Exploration of urine metabolic biomarkers for new-onset, untreated pediatric epilepsy: A gas and liquid chromatography mass spectrometry-based metabolomics study.

Objective: The discovery of objective indicators for recent epileptic seizures will help confirm the diagnosis of epilepsy and evaluate therapeutic effects. Past studies had shortcomings such as the inclusion of patients under treatment and those with various etiologies that could confound the analysis results significantly. We aimed to minimize such confounding effects and to explore the small molecule biomarkers associated with the recent occurrence of epileptic seizures using urine metabolomics.

Effective Epilepsy Surgery for Post-Traumatic West Syndrome Following Abusive Head Trauma.

West syndrome, an infantile developmental and epileptic encephalopathy with a deleterious impact on long-term development, requires early treatment to minimize developmental abnormality; in such cases, epilepsy surgery should be considered a powerful therapeutic option. We describe a 10-month-old female admitted with West syndrome associated with a hemispheric lesion following abusive head trauma. Her seizures were suppressed by hemispherotomy at 12 months of age, leading to developmental improvement.

Different pharmacoresistance of focal epileptic spasms, generalized epileptic spasms, and generalized epileptic spasms combined with focal seizures.

Objective: Among standard treatments for infantile spasms, adrenocorticotropic hormone (ACTH) is reported as the best treatment, but ACTH is ineffective in one-half of the patients. To establish precision medicine, we examined pharmacoresistance of focal epileptic spasms (ES), generalized ES, and generalized ES combined with focal seizures, diagnosed based on the revised seizure classification of ILAE in 2017.

Methods: We conducted a retrospective nationwide study in Japan on the long-term seizure outcome of ES.

Case Report: High-Gamma Oscillations on an Ictal Electroencephalogram in a Newborn Patient With Hypoxic-Ischemic Encephalopathy.

Fast oscillations (FOs) >40 Hz in electroencephalograms (EEGs) are associated with ictogenesis and epileptogenesis in adults and children with epilepsy. However, only a few previous studies showed FOs in neonates. Reported frequencies of such neonatal FOs were in the low-gamma (<60 Hz) band and, therefore, they were not high compared to those in pediatric patients.

Incidence and risk factors of acute encephalopathy with biphasic seizures in febrile status epilepticus.

Objective: To clarify the incidence and risk factors of acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) in pediatric patients with febrile status epilepticus (FSE).

Methods: We retrospectively surveyed patients with FSE (≥20 min and ≥40 min) who were younger than 6 years by mailing a questionnaire to 1123 hospitals in Japan. The survey period was 2 years.

High-frequency EEG activity in epileptic encephalopathy with suppression-burst.

Objective: We explored high-frequency activity in the suppression-burst (SB) pattern of interictal electroencephalogram (EEG) in early infantile epileptic encephalopathy including Ohtahara syndrome (OS) and early myoclonic encephalopathy (EME) to investigate the pathophysiological characteristics of SB.

Methods: Subjects included six patients with the SB EEG pattern related to OS or EME (Group SB). The results were evaluated in comparison to tracé alternant (TA) observed during the neonatal period in nine patients to rule out possible nonspecific relationships between high-frequency activity and periodic EEG patterns (Group TA).

Manifestation of both emetic seizures and sylvian seizures in the same patients with benign partial epilepsy.

Purpose: Benign childhood epilepsy with centro-temporal spikes (BECTS) and Panayiotopoulos syndrome (PS) have different pathophysiologies and show different types of seizures, yet they overlap in some important respects. In an attempt to understand the ways in which they differ from each other and overlap each other, we performed a detailed investigation on patients who had both characteristic types of seizure manifestations, namely, sylvian seizures and emetic seizures.

Subjects And Methods: We recruited consecutive subjects from the EEG database of outpatients who had visited our hospital between 2008 and 2010 and who had been diagnosed with BECTS or PS.

Five pediatric cases of ictal fear with variable outcomes.

Purpose: Ictal fear is an uncommon condition in which fear manifests as the main feature of epileptic seizures. The literature has suggested that ictal fear is generally associated with poor seizure outcomes. We wanted to clarify the variability in seizure outcome of children with ictal fear.

Inhalation of 10% carbon dioxide rapidly terminates Scn1a mutation-related hyperthermia-induced seizures.

The aim of this study was to assess the anticonvulsant effect of carbon dioxide (CO2) on Scn1a mutation-related febrile seizures. We examined physiological changes in the blood gas levels after the induction of hyperthermia-induced seizures (HISs), which were associated with the Scn1a missense mutation. We determined the efficacy of inhalation of 5% or 10% CO2 to treat HISs.

Effects of intravenous diazepam on high-frequency oscillations in EEGs with CSWS.

High-frequency oscillations (HFOs) associated with continuous spike-waves during slow-wave sleep (CSWS) are speculated to be linked to the disturbance of higher brain function. We intended to investigate the generative mechanisms of HFOs in CSWS by clarifying the effects of intravenous injection (IV) of diazepam (DZP), an agonist for the gamma-aminobutyric acid A (GABAA) receptor in the GABAergic interneuron system, in patients who had previously been treated with IV DZP. The subjects were three patients with epilepsy with CSWS.

High-frequency EEG oscillations in hyperthermia-induced seizures of Scn1a mutant rats.

We examined high-frequency oscillations (HFOs) in the ictal cortical EEGs of hyperthermia-induced seizures in a rat model of febrile seizures with an SCN1A mutation as a means of investigating the pathophysiological mechanisms underlying the generation of febrile seizures. We used 13 male homozygous Scn1a-N1417H mutant rats (F344/NSlc-Scn1a(Kyo811)) and 10 wild-type control rats. Generalized tonic-clonic seizures were induced in all mutant rats, and HFOs with frequencies ranging from 200 to 400 Hz were found to precede spikes during the clonic phases of these seizures in the ictal EEGs.

