Status Epilepticus In Sleep Research Articles

Perinatal thalamic injury: MRI predictors of electrical status epilepticus in sleep and long-term neurodevelopment.

Perinatal thalamic injury is associated with epilepsy with electrical status epilepticus in sleep (ESES). The aim of this study was to prospectively quantify the risk of ESES and to assess neuroimaging predictors of neurodevelopment. We included patients with perinatal thalamic injury. MRI scans were obtained in the neonatal period, around three months of age and during childhood. Thalamic and total brain volumes were obtained from the three months MRI. Diffusion characteristics were assessed. Sleep EEGs distinguished patients into ESES (spike-wave index (SWI) >85%), ESES-spectrum (SWI 50-85%) or no ESES (SWI<50%). Serial Intelligence Quotient (IQ)/Developmental Quotient (DQ) scores were obtained during follow-up. Imaging and EEG findings were correlated to neurodevelopmental outcome. Thirty patients were included. Mean thalamic volume at three months was 8.11 (±1.67)ml and mean total brain volume 526.45 (±88.99)ml. In the prospective cohort (n=23) 19 patients (83%) developed ESES (-spectrum) abnormalities after a mean follow-up of 96 months. In the univariate analysis, larger thalamic volume, larger total brain volume and lower SWI correlated with higher mean IQ/DQ after 2 years (Pearson's r=0.74, p=0.001; Pearson's r=0.64, p=0.005; and Spearman's rho -0.44, p=0.03). In a multivariable mixed model analysis, thalamic volume was a significant predictor of IQ/DQ (coefficient 9.60 [p<0.001], i.e., corrected for total brain volume and SWI and accounting for repeated measures within patients, a 1ml higher thalamic volume was associated with a 9.6 points higher IQ). Diffusion characteristics during childhood correlated with IQ/DQ after 2 years. Perinatal thalamic injury is followed by electrical status epilepticus in sleep in the majority of patients. Thalamic volume and diffusion characteristics correlate to neurodevelopmental outcome.

Reduced thalamic volume is strongly associated with electrical status epilepticus in sleep.

To identify the relationship between thalamic volume and electrical status epilepticus in sleep (ESES). We analyzed subcortical gray matter volumes in patients with an ESES pattern on their electroencephalographs. All magnetic resonance imaging scans were considered within normal limits. The patients were not receiving antiepileptic drug at the time of the MRI study. High resolution T1-weighted 3-dimensional MPRAGE scans were assessed for segmentation and quantitative volumetric analysis of the brain by using the "volBrain" method. After correcting for total brain volume, volumes were compared with a group of healthy controls (HCs) and patients with benign childhood epilepsy with centrotemporal spikes (BECTS). Fifteen patients with ESES, 15 patients with BECTS, and 30 HCs were included. The median age of the patients with ESES was 8.5 (range, 5.8-13) years, 8 (range, 5-14) years for the HCs, and 7.8 (range, 4-13.5) years for the patients with BECTS. The total relative thalamic volume was significantly lower in patients with ESES than in the healthy controls (0.87 ± 0.07 vs. 0.93 ± 0.03, p = 0.002), and in patients with ESES than in those with BECTS (0.87 ± 0.07 vs. 0.93 ± 0.03, p = 0.006). There was no significant difference the HCs and patients with BECTS (0.93 ± 0.03 vs. 0.93 ± 0.03, p = 0.999). Both right and left relative thalamic volumes were lower in patients with ESES than in HCs (right thalamus: 0.43 ± 0.04 vs. 0.46 ± 0.02, p = 0.003, left thalamus: 0.44 ± 0.03 vs. 0.47 ± 0.02, p = 0.002), in patients with ESES than in patients with BECTS (right thalamus: 0.43 ± 0.04 vs. 0.46 ± 0.01, p = 0.01, left thalamus: 0.43 ± 0.04 vs. 0.47 ± 0.01, p = 0.007); however, there was no significant difference between the HCs and patients with BECTS (right thalamus: 0.46 ± 0.02 vs. 0.46 ± 0.01, p = 0.999, left thalamus: 0.47 ± 0.02 vs. 0.47 ± 0.01, p = 0.999). This study highlights the association between thalamic involvement and ESES, even when not severe enough to cause a detectable lesion on visual interpretation of MRI.

