Epilepsia Open

OBJECTIVE: Electroencephalographic (EEG) abnormalities especially non-convulsive status epilepticus (NCSE), have been found to be associated with worse outcomes in critically ill patients. We aimed to assess the prevalence of non-convulsive seizures and electroencephalographic abnormalities in critically ill patients. Furthermore, we aimed to investigate any association between the type of EEG abnormality and outcomes including ICU mortality and successful ICU discharge. METHODS: This was a cross-sectional observational study carried out among critically ill patients in a mixed medical-surgical ICU from January 1st, 2018 to May 15th, 2020...

OBJECTIVE: To evaluate the pharmacokinetics (PK), safety and tolerability of brivaracetam (BRV) in neonates with repeated electroencephalographic seizures not controlled with previous antiseizure medications (ASMs). METHODS: Phase 2/3, multicenter, open-label, single-arm study (N01349/NCT03325439) in neonates with repeated electroencephalographic seizures (lasting ≥10 seconds) confirmed by video-electroencephalography, and inadequate seizure control with ≥1 ASMs...

OBJECTIVE: Collaboration among medical facilities is crucial to deliver comprehensive epilepsy care to a diverse and large population of people with epilepsy. We conducted a survey among medical facilities of various sizes throughout Japan to investigate the status of epilepsy care delivery, functioning, and referral. METHODS: With the cooperation of the Japan Neurological Society (1,428 facilities), Japanese Neurosurgical Society (3,489 specialists), and Epilepsy Care Network (948 facilities), a questionnaire was mailed to 5,865 locations that provide epilepsy care in Japan...

Stroke is one of the most common causes of acquired epilepsy, which can also result in disability and increased mortality rates particularly in elderly patients. No preventive treatment for post-stroke epilepsy is currently available. Development of such treatments has been greatly limited by the lack of biomarkers to reliably identify high risk patients. The glymphatic system, including perivascular spaces (PVS) is the brain's waste clearance system and enlargement or asymmetry of PVS (ePVS) is hypothesized to play a significant role in the pathogenesis of several neurological conditions...

OBJECTIVE: In patients with Dravet syndrome (DS), fenfluramine reduced convulsive seizure frequency and provided clinical benefit in nonseizure endpoints (e.g., executive function, survival). In zebrafish mutant scn1 DS models, chronic fenfluramine treatment preserved neuronal cytoarchitecture prior to seizure onset and prevented gliosis; here, we extend these findings to a mammalian model of DS (Scn1a+/- mice) by evaluating the effects of fenfluramine on neuroinflammation (degenerated myelin, activated microglia) and survival...

OBJECTIVE: We aimed to investigate differences in episodic memory networks between patients with temporal lobe epilepsy (TLE) due to hippocampal sclerosis and healthy controls, especially with regards to the parietal memory network (PMN), as well as their relation to neuropsychological memory performance after mesial temporal resection. METHODS: 28 healthy subjects as well as 21 patients with TLE (12 left, 9 right) were investigated using a spatial memory fMRI paradigm, which has been shown to activate the PMN...

OBJECTIVE: To report the interim results of the PERPRISE study (Study 509; NCT04202159), which is evaluating perampanel as the only adjunctive anti-seizure medication (ASM) in adults with focal to bilateral tonic-clonic seizures (FBTCS) or primary generalized tonic-clonic seizures (GTCS). METHODS: PERPRISE is an ongoing 12-month multicenter, prospective, observational, non-interventional study of perampanel in a real-world setting in Germany. Patients are aged ≥18 years with FBTCS or GTCS due to focal or idiopathic generalized epilepsy...

Neuromodulation by means of vagus nerve stimulation (VNS) therapy, reduces seizure frequency and improves quality of life in subjects with drug-resistant epilepsy (DRE), yet its molecular mechanism remains unclear. This study investigates the impact of chronic VNS on lipid bioactive metabolites and fatty acids (FA) in the plasma and red blood cells of seven subjects with DRE. By measuring expression levels of peroxisome proliferator-activated receptor α (PPARα) and sirtuin1 (SIRT1) genes - key regulators in energy and lipid metabolism - and lipid profiles before and after various stages of VNS, this study identifies potential mechanisms by which VNS may reduce seizure frequency...

OBJECTIVE: Neurocysticercosis (NCC) is considered the major cause of epilepsy in endemic regions. In the rural areas of the Bolivian Chaco prevalence of NCC among people with epilepsy (PWE) was 27.4%, according to a population-based survey carried out in 1994. Aim of the study was to estimate the prevalence of Epilepsy Associated with Tonic-Clonic Seizures (EATCS) and to evaluate the prevalence of NCC among PWE in the rural communities of the Bolivian Chaco after 30 years. METHODS: Twenty-two rural communities (total population 12,852) were involved in the study...