Amplitude-integrated EEG colored according to spectral edge frequency.

Objective: To improve the interpretability of figures containing an amplitude-integrated electroencephalogram (aEEG), we devised a color scale that allows us to incorporate spectral edge frequency (SEF) information into aEEG figures. Preliminary clinical assessment of this novel technique, which we call aEEG/SEF, was performed using neonatal and early infantile seizure data.

Methods: We created aEEG, color density spectral array (DSA), and aEEG/SEF figures for focal seizures recorded in seven infants.

High-frequency oscillations in idiopathic partial epilepsy of childhood.

Purpose: We explored high-frequency oscillations (HFOs) in scalp sleep electroencephalography (EEG) studies of patients with idiopathic partial epilepsy (IPE) of childhood in order to obtain a better understanding of the pathologic mechanisms underlying IPE.

Methods: The subjects were 45 patients, including 32 with benign childhood epilepsy with centrotemporal spikes (BCECTS) and 13 with Panayiotopoulos syndrome (PS). A total of 136 EEG records were investigated through temporal expansion and filtering of traces and time-frequency spectral analysis.

Scalp-recorded high-frequency oscillations in childhood sleep-induced electrical status epilepticus.

Because high-frequency oscillations (HFOs) may affect normal brain functions, we examined them using electroencephalography (EEG) in epilepsy with continuous spike-waves during slow-wave sleep (CSWS), a condition that can cause neuropsychological regression. In 10 children between 6 and 9 years of age with epilepsy with CSWS or related disorders, we investigated HFOs in scalp EEG spikes during slow-wave sleep through temporal expansion of the EEG traces with a low-cut frequency filter at 70 Hz as well as through time-frequency power spectral analysis. HFOs (ripples) concurrent with spikes were detected in the temporally expanded traces, and the frequency of the high-frequency peak with the greatest power in each patient's spectra ranged from 97.

Spectral analysis of EEG gamma rhythms associated with tonic seizures in Lennox-Gastaut syndrome.

Purpose: EEG gamma rhythms, which are found in association with epileptic spasms in infants with West syndrome, were explored in the ictal EEGs of tonic seizures in older patients with Lennox-Gastaut syndrome (LGS) to investigate the pathophysiology of the disease.

Methods: The subjects were 20 patients with LGS (11 males, 9 females; age range: 3 years 1 month to 29 years 3 months) who had at least one digitally recorded tonic seizure with minimal artifacts. A time-frequency analysis was applied to each patient's ictal EEG data.

Spectral characteristics of EEG gamma rhythms associated with epileptic spasms.

To elucidate the pathophysiology of epileptic spasms, unaveraged time-frequency spectra of spasm-associated EEG gamma rhythms were investigated in 15 patients with West syndrome or related disorders. Using these unaveraged spectra, we were able to investigate in detail various aspects of the structure of ictal gamma rhythms that could not be examined using averaged spectra. The characteristics of the ictal gamma peaks (peak frequency, power, duration, and the number of peaks in each brain-region for each spasm) were statistically evaluated with respect to their differences among the brain regions and over the time-course of the clusters.

Rasmussen encephalitis associated with SCN 1 A mutation.

Mutations in the SCN 1 A gene, encoding the neuronal voltage-gated sodium channel alpha1 subunit, cause SMEI, GEFS+, and related epileptic syndromes. We herein report the R1575C-SCN 1 A mutation identified in a patient with Rasmussen encephalitis. R1575C were constructed in a recombinant human SCN 1 A and then heterologously expressed in HEK293 cells along with the human beta1 and beta2 sodium channel accessory subunits.

Epilepsy development in infancy with epileptic discharges.

As a part of the study to prevent West syndrome (WS) by early treatment, we assessed what kind of epilepsy developed in infants who showed epileptic discharges in early infancy. EEG examinations were performed on 116 infants born from 1997 to September 2004, both before and after 3 months of corrected age (CA). We divided 45 infants who showed epileptic discharges in early infancy into two groups according to the existence of periventricular leukomalacia (PVL) and retrospectively performed the course observation at the survey point on April 1 in 2005.

EEG dipole characteristics in Panayiotopoulos syndrome.

Purpose: Panayiotopoulos syndrome (PS) is a type of benign childhood partial epilepsy that is frequently associated with abundant multifocal spikes other than main occipital spikes on the EEG. In this study, we investigated the characteristic features of dipoles in PS.

Methods: We performed dipole analysis of the interictal occipital spike discharges seen in 10 children with PS (group A) and in 10 children with other types of symptomatic localization-related epilepsy (group B).

Characteristics of slow waves on EEG associated with epileptic spasms.

Purpose: The high-voltage slow waves (HVSs) on EEG associated with epileptic spasms were investigated to clarify their characteristics and their relation to the pathophysiology of spasms in West syndrome and related disorders.

Methods: In 14 patients, digitally recorded EEG segments showing the ictal HVSs were extracted and their traces were overlaid by using an average reference. The ictal HVSs were also averaged to build maps for investigation of the pattern of potential distribution over the scalp.

Very fast rhythmic activity on scalp EEG associated with epileptic spasms.

Purpose: Very fast activity was investigated on the ictal EEGs of epileptic spasms to elucidate the pathophysiology of West syndrome (WS) and related disorders from a novel point of view.

Methods: The traces of scalp ictal EEG of spasms temporally were expanded in 11 patients whose clinical diagnosis was symptomatic WS in six, cryptogenic WS in two, Aicardi syndrome in one, and symptomatic generalized epilepsy after WS in the remaining two. Time evolution of averaged power spectra of the ictal fast activity also was analyzed in each patient.