Status Epilepticus In Sleep Research Articles

Electroencephalographic patterns during sleep in children with chromosome 15q11.2-13.1 duplications (Dup15q).

Our objective was to define the EEG features during sleep of children with neurodevelopmental disorders due to copy number gains of 15q11-q13 (Dup15q). We retrospectively reviewed continuous EEG recordings of 42 children with Dup15q (mean age: eight years, 32 with idic15), and data collected included background activity, interictal epileptiform discharges, sleep organization, and ictal activity. Three patterns were recognized: Pattern 1: Alpha–delta sleep was noted in 14 children (33%), not associated with any clinical changes. Pattern 2: Electrical status epilepticus in sleep was noted in 15 children (35%), all diagnosed with treatmentresistant epilepsy. Thirteen of the 15 children had clinical seizures. Pattern 3: Frequent bursts of high amplitude bifrontal predominant, paroxysmal fast activity (12–15 Hz) during non-REM sleep was noted in 15 children (35%). All 15 children had treatment-resistant epilepsy. This is the first report of electroencephalographic patterns during sleep of children with Dup15q reporting alpha-delta rhythms, CSWS, and high amplitude fast frequencies. Alpha-delta rhythms are described in children with dysautonomia and/or mood disorders and CSWS in children with developmental regression. The significance of these findings in cognitive function and epilepsy for the children in our cohort needs to be determined with follow-up studies.

How to Manage Electrical Status Epilepticus in Sleep.

Electrical status epilepticus in sleep is an age-dependent syndrome with the characteristic pattern of continuous spike and waves during non-rapid eye movement sleep. Most children can present developmental deterioration. The demonstration of the EEG pattern has to rely on all night long EEG recordings. A comprehensive neuropsychologic evaluation with periodic reassessment should be performed. For the idiopathic forms of electrical status epilepticus in sleep, clobazam could be considered as the first-line therapy; in the other cases, corticosteroids, in particular intravenous methylprednisolone pulse therapy, remain the most effective and should be considered the therapy of choice.

Serum inflammatory mediators correlate with disease activity in electrical status epilepticus in sleep (ESES) syndrome.

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.

PP14.4 – 2487: Comparison of three spike and wave index quantification methods in patients with electrical status epilepticus in sleep: Variability between observers and methods

Objective Different methods have been described in patients with electric status epilepticus during slow sleep (ESES) to calculate the spike-wave index (SWI). We measured the concordance between observers and SWI data using 3 methods. Methods We selected 3/10 SWI calculating methods described to apply in 17 whole night EEG recordings of children with clear ESES: (A1) the classic but long lasting method by Tassinari; (A2) following Aebey et al., 2005; and (A3) by Sanchez et al., 2012. Two experts separately analyzed them using the 3 methods. We used Intraclass Correlation Coefficient (ICC) as a measure of reliability to compare partial and final SWI between methods and between observers. Results SWI between methods: A3>A1>A2. Time consumption: A1 92 min, A2 33 min and A3 8 min. 15/17 patients showed SWI over the classical 85% cutoff values for the 3 methods; 2 patients showed SWI at least over 60%. The inter-observer ICC was Conclusion The SWI is higher in first sleep cycles, and decreases through the night. The SWI value for A3 depends very much on arousals. In spite of having selected unequivocal ESES, some patients did not reach the classical SWI value of 85% but reached the 60%, supporting the actual recommendation of consider ESES with lower indexes. The three quantification methods selected showed high concordance values, so the usefulness of brief methods is of great advantage in terms of time consumption.

Benign childhood epilepsy with Centro-Temporal spikes (BCECTSs), electrical status epilepticus in sleep (ESES), and academic decline — How aggressive should we be?

Since many of the children with BCECTSs display electrical status epilepticus during sleep and many present with different comorbidities, mainly ADHD and behavioral disturbances, clinicians are often confronted with the dilemma of how aggressive they should be with their efforts of normalizing the EEG. We conducted a retrospective study by screening medical records of all consecutive patients with BCECTSs, spike–wave index (SWI) >30%, and ADHD/ADD that were evaluated in our pediatric epilepsy service and were followed up for at least two years. Patients with neurocognitive deterioration detected by formal testing were excluded. A total of 17 patients with mean age of 6.9years at BCECTS diagnosis were identified. The patients' mean SWI was 60% and that dense electrical activity lasted 1.5years on average (range: 1–4.5years). Six children were formally diagnosed with learning disabilities in addition to ADD/ADHD. All of them were treated with an average of three antiepileptic medications, mainly for the purpose of normalizing the EEG, but none of them was treated with steroids or high-dose diazepam. The mean duration of follow-up was 5.5years. A cognitive or behavioral deterioration was not detected in any of them. Our data suggest that when treating a child with BCECTSs, high SWI, and school difficulties, the most critical parameter that determines the necessity of using second-line antiepileptic agents such as steroids or high-dose diazepam is a formal psychological evaluation that proves cognitive (I.Q.) decline. Otherwise, these agents may be avoided.

