, which was most pronounced at the site of stimulation. A significant reduction in the slope after rTMS treatment suggested an improvement in the E-I imbalance in the SeLECTS.

The fate of spikes in self-limited epilepsy with centrotemporal spikes: Are clinical and baseline EEG features effective?

OBJECTIVE: The aim of this study is to evaluate the effects of clinical and electroencephalographic features on spike reduction with a focus on the first EEG characteristics in self-limited epilepsy with centrotemporal spikes (SeLECTS). METHODS: This retrospective study was conducted on SeLECTS patients of with at least five years follow-up and at least two EEG recordings in which spike wave indexes (SWI) were calculated. RESULTS: 136 patients were enrolled. Median SWI in the first and last EEGs were 39% (7.6-89%) and 0 (0-112%). Gender, seizure onset age, psychiatric diseases, seizure characteristics (semiology, duration, and relationship to sleep), last EEG time, and spike lateralization in the first EEG did not have a statistically significant effect on the SWI change. Multinomial logistic regression analysis revealed that presence of phase reversal, interhemispheric generalization, and SWI percentage had a significant effect on spike reduction. The frequency of seizures was also significantly decreased in patients with a greater decrease in SWI. Both valproate and levetiracetam were statistically superior in suppressing SWI, with no significant difference between them. CONCLUSION: Interhemispheric generalization and phase reversal in the first EEG in SeLECTS had negative effects on the spike reduction. The most effective ASMs in reducing spikes were valproate and levetiracetam.

Evaluation of Thiol-Disulfide Homeostasis with Electrical Status Epilepticus in Slow Sleep (ESES).

Background: Electrical status epilepticus in sleep (ESES) is an epileptic syndrome specific to childhood and has a broad clinical spectrum that included seizures, behavioral/cognitive impairments, and motor neurological symptoms. Antioxidants are seen as promising neuroprotective strategies for the epileptic state by combating the harmful effects of excessive oxidant formation in mitochondria. Objective: This study aims to evaluate the thiol-disulfide balance and to determine whether it can be used in the clinical and electrophysiological follow-up of patients with ESES, especially in addition to the electroencephalography (EEG) examination. Methods: The study included 30 patients, aged 2-18 years and diagnosed with ESES in the Pediatric Neurology Clinic of the Training and Research Hospital and a control group of 30 healthy children. Total thiol, native thiol, disulfide, and ischemia-modified albumin (IMA) levels were measured, and disulfide-thiol ratios were calculated for both groups. Results: Native thiol and total thiol levels were significantly lower and IMA level and disulfide-native thiol percentage ratio were significantly higher in the ESES patient group than in the control group. Conclusion: Serum thiol-disulfide homeostasis is an accurate marker of oxidative stress in ESES, and standard and automated measures of thiol-disulfide balance as an indicator of oxidative stress showed a shift toward oxidation in ESES patients in this study. The negative correlation between spike-wave index (SWI) and thiol levels, and serum thiol-disulfide levels suggest that they can be used as biomarkers for follow-up of patients with ESES in addition to EEG. IMA can also be used for long-term response to monitoring purposes at ESES.

SQNN: a spike-wave index quantification neural network with a pre-labeling algorithm for epileptiform activity identification and quantification in children.

Objective.Electrical status epilepticus during slow sleep (ESES) is a phenomenon identified by strong activation of epileptiform activity in the electroencephalogram (EEG) during sleep. For children disturbed by ESES, spike-wave index (SWI) is defined to quantify the epileptiform activity in the EEG during sleep. Accurate SWI quantification is important for clinical diagnosis and prognosis. To quantify SWI automatically, a deep learning method is proposed in this paper.Approach.Firstly, a pre-labeling algorithm (PreLA) composed of the adaptive wavelet enhanced decomposition and a slow-wave discrimination rule is designed to efficiently label the EEG signal. It enables the collection of large-scale EEG dataset with fine-grained labels. Then, an SWI quantification neural network (SQNN) is constructed to accurately classify each sample point as normal or abnormal and to identify the abnormal events. SWI can be calculated automatically based on the total duration of abnormalities and the length of the signal.Main results.Experiments on two datasets demonstrate that the PreLA is effective and robust for labeling the EEG data and the SQNN accurately and reliably quantifies SWI without using any thresholds. The average estimation error of SWI is 3.12%, indicating that our method is more accurate and robust than experts and previous related works. The processing speed of SQNN is 100 times faster than that of experts.Significance.Deep learning provides a novel approach to automatic SWI quantification and PreLA provides an easy way to label the EEG data with ESES syndromes. The results of the experiments indicate that the proposed method has a high potential for clinical diagnosis and prognosis of epilepsy in children.