OBJECTIVE: Defects in RARS2 cause cerebellopontine hypoplasia type 6 (Pontocerebellar Hypoplasia Type 6, PCH6, OMIM: #611523), a rare autosomal recessive inherited mitochondrial disease. Here, we report two male patients and their respective family histories. METHODS: We describe the clinical presentation and magnetic resonance imaging (MRI) findings of these patients. Whole-exome sequencing was used to identify the genetic mutations. RESULTS: One patient showed hypoglycemia, high lactic acid levels (fluctuating from 6...

OBJECTIVE: We aimed to find predictors for smartphone application based Ketogenic Diet (KD) treatment effectiveness and safety. METHODS: The efficacy was evaluated according to the reduction of seizure frequency after the intervention of KD, safety was evaluated based on adverse effects. The ordinal logistic regression analysis was used to explore the influencing factors of efficacy. RESULTS: The study sample included 116 males and 65 females with a median age of 2...

OBJECTIVE: Stereotactic electroencephalography-guided three-dimensional radiofrequency thermocoagulation (SEEG-3D RFTC) is a minimally invasive treatment for medial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS). This study aimed to investigate the long-term prognosis after SEEG-3D RFTC treatment in patients with MTLE-HS. METHODS: This single-center retrospective study included 28 patients with MTLE-HS treated with SEEG-3D RFTC from January 2016 to May 2018...

OBJECTIVE: To evaluate the effectiveness and safety of perampanel (PER) monotherapy (MT) or add-on therapy (AT) in Chinese children with epilepsy, as well as to evaluate the data from routine therapeutic drug monitoring (TDM) of PER for these pediatric patients. METHODS: This retrospective and observational study was carried out on children with epilepsy (n = 340) from 2020 to 2022 at the Children's Hospital of Nanjing Medical University. Outcome measures were the responder rate (50% or greater seizure reduction), long-term efficacy, and tolerability (number and types of adverse events) in MT and AT groups...

Focal cortical dysplasia (FCD) represents the most common cause of drug-resistant epilepsy in adult and pediatric surgical series. However, genetic factors contributing to severe phenotypes of FCD remain unknown. We present a patient with an exceptionally rapid development of drug-resistant epilepsy evolving in super-refractory status epilepticus. We performed multiple clinical (serial EEG, MRI), biochemical (metabolic and immunological screening), genetic (WES from blood- and brain-derived DNA) and histopathological investigations...

OBJECTIVE: Cardio-facio-cutaneous syndrome (CFCS) is a genetic disorder due to variants affecting genes coding key proteins of the Ras/MAPK signalling pathway. Among the different features of CFCS, neurological involvement, including cerebral malformations and epilepsy, represents a common and clinically relevant aspect. Status epilepticus (SE) is a recurrent feature, especially in a specific subgroup of CFCS patients with developmental and epileptic encephalopathy (DEE) and history of severe pharmacoresistant epilepsy...

OBJECTIVE: Neurostimulation is an emerging treatment for patients with drug-resistant epilepsy, which is used to suppress, prevent, and terminate seizure activity. Unfortunately, after implantation and despite best clinical practice, most patients continue to have persistent seizures even after years of empirical optimization. The objective of this study is to determine optimal spatial and amplitude properties of neurostimulation in inhibiting epileptiform activity in an acute hippocampal seizure model...

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OBJECTIVE: Thinning of the peripapillary retinal nerve fiber layer (p-RNFL), as measured by optical coherence tomography (OCT), was recently introduced as a promising marker for cerebral neuronal loss in people with epilepsy (PwE). However, its clinical implication remains to be elucidated. We thus aimed to (1) systematically characterize the extent of the retinal neuroaxonal loss in a broad spectrum of unselected PwE and (2) to evaluate the main clinical determinants. METHODS: In this prospective study, a spectral-domain OCT evaluation was performed on 98 well-characterized PwE and 85 healthy controls (18-55 years of age)...

OBJECTIVE: Identification of EEG waveforms is critical for diagnosing Lennox-Gastaut Syndrome (LGS) but is complicated by the progressive nature of the disease. Here, we assess the interrater reliability (IRR) among pediatric epileptologists for classifying EEG waveforms associated with LGS. METHODS: A novel automated algorithm was used to objectively identify epochs of EEG with transient high power, which were termed events of interest (EOIs). The algorithm was applied to EEG from 20 LGS subjects and 20 healthy controls during NREM sleep, and 1350 EOIs were identified...

OBJECTIVE: Cenobamate is a recently approved antiseizure medication that proved to be safe and effective in randomized controlled trials. However, little is known about its impact on some areas frequently affected in epilepsy. For this reason, we explored the effects of cenobamate in cognitive performance, as well as on negative affectivity and quality of life in a sample of patients with drug-resistant epilepsy. METHODS: Two prospective cohort studies were carried out...