Levetiracetam efficacy in children with epilepsy with electrical status epilepticus in sleep

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 30–50mg/kg/day. Electroencephalography recordings and neuropsychological evaluations were performed repeatedly for 3–75months 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 3–4months 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.

Global Synchronization of Multichannel EEG in Patients With Electrical Status Epilepticus in Sleep.

In the research field of epilepsy, it is a challenge to understand the transition of brain activity to electrical status epilepticus in sleep (ESES). In this study, an S-estimator method is proposed to describe the course of global synchronization in multichannel electroencephalograph (EEG) from awake to sleep in 11 patients with ESES. The study confirms that there is a significant increase in spikes and global synchronization from awake to sleep. It is also found that global synchronization is strongly correlated with spikes. The proposed method has the potential of revealing the intrinsic features of EEG signals and the underlying brain dynamics in ESES.

The Clinical and Neurophysiological Features of Epileptic Syndromes Associated with Benign Focal Epileptiform Discharges of Childhood

Benign focal epileptiform discharge of childhood (BFEDC) is an age-dependent pattern determined in electroencephalograms (EEGs), which is associated with idiopathic benign focal epilepsy (BFE). Studies of BFE revealed symptomatic phenocopies in patients with cerebral struc- tural abnormalities in such conditions as infantile cerebral palsy and malformations. Some arguments against the «benign» nature of BFEDC are presented, since BFEDC may impair various cognitive functions and behavior (e.g., cause epileptic encephalophathies). Objective. To determine the clinical and neurophysiological features of epileptic syndromes associated with prolonged epileptiform activity during sleep in children and adolescents, as well as approaches to rational therapy. Patients and Methods . A total of 1862 children aged 2–18 admitted to the specialized Department of Psychoneurology of the Voronezh Regional Children Clinical Hospital No 1 in 2004–2007, who had epileptic seizures and non-epileptic neurological disorders, were exam- ined. The children underwent assessment of the neurological status, neuropsychological assessment, and video-EEG monitoring. The spike- wave index (SWI) was calculated and magnetic resonance imaging (MRI) of the brain was performed to register the epileptiform activity during sleep. Results and Discussion. It was demonstrated that when ISW of BFEDC patterns is >30%, evolution into epileptic encephalopathy was observed in 66% of patients (including epilepsy with electrical status epilepticus in sleep in 49% of patients and cognitive epileptiform disinte- gration in 17% of patients). The results prove the justification of prescribing antiepileptic drugs to patients with SWI ≥30% even if they have no epileptic seizures. Duo-therapy with valproate and ethosuximide or levetiracetam is most the effective therapy. Further prospective studies for children with BFEDC will give new insight into this area.

Panayiotopoulos syndrome: A case series from Turkey

The aim of the study was to evaluate the demographic, clinical, and EEG characteristics of patients with Panayiotopoulos syndrome (PS) and the course of their illness.Thirty-eight patients followed up with a diagnosis of PS between January 2011 and December 2013 were evaluated.We found high rates of personal history of febrile convulsions, breath-holding spells, and family history of febrile convulsions, afebrile convulsion/epilepsy, migraine, and breath-holding spells. Seizures started before the age of eight in 87% of the patients, and the mean age at seizure onset was 4.6years. Seizures were sleep-related in 81.5%, and autonomic status was seen in a third of the patients. The number of seizures was between 2 and 10 in 66% of the patients. The most common symptoms were ictus emeticus, eye/head deviation, and altered consciousness. Rolandic features were seen in 26% of the patients, and visual symptoms in 5%. Multifocal epileptiform discharges on EEG were identified in 84% of the patients. Two or more antiepileptic drugs were required in only 13% of the patients. Evolution to electrical status epilepticus in sleep and Gastaut-type epilepsy were seen in patients with more than ten seizures.The high rates of febrile convulsions, afebrile convulsions/epilepsy, migraine, and breath-holding spells in the patients and families suggest the importance of genetic factors and, perhaps, a common pathogenesis. However, the high rates of febrile convulsions and breath-holding spells in patients can be related to a misdiagnosis because of the similar symptoms. Despite its disturbing symptoms, PS is a benign epileptic syndrome requiring multiple antiepileptic drug use only in a small proportion of patients.