SQNN: A Spike-wave index Quantification Neural Network with a pre-labeling algorithm for epileptiform activity identification and quantification in children.

OBJECTIVE: Electrical status epilepticus during slow sleep (ESES) is a phenomenon identified by strong activation of epileptiform activity in the electroencephalogram (EEG) during sleep. For children disturbed by ESES, spike-wave index (SWI) is defined to quantify the epileptiform activity in the EEG during sleep. Accurate SWI quantification is important for clinical diagnosis and prognosis. To quantify SWI automatically, a deep learning method is proposed in this paper. APPROACH: Firstly, a pre-labeling algorithm (PreLA) composed of the adaptive wavelet enhanced decomposition and a slow-wave discrimination rule is designed to efficiently label the EEG signal. It enables the collection of large-scale EEG dataset with fine-grained labels. Then, an SWI Quantification Neural Network (SQNN) is constructed to accurately classify each sample point as normal or abnormal and to identify the abnormal events. SWI can be calculated automatically based on the total duration of abnormalities and the length of the signal. MAIN RESULTS: Experiments on two datasets demonstrate that the PreLA is effective and robust for labeling the EEG data and the SQNN accurately and reliably quantifies SWI without using any thresholds. The average estimation error of SWI is 3.12%, indicating that our method is more accurate and robust than experts and previous related works. The processing speed of SQNN is 100 times faster than that of experts. SIGNIFICANCE: Deep learning provides a novel approach to automatic SWI quantification and PreLA provides an easy way to label the EEG data with ESES syndromes. The results of the experiments indicate that the proposed method has a high potential for clinical diagnosis and prognosis of epilepsy in children.

Spike-Wave Index Assessment and Electro-Clinical Correlation in Patients with Encephalopathy Associated with Epileptic State During Slow Sleep (ESES / CSWS); Single-Center Experience.

PURPOSE: This study aimed to describe the electroclinical spectrum and neurocognitive outcome in children with epileptic encephalopathy with status epilepticus during sleep (ESES) according to the EEG patterns. METHODS: Records of 48 (19 males, 29 females) patients with ESES/CSWS syndrome were retrospectively evaluated for data on sleep and awake EEGs, psychometric tests, and brain MRI. Patients with a spike-wave index (SWI) of at least 50 % in the NREM sleep EEG were included in the study. Electrophysiologic findings were separated into two groups based on SWI: SWI>85-100 % (typical ESES) and SWI < 85 % (atypical ESES). The neurocognitive prognosis was also evaluated in two groups; favorable and unfavorable. RESULTS: The median age at the onset of ESES was 6 years and 5 months and ranged from 3 to 13 years. The median duration of follow-up after the ESES diagnosis was 57 months (range 24-150 months). Etiology was evaluated in three groups: symptomatic/structural, idiopathic, and unknown (cryptogenic). Twenty-seven (56.25 %) patients had atypical ESES patterns and 21 patients (43.75 %) had typical ESES patterns. Twenty-eight patients (58.3 %) had cognitive deterioration. Long term neurocognitive outcome was unfavorable in half of the patients. Symptomatic/structural etiology was more common in patients with unfavorable (p < 0.001) outcomes. The median age at the diagnosis of ESES (p < 0.001) was significantly earlier in the patients with unfavorable neurocognitive outcomes. The longer duration of ESES(p < 0.001), and the longer time between the onset of epilepsy and ESES (p = 0.039) was significantly associated with unfavorable outcomes. We found that patients with typical ESES had a higher risk for poor neurocognitive outcomes than patients with atypical ESES (OR: 31.096 [1.565-617.696]). CONCLUSION: The long-term outcome of ESES is exceedingly variable. An unfavorable neurocognitive outcome seems to be related to ESES with a long-duration and early-onset epileptic activity, SWI ≥ 85 %, and etiology.