Scalp EEG high frequency oscillations as a biomarker of treatment response in epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS)

PurposeWe investigated whether the presence of interictal scalp EEG high frequency oscillations (HFOs) in children with epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS) can predict seizure and cognitive outcome after steroid therapy. MethodsTwenty-two children with CSWS were prospectively enrolled and received methylprednisolone therapy. Interictal scalp HFOs, spike wave index (SWI) and intelligence quotient (IQ) were assessed before and after the treatment. The children were divided into two groups based on the early seizure reduction ratio at 2 weeks (≥50%, “response group”; otherwise “non-response group”). The “response group” was further divided into two subgroups (“relapse” and “non-relapse” subgroups) according to the late seizure outcome (after 3 months). ResultsInterictal HFOs and electrical status epilepticus in sleep (ESES) (defined as SWI ≥ 85%) were detected in all children at the baseline. In the response with relapse group (n = 11), the detection ratio of HFOs was significantly higher than that of ESES at 2 weeks (81.2 vs. 27.3%), 3 months (90.9 vs. 36.4%), and 6 months (100 vs. 54.5%) post-therapy. In the non-response group (n = 4), both HFOs and ESES persisted in all children. The average IQ improved significantly only in the response with non-relapse group. The persistence of HFOs negatively correlated with both the average IQ, yet the persistence of ESES did not. ConclusionInterictal scalp HFOs may be a favorable non-invasive biomarker of predicting seizure and cognitive outcome in CSWS.

Language and behavioral outcomes of treatment with pulse-dose prednisone for electrical status epilepticus in sleep (ESES)

Few studies have examined treatment response in electrical status epilepticus in sleep (ESES), and fewer still have evaluated the effect of corticosteroid treatment employing a pulse-dose regimen. The aim of this study was to examine the effectiveness of pulse-dose prednisone in treating language and behavioral disturbances that often accompany ESES. The sample included 17 patients age 5 to 10 years at time of baseline electroencephalogram (EEG) and neuropsychological assessments. For all patients, focal, multifocal, or generalized spike and wave activity occupied greater than 50% of the nonrapid eye movement (REM) sleep record. Patients were seen for follow-up EEG recording and neuropsychological testing with an average of 10 months following initiation of pulse-dose prednisone. Improvement in language or behavior was examined in relation to resolution of ESES on EEG, age at seizure onset and treatment, duration of ESES, duration of treatment, lesional versus nonlesional epilepsy, history of language or behavioral regression, seizure control at follow-up, and intelligence quotient (IQ). With the exception of a greater likelihood of patients with low IQ to demonstrate improvement in language or behavior, improvement was seen in most patients, irrespective of ESES or other factors.

Functional brain connectivity in electrical status epilepticus in sleep

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. 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. 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. 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.

Treatment in epileptic encephalopathy with ESES and Landau–Kleffner syndrome

Epileptic encephalopathy with electrical status epilepticus in sleep (ESES) is an age-related syndrome with acquired neurodevelopmental deficits in relation to epilepsy with many seizure types. Early and accurate treatment is aimed at prevention or reduction of associated cognitive deficits and, to a lesser extent, control seizures. Evidence for an optimal treatment strategy is still lacking. Here, we review the current literature on treatment efficacy in patients with epileptic encephalopathy with ESES.

Electrical status epilepticus in sleep, a constitutive feature of Christianson syndrome?