Temporoparietal Resection in a Patient With Landau-Kleffner Syndrome

Landau-Kleffner syndrome (LKS) was originally described by Landau, a neurologist, and Kleffner, a speech pathologist, in 1957 as an epileptic disorder with an acquired aphasia. LKS is a childhood epileptic encephalopathy on the electroclinical spectrum of electrical status epilepticus in sleep (ESES). ESES is an electrographic pattern in which there is significant activation of spike wave discharges throughout slow wave sleep. LKS is an electroclinical syndrome presenting with language regression, seizures, and behavior abnormalities with the electroencephalography (EEG) findings of ESES. Other related syndromes include continuous spike and wave in slow wave sleep (CSWS) and benign childhood epilepsy with centrotemporal spikes (BECTS). In contrast to these other disorders, the primary clinical manifestation of LKS is language regression. Additional clinical findings seen in LKS include attention-deficit disorder, irritability, and autistic-like behaviors. Seizures are present in 70%-80% of children with LKS and typically occur infrequently. The findings on neuroimaging are typically normal in LKS. Although this epileptic encephalopathy resolves spontaneously in adolescence, there is often permanent neurologic sequela, especially if the epilepsy is pharmacoresistant. Numerous therapies have been used in LKS, including antiepileptics such as ethosuximide, valproic acid, and benzodiazepines; corticosteroids; adrenocorticotropic hormone; and immunotherapy. In cases of pharmacoresistance, surgical therapy such as multiple subpial transections has been performed. In children with pharmacoresistant ESES with lateralization, cortical resection has been previously reported but is not commonly used, even in those with lateralized seizure onset.

Patients with Electrical Status Epilepticus in Sleep and Normal Magnetic Resonance Imaging Have Smaller Thalamic Volume Than Controls (P7.332)

Patients with Electrical Status Epilepticus in Sleep and Normal Magnetic Resonance Imaging Have Smaller Thalamic Volume Than Controls (P7.332)

Subunit composition of glutamate and gamma-aminobutyric acid receptors in status epilepticus

To describe the subunit composition of glutamate and gamma-aminobutyric acid (GABA) receptors in brain tissue from patients with different types of status epilepticus. The subunit composition of glutamate and GABA receptors was analyzed in: (1) surgical brain samples from three patients with refractory convulsive status epilepticus, three patients with electrical status epilepticus in sleep, and six patients with refractory epilepsy, and (2) brain autopsy samples from four controls who died without neurological disorders. Subunit expression was quantified with Western blotting and messenger ribonucleic acid (mRNA) expression was quantified with reverse polymerase chain reaction. Western blot analysis demonstrated the following patterns (as compared to controls): (1) alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptors: elevated GluA1/GluA2 ratio in electrical status epilepticus in sleep (465%±119) and refractory epilepsy (329%±125; p<0.01); (2) N-methyl-d-aspartate (NMDA) receptors: increased GluN2B/GluN2A ratio in electrical status epilepticus in sleep (3682%±1000) and refractory convulsive status epilepticus (3520%±751; p<0.05); (3) GABA receptors: elevated α2/α1 ratio in refractory epilepsy (321%±138; p<0.05) and refractory convulsive status epilepticus (346%±74; p<0.05); and (4) patients with underlying malformation of cortical development had increased ratios in GluA1/GluA2 (382%±149; p<0.01), GluN2B/GluN2A (3321%±1581; p<0.05) and α2/α1 (303%±86; p<0.01). Quantification of mRNA demonstrated an elevated GABRA2/GABRA1 ratio in refractory epilepsy (712; p<0.05) as compared to controls. The subunit composition of glutamate and GABA receptors in patients with status epilepticus mirrors that found in animal models of refractory status epilepticus and may promote self-sustaining seizures. Receptor subunit changes may provide additional targets for improved treatment.