Expanding the clinical and EEG spectrum of CNKSR2-related encephalopathy with status epilepticus during slow sleep (ESES).

OBJECTIVE: To investigate the clinical and EEG features of Encephalopathy with Status Epilepticus during slow Sleep (ESES) related to CNKSR2 pathogenic variants. METHODS: Detailed clinical history, repeated wakefulness/overnight sleep EEGs, brain MRI were collected in five patients, including one female, with CNKSR2-related ESES. RESULTS: Neurodevelopment in infancy was normal in two patients, delayed in three. Epilepsy onset (age range: 2-6 years) was associated with appearance or aggravation of cognitive impairment, language regression and/or behavioral disorders. Worsening of epilepsy and of cognitive/behavioral disturbances paralleled by enhancement of non-rapid eye movement (NREM) sleep-related, frontally predominant, EEG epileptic discharges [spike-wave-index (SWI): range 60-96%] was consistent with ESES. In three patients, episodes of absence status epilepticus or aggravation of atypical absences occurred, in this latter case associated with striking increment of awake SWI. Speech/oro-motor dyspraxia was diagnosed in four patients. In two patients, long-term follow-up showed epilepsy remission and persistence of mild/moderate cognitive disorders and behavioral disturbances into adulthood. CONCLUSIONS: Novel findings of our study are occurrence also in females, normal neurodevelopment before epilepsy onset, epilepsy aggravation associated with enhanced awake SWI, mild/moderate evolution in adulthood and language disorder due to speech/oro-motor dyspraxia. SIGNIFICANCE: Our findings expand the phenotypic spectrum of CNKSR2-related ESES.

Electrical status epilepticus in sleep (ESES) - Treatment pattern and EEG outcome in children with very high spike-wave index.

OBJECTIVE: The objective of the study was to determine electrical status epilepticus in sleep (ESES) outcome in children with very high spike-wave index (SWI; ≥85%), and assess treatment pattern. METHODS: Medical records of children 1-17 years old with ESES were reviewed. In this study, ESES is defined as SWI in non-rapid eye movement (non-REM) sleep of ≥85%. Electrical status epilepticus in sleep resolution is defined as reduction of SWI to <50%. RESULTS: Complete data were available in 33 children. Age at ESES diagnosis ranged from 32 to 165 months, median 76 months. The median duration of follow-up was 33 months. Two-thirds of the children were on one or more antiepileptic drugs (AED) at ESES diagnosis. Antiepileptic drugs were used as first treatment for ESES in 24/33 (73%). Electrical status epilepticus in sleep initially resolved in 76%, but 56% had subsequent relapse. The relapse rate was higher for steroids (89%) and benzodiazepines (60%) as compared with nonbenzodiazepine AEDs (29%). At last follow-up, ESES resolved in 21 children (64%). Electrical status epilepticus in sleep resolution was associated with seizure freedom (Fisher's exact, p < 0.05). SIGNIFICANCE: Using electroencephalogram (EEG) criteria, ESES resolved in 64%. We found high failure rate of first-line AEDs in preventing ESES, and high relapse rate. Standardization of ESES management is urgently needed.

Electroencephalographic abnormalities are correlated with cognitive deficits in children with benign childhood epilepsy with centrotemporal spikes: A clinical study of 61 cases.

OBJECTIVE: The objective of this study was to explore the effect of spikes on cognition in patients with benign childhood epilepsy with centrotemporal spikes (BECTS) and to identify electroencephalography (EEG) markers enabling early detection of cognitive impairment. METHODS: Sixty-one children with BECTS diagnoses and 60 age- and education-matched healthy controls were enrolled. Four-hour EEG recordings were analyzed for each patient to check for interictal spikes, high-frequency oscillations (HFOs), nondipole spikes, and other atypical EEG features and to examine the spike-wave index of nonrapid eye movement (NREM) sleep. All 121 children underwent a series of neuropsychological tests to assess cognitive function. RESULTS: Patients with a high NREM sleep discharge index (≥55%) in the first sleep cycle exhibited significantly lower scores for arithmetic calculation, executive function, and attention and memory tests than patients with a low discharge index (<55%). Eight patients with HFOs exhibited even poorer performance than HFO-negative patients for arithmetic calculation, executive function, vocabulary comprehension, visual perception, vocal perception, spatial memory ability, and response ability. Children with bilateral discharge exhibited poorer ability in three-dimensional spatial imaging test, poorer memory, and slower response than did those with unilateral discharge (P < .05). Nondipole spikes, multiple asynchronous discharges, and generalized spike-wave discharges respectively had an impact on calculation ability, memory, and reaction ability respectively (P < .05). CONCLUSIONS: Spike frequencies in stage 3 and 4 sleep varied from those observed in stage 1 and 2 sleep; the highest spike frequency was in stage 2 sleep. High NREM sleep discharge index (i.e., ≥55%) and HFOs were linked to the highest risk for cognitive deficit, while bilateral discharges, nondipole spikes, multiple asynchronous discharges, and generalized spike-wave discharges were less indicative of cognitive impairment.

