The Use of Perampanel in the Treatment of Lance-Adams Syndrome.

Lance Adams syndrome (LAS) is characterized by chronic action or intention myoclonus resulting from cerebral hypoxia. Perampanel, a non-competitive antagonist of aamino-3-hydroxy-5methyl-4 isooxazoleproprionic acid glutamate receptor, has demonstrated some efficacy in myoclonic epilepsy and other types of myoclonus. We report significant benefit in a patient with LAS treated with add on perampanel and provide a review of the relevant literature.

Factors associated with placebo response rate in randomized controlled trials of antiseizure medications for focal epilepsy.

Objective: Randomized controlled trials (RCTs) are necessary to evaluate the efficacy of novel treatments for epilepsy. However, there have been concerning increases in the placebo responder rate over time. To understand these trends, we evaluated features associated with increased placebo responder rate.

Networks through the lens of high-frequency oscillations.

To date, there is no neurophysiologic or neuroimaging biomarker that can accurately delineate the epileptogenic network. High-frequency oscillations (HFO) have been proposed as biomarkers for epileptogenesis and the epileptogenic network. The pathological HFO have been associated with areas of seizure onset and epileptogenic tissue.

External validation of the Memory Assessment Clinics Scale for Epilepsy (MAC-E).

Objective: This study aimed to externally validate the Memory Assessment Clinics Scale for Epilepsy (MAC-E), a brief self-report measure of subjective memory complaints in adults with epilepsy.

Methods: A cross-sectional study was conducted including adults with focal pharmacoresistant epilepsy from three Level 4 epilepsy centers in the U.S.

Epilepsy surgery education and practice around the globe: An ILAE taskforce report.

Up to 80% of the world's population with epilepsy lives in low and middle-income countries. Around one-third of these patients will have drug-resistant epilepsy, for which epilepsy surgery is an option. Unfortunately, many of these regions, as well as some more developed nations, lack sufficient epilepsy surgery units and trained neurosurgeons.

Combining three-phase EEG pattern and ipsilateral isolated eye deviation to predict seizure focus in the frontal interhemispheric fissure.

Objective: Identification of seizure focus on the interhemispheric fissure (IHF) is often challenging at the step of scalp video-EEG monitoring on preoperative evaluations. We previously reported ictal three-phase EEG patterns (3Ph-EEG) and isolated eye deviation (isolated ED) to side of the seizure focus during seizures, each of which is available for identifying the seizure focus for frontal IHF (F-IHF). This study aimed to evaluate the accuracy of predicting the F-IHF focus using a combination of 3Ph-EEG and isolated ED in patients with frontal lobe epilepsy and the accuracy of predicting lateralization of the F-IHF focus using the predominance of findings.

Raphe and ventrolateral medulla proteomics in sudden unexplained death in childhood with febrile seizure history.

Sudden unexplained death in childhood (SUDC) is death of a child ≥ 12 months old that is unexplained after autopsy and detailed analyses. Among SUDC cases, ~ 30% have febrile seizure (FS) history, versus 2-5% in the general population. SUDC cases share features with sudden unexpected death in epilepsy (SUDEP) and sudden infant death syndrome (SIDS), in which brainstem autonomic dysfunction is implicated.

Neuropsychological Outcomes in 6-Year-Old Children of Women With Epilepsy: A Prospective Nonrandomized Clinical Trial.

Importance: Antiseizure medications (ASMs) are potential teratogens commonly prescribed for multiple indications. ASM fetal exposure can impair neurodevelopment. Folate improves pregnancy outcomes, but higher doses may pose risks.

Corpus callosotomy for refractory epileptic spasms: Systematic review and meta-analysis.

Objective: We systematically reviewed the existing literature on the efficacy of corpus callosotomy (CC) in children and adults with refractory epileptic spasms (ES) and analyzed clinical determinants of seizure outcomes.

Methods: The Preferred Report Items for Systematic Reviews and Meta-Analysis Guidelines (PRISMA) were followed. We systematically searched MEDLINE, EMBASE and Cochrane databases up to December 2023 for original research articles on using CC to treat refractory ES.

Cerebellar transcranial magnetic stimulation to treat drug-resistant epilepsy: A randomized, controlled, crossover clinical trial.

Objective: Epilepsy is one of the most prevalent brain diseases. Approximately one third of patients consistently experience drug-resistant epilepsy (DRE), a condition where seizures persist despite the use of antiseizure medications. Exploration of new therapies for DRE is urgently needed.

Evaluation of self-stigma in patients with epilepsy: Validation of the self-stigma scale to Spanish (ESSS-S).