Christianson syndrome (CS) is a X-linked neurodevelopmental disorder, including severe intellectual disability (ID), progressive microcephaly, ataxia, autistic behaviour (ASD), near absent speech, and epilepsy. Electrical status epilepticus in sleep (ESES) has been reported in two patients. We describe five male patients from three unrelated families with Christianson syndrome caused by a pathogenic nucleotide variation or a copy-number variation involving SLC9A6. ESES was present in three out of the five patients in the critical age window between 4 and 8 years. All patients presented with severe intellectual disability, autistic features, and hyperactivity. Epilepsy onset occurred within the first two years of life. Seizures were of various types. In the two boys with a 20-years follow-up, epilepsy was drug-resistant during childhood, and became less active in early adolescence. Psychomotor regression was noted in two patients presenting with ESES. It was difficult to assess to what extent ESES could have contributed to the pathophysiological process, leading to regression of the already very limited communication skills. The two published case reports and our observation suggests that ESES could be a constitutive feature of Christianson syndrome, as it has already been shown for other Mendelian epileptic disorders, such as GRIN2A and CNKSR2-related developmental epileptic encephalopathies. Sleep EEG should be performed in patients with Christianson syndrome between 4 and 8 years of age. ESES occurring in the context of ID, ASD and severe speech delay, could be helpful to make a diagnosis of CS.

Amantadine: A new treatment for refractory electrical status epilepticus in sleep

PurposeElectrical status epilepticus in sleep (ESES) is an electrographic abnormality linked to language abnormalities and cognitive dysfunction and specifically associated with Landau–Kleffner syndrome (LKS), the syndrome of continuous spike and wave in slow-wave sleep (CSWS), and autistic regression with epileptiform EEG (AREE). As first-line therapies for treatment of ESES display inadequate efficacy and confer substantial risk, we set out to describe our center's experience with amantadine in the treatment of ESES. MethodsPatients with video-EEG-confirmed ESES who received amantadine were retrospectively identified in a clinical EEG database. Spike–wave index, before and after amantadine exposure, was compared in a pairwise fashion. In an exploratory analysis, we cataloged reported changes in language functioning, cognition, and autistic features, which accompanied treatment. ResultsWe identified 20 patients with ESES-associated syndromes. Median cumulative weighted average amantadine dosage was 2.1 mg/kg/d (interquartile range (IQR): 1.1, 4.5), and median duration of therapy was 11.5 months (IQR: 7.8, 26.6). In comparison with median baseline spike–wave index (76%), post-amantadine spike–wave index (53%) was reduced, with P = 0.01. Six (30%) patients exhibited complete (or nearly complete) resolution of ESES. A majority of patients exhibited subjective cognitive, linguistic, or behavioral benefit. Amantadine was generally well-tolerated despite substantial dosage and duration of therapy. ConclusionsThis study suggests that amantadine may be effective in the treatment of ESES-associated syndromes but warrants replication in a more rigorous study.

The clinical and electroencephalographic spectrum of ESES (Encephalopathy with status epilepticus in sleep) in Indian children (P2.300)

The clinical and electroencephalographic spectrum of ESES (Encephalopathy with status epilepticus in sleep) in Indian children (P2.300)

Treatment of electrical status epilepticus in sleep: Clinical and EEG characteristics and response to 147 treatments in 47 patients.

Electrical status epilepticus in sleep (ESES) syndrome is characterized by near-continuous sleep-induced epileptiform activity and acquired cognitive deficits. Treatment is assumed mandatory to improve cognitive outcome. We aimed to compare EEG characteristics, subjective evaluation and objective neuropsychological assessment as measures to evaluate treatment efficacy, and to analyze possible predictors. We retrospectively included patients with ESES syndrome treated in our center. Treatment effect was analyzed on sleep EEG spike wave index (SWI) and cognitive functioning. 47 patients had 147 (43 steroid and 104 non-steroid) treatments. Cognitive improvement was reported after 36% of treatments at first follow-up and 45% of treatments at last follow-up. The median SWI change for treatments resulting in subjective cognitive improvement was -44%, and 0% for those not resulting in subjective cognitive improvement at first follow-up (p=0.008) and -50% vs. +5% at last follow-up (p=0.002). No clear association between subjective cognitive improvement and IQ change, and between SWI and IQ change was found. By means of logistic regression we found that steroid treatment, as compared to non-steroid treatment, was associated with cognitive improvement at first follow-up (multivariate OR after multiple imputation 2.5, 95% CI 1.1-5.7), while at last follow-up, higher age at diagnosis was related to cognitive improvement only in univariate analysis (OR 1.02, 95% CI 1.01-1.04). We found that in children with ESES, cognitive improvement after treatment was strongly associated with SWI decrease, while it was not reflected by a significant IQ increase. Steroid treatment was most successful in improving cognitive performance.