[Epileptiform activity index for the evaluation of treatment efficacy in patients with epileptic encephalopathy]. / Indeks épileptiformnoi aktivnosti v otsenke éffektivnosti lecheniia épilepticheskoi éntsefalopatii.

Continuous spike and waves during sleep (CSWS) may lead to different cognitive, motor and behavioral symptoms. CSWS is characterized by an electroencephalography (EEG) pattern of electrical status epilepticus during sleep (ESES). The article reviews the main criteria of ESES, localization of EEG-changes and main EEG-patterns. An analysis of abnormal neuroimaging findings in patients with ESES was performed. The main pathophysiologic theories of ESES pattern formation are reviewed. ESES diagnosis is usually based on rough visual assessment of the number of spikes and waves, but other methods of assessment are also utilized, including spike-wave index (SWI), which is widely used in clinical practice. SWI reflects the percentage of sleep covered with spikes and waves. Two different strategies of SWI calculation are used, but none of them is officially validated. Moreover, there is no consensus on the period of sleep to be used for the calculation of SWI. The article presents a detailed review of calculation and utilization of SWI. It is concluded that further research is needed to develop an optimal algorithm of SWI calculation.

Personality profile and health-related quality of life in adults with previous continuous spike-waves during slow sleep syndrome.

INTRODUCTION: Epilepsy with continuous spike-waves during slow sleep syndrome (CSWSS) is characterized by various seizure types, a characteristic EEG pattern and neuropsychological disorders. The main purpose of this study was to evaluate the long-term outcome of CSWSS occurred in childhood and to evaluate the variables that could influence the quality of social adaptation and the personality profile. MATERIAL AND METHODS: This is a prospective study on 24 young adults with previous CSWSS (median age 24.5 yrs) who were enrolled between January and July 2011 at the G. Gaslini Children's Hospital, Genoa, Italy. Patients were divided into two groups: twelve with previous spike-wave index (SWI > 85%) defined as typical CSWSS (T-CSWSS) and twelve with previous SWI = 50-85% defined as atypical CSWSS (A-CSWSS). All the subjects were submitted to Minnesota Multiphasic Personality Inventory-2 (MMPI-2), Psychological General Well-Being Index (PGWBI), and to a structured interview. RESULTS: A correlation was observed with the severity of EEG abnormalities expressed by the SWI and outcome. The T-CSWSS group showed a significantly lower perceived well-being. Similarly in the T-CSWSS group the percentage of MMPI-2 clinical scales with T-scores ≥65 was higher than in the A-CSWSS group. Finally, a significant lower schooling in the T-CSWSS group was observed. CONCLUSION: There seem to be two forms of the same disease, with similar onset and clinical evolution but a different outcome regarding the social and psychological conditions. The outcome of the social adaptation and of the personality consciousness was related with the severity of the EEG abnormalities: more favorable in patients with less intense SWI activity (A-CSWSS) compared those with a more severe EEG impairment (T-CSWSS).

Cognition in patients with benign epilepsy with centrotemporal spikes: A study with long-term VEEG and RS-fMRI.