Objective: The Epilepsy Self-Stigma Scale (ESSS) has been developed in Japan for patients with epilepsy (PWE). We aimed to validate the scale in Spanish and examine its validity and reliability.

Methods: The transcultural adaptation of the ESSS was conducted using translation and back-translation, along with pilot testing and an expert panel review.

Return to full baseline functionality after repeated intermittent use of midazolam nasal spray in patients with seizure clusters: Post hoc analysis of an open-label extension trial.

Objective: To characterize the time to return to full baseline functionality (RTFBF) in seizure cluster episodes (SCEs) treated with one or two midazolam nasal spray (MDZ-NS/Nayzilam®) doses over the course of repeated intermittent use in patients with seizure clusters (SCs).

Methods: Post hoc analysis of an open-label extension trial in patients (≥12 years) with SCs (ARTEMIS-2/P261-402: NCT01529034, 2011-004109-25). Caregivers administered MDZ-NS 5 mg when patients experienced an SC; a second 5-mg dose could be given if seizures did not terminate within 10 min or recurred within 10 min-6 h.

Cerebellar activity in PINK1 knockout rats during volitional gait.

Preclinical models of Parkinson's disease are imperative to gain insight into the neural circuits that contribute to gait dysfunction in advanced stages of the disease. A PTEN-induced putative kinase 1 knockout early-onset model of Parkinson's disease may be a useful rodent model to study the effects of neurotransmitter degeneration caused by a loss of PTEN-induced putative kinase 1 function on brain activity during volitional gait. The goal of this study was to measure changes in neural activity at the cerebellar vermis at 8 months of age.

Cerebellar activity in hemi-parkinsonian rats during volitional gait and freezing.

Parkinson's disease is a neurodegenerative disease characterized by gait dysfunction in the advanced stages of the disease. The unilateral 6-hydroxydopamine toxin-induced model is the most studied animal model of Parkinson's disease, which reproduces gait dysfunction after >68% dopamine loss in the substantia nigra pars compacta. The extent to which the neural activity in hemi-parkinsonian rats correlates to gait dysfunction and dopaminergic cell loss is not clear.

Associations Between Testing and Treatment Pathways in a Case of Pediatric Nonlesional Epilepsy: A Census Survey of NAEC Center Directors.

Objective: Epilepsy surgery is vital in managing of children with drug-resistant epilepsy. Noninvasive and invasive testing modalities allow for evaluation and treatment of children with drug-resistant epilepsy. Evidence-based algorithms for this process do not exist.

The human claustrum tracks slow waves during sleep.

Slow waves are a distinguishing feature of non-rapid-eye-movement (NREM) sleep, an evolutionarily conserved process critical for brain function. Non-human studies suggest that the claustrum, a small subcortical nucleus, coordinates slow waves. We show that, in contrast to neurons from other brain regions, claustrum neurons in the human brain increase their spiking activity and track slow waves during NREM sleep, suggesting that the claustrum plays a role in coordinating human sleep architecture.

Temporal integration in human auditory cortex is predominantly yoked to absolute time, not structure duration.

Sound structures such as phonemes and words have highly variable durations. Thus, there is a fundamental difference between integrating across absolute time (e.g.

Generalized spike-waves in idiopathic generalized epilepsies: Does their frequency matter?

Objectives: We hypothesized that the frequency (in Hertz) of generalized spike-waves (GSWs) in patients with idiopathic generalized epilepsy (IGE) has associations with the syndromic diagnosis as well as with the prognosis of patients (their response to medical treatment).

Methods: This was a retrospective study of a prospectively developed database. All patients with a diagnosis of IGE were studied at the epilepsy center at Shiraz University of Medical Sciences, Shiraz, Iran, from 2008 until 2022.

A Retrospective Review of Reclassification of Variants of Uncertain Significance in a Pediatric Epilepsy Cohort Undergoing Genetic Panel Testing.

Background: The interpretation and communication of variant of uncertain significance (VUS) genetic results often present a challenge in clinical practice. VUSs can be reclassified over time into benign/likely benign (B/LB) or pathogenic/likely pathogenic (P/LP) based on the availability of updated data. We evaluate the frequency of VUS reclassification in our tertiary care epilepsy cohort undergoing epilepsy genetic panel (EGP) testing.

Sleep-related hypermotor seizures originating from the occipital lobe.

We present two unique cases of sleep-related hypermotor epilepsy (SHE) originating from the occipital lobe. Patients with sleep-related seizures and drug-resistant occipital lobe epilepsy were identified from the ANPHY lab stereo-electroencephalography (SEEG) research database at the Duke Comprehensive Epilepsy Center. We identified two young females with frequent sleep-related focal seizures and occasional focal to bilateral tonic clonic seizures characterized by hypermotor movements.