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.

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. 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. 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. 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.

Christianson syndrome: An underestimated cause of electrical status epilepticus in sleep?

Objective: Christianson syndrome (CS) is a syndromic form of X-linked severe ID associated to progressive microcephaly, ataxia, autistic behaviour, near absent speech and epilepsy in 90% of cases. The natural history of seizures and EEG abnormalities is insufficiently known. We report two additional families including patients with electrical status epilepticus in sleep (ESES) previously described in only two patients. Methods: We describe two unrelated families with CS caused by SLC9A6 mutations (one frameshift and one partial deletion), each including two boys. One family has been followed for over 20 years, providing insights into the natural course of epilepsy in this syndrome. Results: Onset of epilepsy occurred within the first two years of life in all patients. Seizures were of various types. First EEG recordings showed abnormal fast-background activity with poor antero-posterior organization as reported in the literature. In the two boys with a 20 year follow-up, epilepsy was drug-resistant during childhood, then became less active in early adolescence. EEG background rhythm slowed over time to 4–5Hz after the age of 8 years. One patient from the first family fulfilled the electrical criteria of ESES, between 6 and 8 years of age, while sleep-EEG had not been performed in his brother during this range of age. ESES was diagnosed in the older brother from the second family since the age of 4 years and 10 months. The four boys presented severe ID, autistic features with agitation limiting frequent sleep EEG recordings. Conclusion: Our observations and previous reports, suggest that ESES might be an underestimated feature in CS and may participate to the psychomotor degradation of the patients. Interestingly, SLC9A6 mutations underlie dysfunction in the sodium-hydrogen exchanger NHE6 implicated in plasticity of glutamatergic synapses. Sleep EEGs should be performed in these infants between 4 and 8 years of age.

Sleep slow waves in idiopathic epileptic encephalopathy with status epilepticus in sleep (ESES) during active and recovery phase

Sleep slow waves in idiopathic epileptic encephalopathy with status epilepticus in sleep (ESES) during active and recovery phase

Neuropsychological Outcome in Perinatal Stroke Associated With Epileptiform Discharges in Sleep.

Patients with arterial perinatal stroke often suffer long-term motor sequelae, difficulties in language, social development, and behaviour as well as epilepsy. Despite homogeneous lesions, long-term behavioural and cognitive outcomes are variable and unpredictable. Sleep-related epileptic encephalopathies can occur after early brain injury and are associated with global developmental delays. We hypothesized that sleep-potentiated epileptiform abnormalities are associated with worse developmental outcomes after perinatal stroke. Participants were identified from a population-based cohort (Alberta Perinatal Stroke Project). Inclusion criteria were magnetic resonance imaging-confirmed arterial perinatal stroke, age 4 to 18 years, electroencephalogram (EEG) including sleep, and comprehensive neuropsychological evaluation. Sleep-related EEG abnormalities were categorized by an epileptologist blinded to the cognitive outcome. Associations between EEG classification and neuropsychological outcomes were explored (t tests, Bonferroni correction for multiple comparisons). Of 128 potentially eligible participants, 34 (53% female) had complete EEG (mean age, 8.1 years; range, 0.2-16.4) and neuropsychology testing (mean age, 9.8 years; range 4.4-16.7). Twelve (35%) were classified as having electrical status epilepticus in sleep. Patients with abnormal EEGs were more likely to have statistically worse scores when corrected for multiple comparisons, in receptive language (median, 1st percentile; IQR 1-7th percentile; p<0.05), and externalizing behaviours (median, 82nd percentile; IQR, 79-97th percentile; p<0.05). Developmental outcome in language and behaviour in children with arterial perinatal stroke is associated with electrical status epilepticus in sleep. Increased screening with sleep EEG is suggested, whereas further studies are necessary to determine if treatment of EEG abnormalities can improve outcome.