OBJECTIVE: The purpose of this study was to investigate the relationship between alterations of functional brain network and cognition in patients with benign epilepsy with centrotemporal spikes (BECTS) as a function of spike-wave index (SWI) during slow wave sleep. METHODS: Resting-state functional magnetic resonance imaging (RS-fMRI) data and Intelligence Quotient (IQ) were collected from two groups of patients with BECTS, including a SWI<50% group (5 cases) and a SWI≥50% group (7 cases). The SWI was calculated from the long-term video-electroencephalogram monitoring (one sleep cycle was included at least). The RS-fMRI data were analyzed by regional homogeneity (ReHo) method. RESULTS: There were three main findings. Firstly, Full Intelligence Quotient (FIQ), Verbal Intelligence Quotient (VIQ), and Performance Intelligence Quotient (PIQ) of the SWI≥50% group were significantly lower than SWI<50% group (p<0.05). Secondly, there was a negative correlation between the FIQ, VIQ, PIQ, and SWI (p<0.05), and the FIQ, VIQ, and PIQ were not dependent on age, age of onset, disease course, years of education, and total number of seizures (p>0.05). Finally, compared with the SWI<50% group, the SWI≥50% group showed increased ReHo in the bilateral precentral gyrus, bilateral premotor area, bilateral subcortical structure, right temporal lobe, and bilateral insular lobe, while they showed decreased ReHo in the posterior cingulate cortex and posterior of right inferior temporal lobe. CONCLUSIONS: The alterations of functional brain network caused by the frequent discharges during slow wave sleep could affect cognition in patients with BECTS.

Response to adrenocorticotropic in attention deficit hyperactivity disorder-like symptoms in electrical status epilepticus in sleep syndrome is related to electroencephalographic improvement: A retrospective study.

INTRODUCTION: Encephalopathy with electrical status epilepticus in sleep (ESES) syndrome is a rare epilepsy syndrome of childhood that is characterized by sleep-induced epileptiform discharges and problems with cognition or behavior. The neuropsychiatric symptoms in ESES syndrome, among which the ADHD-like symptoms are prominent, bear a close resemblance to symptoms in various developmental disorders. Positive response to adrenocorticotropic hormone (ACTH) is associated with the normalization of the EEG and improvement of neuropsychiatric function. This study aimed to determine the improvement in ADHD-like symptoms in response to ACTH and establish a relationship between improvement in clinical symptoms and EEG parameters. METHODS: Seventy-five patients with ESES syndrome, who had clinically displayed ADHD-like symptoms, had been treated with ACTH for ESES, and their medical records were retrospectively reviewed. Sleep EEGs were recorded at referral and follow-up visits, and short courses of ACTH were administered when spike-wave index (SWI) was ≥15%. The assessment of treatment effectiveness was based on reduction in SWI and the clinician-reported improvement in ADHD-like symptoms. Statistical analyses were conducted in order to investigate the relationship between the clinical and EEG parameters. RESULTS: Following treatment with ACTH, a reduction in SWI in all the patients was accompanied by a mean improvement of 67% in ADHD-like symptoms. Disappearance/reduction of foci and cessation/reduction of seizures were achieved in patients with formerly antiepileptic-resistant seizures. Multiple linear regressions established that pretreatment SWI and treatment delay predicted posttreatment SWI, while reduction in SWI, treatment delay, and the presence of foci predicted improvement in ADHD-like symptoms. DISCUSSION: Improvement in ADHD-like symptoms showed high correlation and was timely with the resolution of ESES. It is suggested that ESES and ADHD may be the two different expressions of a common neurobiological abnormality. With enhanced interpretation of sleep EEG, a more thorough assessment and treatment of neurodevelopmental disorders is possible.

Influence of epileptic activity during sleep on cognitive performance in benign childhood epilepsy with centrotemporal spikes.