O96 – 1638 Electrical status epilepticus in sleep (ESES): etiology, clinical picture and course

O96 – 1638 Electrical status epilepticus in sleep (ESES): etiology, clinical picture and course

O97 – 1635 Treatment of electrical status epilepticus in sleep (ESES): efficacy and unsolved questions

O97 – 1635 Treatment of electrical status epilepticus in sleep (ESES): efficacy and unsolved questions

Continuous Spikes and Waves during Sleep: Electroclinical Presentation and Suggestions for Management

Continuous spikes and waves during sleep (CSWS) is an epileptic encephalopathy characterized in most patients by (1) difficult to control seizures, (2) interictal epileptiform activity that becomes prominent during sleep leading to an electroencephalogram (EEG) pattern of electrical status epilepticus in sleep (ESES), and (3) neurocognitive regression. In this paper, we will summarize current epidemiological, clinical, and EEG knowledge on CSWS and will provide suggestions for treatment. CSWS typically presents with seizures around 2–4 years of age. Neurocognitive regression occurs around 5-6 years of age, and it is accompanied by subacute worsening of EEG abnormalities and seizures. At approximately 6–9 years of age, there is a gradual resolution of seizures and EEG abnormalities, but the neurocognitive deficits persist in most patients. The cause of CSWS is unknown, but early developmental lesions play a major role in approximately half of the patients, and genetic associations have recently been described. High-dose benzodiazepines and corticosteroids have been successfully used to treat clinical and electroencephalographic features. Corticosteroids are often reserved for refractory disease because of adverse events. Valproate, ethosuximide, levetiracetam, sulthiame, and lamotrigine have been also used with some success. Epilepsy surgery may be considered in a few selected patients.

Research advances in electrical status epilepticus in sleep

Research advances in electrical status epilepticus in sleep

In search of the Rosetta Stone for ESES

We read with interest the report by Sanchez Fernandez et al. (2012) describing a lack of a common language among North-American pediatric epileptologists regarding encephalopathy with electrical status epilepticus during sleep or continuous spike-and-wave during sleep (ESES/CSWS) and related conditions. In this paper, the authors suggested the use of ‘‘ESES’’ when referring to the EEG pattern, and ‘‘CSWS’’ when referring to the epileptic encephalopathy. However this suggestion was not supported by an explanation or by survey results. In our view, there is no reason to consider the term “ESES” as indicative of EEG pattern, or for “CSWS” as the syndrome. Indeed, “CSWS” is

Does the Reduction of Epileptiform Activity in Patients with Electrical Status Epilepticus in Sleep Treated with High-Dose Diazepam Persist over Time? (P03.015)

Does the Reduction of Epileptiform Activity in Patients with Electrical Status Epilepticus in Sleep Treated with High-Dose Diazepam Persist over Time? (P03.015)

Centrotemporal spikes during NREM sleep: The promoting action of thalamus revealed by simultaneous EEG and fMRI coregistration.

Benign childhood epilepsy with centrotemporal spikes (BECTS) has been investigated through EEG–fMRI with the aim of localizing the generators of the epileptic activity, revealing, in most cases, the activation of the sensory–motor cortex ipsilateral to the centrotemporal spikes (CTS). In this case report, we investigated the brain circuits hemodynamically involved by CTS recorded during wakefulness and sleep in one boy with CTS and a language disorder but without epilepsy. For this purpose, the patient underwent EEG–fMRI coregistration. During the “awake session”, fMRI analysis of right-sided CTS showed increments of BOLD signal in the bilateral sensory–motor cortex. During the “sleep session”, BOLD increments related to right-sided CTS were observed in a widespread bilateral cortical–subcortical network involving the thalamus, basal ganglia, sensory–motor cortex, perisylvian cortex, and cerebellum.In this patient, who fulfilled neither the diagnostic criteria for BECTS nor that for electrical status epilepticus in sleep (ESES), the transition from wakefulness to sleep was related to the involvement of a widespread cortical–subcortical network related to CTS. In particular, the involvement of a thalamic–perisylvian neural network similar to the one previously observed in patients with ESES suggests a common sleep-related network dysfunction even in cases with milder phenotypes without seizures. This finding, if confirmed in a larger cohort of patients, could have relevant therapeutic implication.

Response to treatment with methylprednisolone pulses in children with electrical status epilepticus in sleep (ESES)

No abstract available