Reduced thalamic volume in patients with Electrical Status Epilepticus in Sleep

PurposeTo test whether patients with Electrical Status Epilepticus in Sleep (ESES) and normal neuroimaging have a smaller thalamic volume than expected for age and for total brain volume. MethodsCase-control study comparing three groups of subjects of 4–14 years of age and normal magnetic resonance imaging: 1) ESES patients, 2) patients with refractory epilepsy control group, and 3) healthy controls. Thalamic and total brain volumes were calculated using an algorithm for automatic segmentation and parcellation of magnetic resonance imaging. ResultsEighteen ESES patients, 29 refractory epilepsy controls and 51 healthy controls were included. The median (p25–p75) age was 8.8 (7.5–10.3) years for ESES patients, 11 (7–12) years for healthy controls, and 9 (6.3–11.2) years for refractory epilepsy controls. After correcting for total brain volume and age, the left thalamus was not statistically significantly smaller in ESES patients than in healthy controls (p=0.077), in ESES patients than in refractory epilepsy controls (p=0.056); but the right thalamus was smaller in ESES patients than in healthy controls (p=0.044), and in ESES patients than in refractory epilepsy controls (p=0.033). ConclusionPatients with ESES and normal magnetic resonance imaging have smaller relative thalamic volume controlling for age and total brain volume.

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

IntroductionEtiology 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. MethodsIn 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. ResultsFollowing 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. ConclusionThe 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.

Immediate termination of electrical status epilepticus in sleep after hemispherotomy is associated with significant progress in language development

To study the effect of hemispherotomy on electrical status epilepticus in sleep (ESES) and language development. Children with a confirmed diagnosis of ESES prior to surgery and a minimum of 24 months of developmental follow-up data were compared with age-matched controls without ESES. Language quotients (LQs) were calculated before and after surgery. Eleven patients (five females, six males) and 21 controls (11 females, 10 males) were included. Before surgery a significantly higher number of children in the study group (n=9) demonstrated severe developmental delay compared with children in the control group (n=13; p=0.015). In the study group ESES remitted immediately after surgery in 10 children, and a significant catch-up in LQs was observed in this group (preoperative mean 40.0 [standard deviation (SD) 22.2, interquartile range (IQR) 30.0-62.0]; postoperative mean 73.0 [SD 33.5, IQR 41.0-97.0]; p=0.037). There was no significant difference compared with controls at last follow-up after surgery (study group: five with severe impairment; control group: eight with severe impairment [p=0.971]). Overall, a favourable developmental outcome was associated with freedom from seizures (seizure-free group: median preoperative LQ 61.5, median postoperative LQ 78.0 [p=0.017]; seizure group: median preoperative LQ 35.5, median postoperative LQ 56.5 [p=0.273]) and antiepileptic drug withdrawal (off medication: median preoperative LQ 49.5, median postoperative LQ 78.0 [p=0.011]; on medication: median preoperative LQ 78.0, median postoperative LQ 83.5 [p=0.889]). Children with ESES showed significantly lower preoperative language abilities than children without ESES. In cases with remission of ESES after surgery, marked improvement in LQs was noticed. This improvement cannot be fully explained by seizure-freedom alone as seizure-free children without preoperative ESES showed less improvement.

Treatment of Atypical Evolutions of Idiopathic Focal Epilepsies in Childhood

The relationship between continuous spike-and-wave discharges during slow sleep (CSWSS) and neuropsychological impairment (cognitive functions, memory consolidation, and language and behavior disturbances) has been clearly demonstrated. These phenomena occur not only in symptomatic cases of CSWSS or electrical status epilepticus in sleep (ESES) syndrome, but also in the different conditions that constitute the spectrum of atypical evolutions of idiopathic focal epilepsies of childhood (IFEC). In spite of continuous advances in treatment, management is still difficult and challenging. Treatment options of atypical evolutions of IFEC include benzodiazepines, sulthiame, levetiracetam, valproic acid, and ethosuximide. Other drugs such as lacosamide and acetazolamide have been proposed. Usual antiepileptic drugs such as carbamazepine, phenobarbital, phenytoin, oxcarbazepine, lamotrigine, and topiramate were proven to be capable of inducing ESES. Steroids and intravenous immunoglobulins were recommended in refractory cases. Surgery, ketogenic diet, and vagal nerve stimulation were also used in some cases. Our interest regarding a scheme of treatment in atypical evolutions of IFEC is centered on two questions: (1) Is it possible to prevent the evolution of IFEC into atypical focal epilepsies of childhood, status epilepticus of IFEC, Landau–Kleffner's syndrome, or ESES syndrome? (2) Which is the treatment of choice once one of these conditions is installed?