BACKGROUND: Benign childhood epilepsy with centrotemporal spikes is benign childhood epilepsy, presenting between 4 and 10 years of age, characterized by typical clinical and EEG findings. Despite excellent prognosis, there are reports of mild cognitive, language, fine motor and behavioral difficulties. In its atypical form - electrical status epilepticus during slow wave sleep, continuous epileptiform activity during sleep lead to severe neurocognitive deterioration. Our objective was to investigate the influence of abundant sleep epileptiform activity, not fulfilling the criteria for electrical status epilepticus during Slow Wave Sleep, discovered randomly in children without overt intellectual impairment. METHODS: We retrospectively reviewed the charts and EEG's of 34 children with benign childhood epilepsy with centrotemporal spikes, who underwent neurocognitive evaluation. The neurocognitive battery included items in the following domains: attention span, memory, language, fine motor and behavior. Patients were divided into two groups according to the spike wave index on sleep EEG, with a cut-off point of 50%. The groups were compared regarding to neurocognitive performance. OUTCOMES: Children with epileptiform activity of more than 50%, were diagnosed at a significantly younger age (5.13 ± 1.94 years vs. 7.17 ± 2.45, p = 0.014 T test), had less controlled seizures and received more antiepileptic drugs. However, there was no difference in neurocognitive performance, except in fine motor tasks (Pegboard), where children with more abundant activity were scored lower (-0.79 ± 0.96 vs. 0.20 ± 1.05, p = 0.011, T test). CONCLUSION: Our study did not show negative cognitive effect of abundant epileptiform activity discovered randomly in children with benign childhood epilepsy with centrotemporal spikes, warranting aggressive treatment.

ACTH has beneficial effects on stuttering in ADHD and ASD patients with ESES: A retrospective study.

INTRODUCTION: Etiology of stuttering remains unknown and no pharmacologic intervention has been approved for treatment. We aimed to evaluate EEG parameters and the effect of adrenocorticotropic hormone (ACTH) therapy in stuttering. METHODS: In this retrospective study, 25 patients with attention deficit and hyperactivity (ADHD) or autism spectrum disorder (ASD), and comorbid stuttering were followed and treated with ACTH for electrical status epilepticus in sleep (ESES). Sleep EEGs were recorded at referral and follow-up visits and short courses of ACTH were administered when spike-wave index (SWI) was ⩾15%. The assessment of treatment effectiveness was based on reduction in SWI, and the clinician-reported improvement in stuttering, and ADHD or ASD. Statistical analyses were conducted in order to investigate the relationship between the clinical and EEG parameters. RESULTS: Following treatment with ACTH, a reduction in SWI in all the patients was accompanied by a 72% improvement in ADHD or ASD, and 83.8% improvement in stuttering. Twelve of the 25 patients with stuttering showed complete treatment response. Linear regressions established that SWI at final visit significantly predicted improvement in ADHD or ASD, and in stuttering. If symptoms had recurred, improvement was once again achieved with repeated ACTH therapies. Stuttering always improved prior to, and recurred following ADHD or ASD. CONCLUSION: The underlying etiology leading to ESES may play a significant role in the pathophysiology of stuttering and connect stuttering to other developmental disorders. ACTH therapy has beneficial effects on stuttering and improves EEG parameters.

Effect of large dose of Methylprednisolone on epilespy combined with electrical status epilepticus during sleep in children / 中华实用儿科临床杂志

Objective To investigate the clinical effect of large dosage of Methylprednisolone on epilespy combined with electrical status epilepticus during sleep(ESES) in children.Methods Forty-six epielpsy patients with ESES were treated with additive large dosage of Methylprednisolone.The seizures and video electroencephalogram discharges were observed before and after using Methylprednisolone.The Methylprednisolone effect in eliminating the methylprednisolone of ESES and controlling of clinical seizures and improving cognitive function were analyzed.Results Two cases were lost and 44 cases were included,male 30,female 14,the age at onset was (5.37 ±2.52) (2-9) years old.The duration of follow-up was (4.12 ± 2.00)(1-9) years old.The age at diagnosis of ESES was (8.17 ± 2.09) (4.0-12.5) years old.The efficacy of Methylprednisolone on seizures was 72.7％ (32/44 cases),while the efficacy of Methyl prednisolone on electroencephalograph (EEG) was 59.1％ (26/44 cases).For patients who were resistant to Levetiracetam or Clonazepam,Methylprednisolone was still effective.Intelligence quotient had no significant changes before and after treatment(P ＞ 0.05).The earlier onset age,the worse effect of Methylprednisolone.The efficacy of methylprednisolone for atypical benign epilepsy with cento-temporal spike(BECT) was higher than other syndromes.Conclusions Large dosage of Methyl prednisolone therapy for children with ESES,especially for those resistant to traditional or new antiepileptic drug for ESES,was effective and safe.The onset age and syndrome classification may have a certain value for prognosis and prediction of the effect of Methylprednisolone treatment.

Acetazolamide for electrical status epilepticus in slow-wave sleep.

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.