Electrical status epilepticus in sleep (ESES)/continuous spikes and waves during slow sleep (CSWS) syndrome in children: An electroclinical evaluation according to the EEG patterns.

The aim of this study was to describe the electroclinical spectrum in children with electrical status epilepticus in sleep (ESES)/continuous spikes and waves during slow sleep (CSWS) syndrome according to the EEG patterns. Clinical data of 44 patients with ESES/CSWS syndrome who were treated and followed at least two years were analyzed. Records of EEGs of patients were reevaluated to determine two aspects of the ESES pattern: (1) the spike-wave index (SWI) on the NREM sleep EEG (Group I: typical vs. atypical ESES pattern (33/11 patients)) and (2) the area of maximum amplitude of continuous epileptic activity (Group II: anterior vs. posterior ESES pattern (33/11 patients)). Symptomatic etiology was more defined in patients with the typical ESES pattern (40%) than the group with the atypical ESES pattern (9%) by a factor of four. All patients were receiving at least two antiepileptic drug (AED) treatments. Eighteen patients (41%) received AEDs plus ACTH therapy. Complete disappearance of the ESES pattern on the EEG was observed in 18 patients (41%), more than 50% reduction was observed in five patients (11%), less than 50% reduction was observed in eight patients (18%), and no response was observed in five patients (11%). No significant difference was found when comparing the groups in terms of reduction of seizures and the SWI. Seizure outcome at the two-year follow-up was similar between the group with ESES treated with AEDs plus ACTH and the group with ESES treated with AEDs without ACTH therapy. This study demonstrated that the rate of the SWI (typical vs. atypical ESES) and the maximum amplitude of the ESES pattern (anterior vs. posterior) have no significant correlation with seizure control and reduction of the SWI on the EEG in children with ESES syndrome.

The yield of video-EEG telemetry evaluation for non-surgical candidate children.

Video EEG (VEEG) is performed for most pediatric patients in order to evaluate unclear paroxysmal events and improve our understanding of difficult to control epileptic patients. To characterize the video EEG studies on children who are not candidates for surgery in order to identify the parameters that affect results in level of improving the rate of acquisition, as well as improving the ability to expect the likelihood of epilepsy and of gathering new information as a result of the VEEG. Retrospective chart analysis of all consecutive patients who underwent VEEG in two VEEG monitoring units. 323 children of a mean age of 7 years (STD 4.73, range 0-17 years) were monitored for a mean duration of 2 days (STD 1.65, range 1-10 days). The main reasons for monitoring were: evaluation of unclear events (n=234), evaluation of previously diagnosed epilepsy (n=36) and confirmation of Electrical Status Epilepticus in Sleep (ESES) (n=34). The main event types for evaluation were: staring episodes (n=67), myoclonic jerks (n=35) and abnormal eye movement (n=22). Suspected events were captured in 70% of the patients. There was a positive correlation between acquisition of suspected events and each of the following: duration of the monitoring, the frequency of investigated events per history, the type of investigated events. A prior interictal epileptic activity on routine EEG was a positive predictor of an event to be epileptic (p=0.003). Amongst the group of known epileptic patients, VEEG had role in changing diagnosis in 53% of patients. Many of them had focal interictal epileptiform activity in their routine EEG. Selecting patients with frequent events and longer monitoring periods increase the yield of VEEG. Looking carefully into clinical characteristics of the patient prior to VEEG can clarify diagnosis therefore render the VEEG test superfluous to subgroups of patients. Prior routine epileptic EEG, coexistence of other seizure types, behaviors accompanying the investigated habitual behavior and abnormalities in other investigations (MRI, cognitive function and EEG) are the parameters that can predict diagnosis of epilepsy. Precise diagnosis in known epileptic patients as a result of VEEG is more likely for those with focal interictal epileptiform discharges in routine EEG.