Epilepsia. Official Journal of the International League Against Epilepsy

Abstract

Objective

To assess pharmacokinetics and safety of diazepam nasal spray (NRL‐1; VALTOCO®) in pediatric and adult patients with epilepsy in seizure and nonseizure states.

Methods

A single dose of diazepam nasal spray (5, 10, 15, or 20 mg based on weight) was administered during each of two conditions (ictal/peri‐ictal and interictal condition) to patients 6‐65 years old with partial or generalized epilepsy with motor seizures or seizures with clear alteration of awareness; a second dose was permitted if needed for persistent seizures. Dosing could be ...

Abstract

Objective

To evaluate the interactions of metabolic neuronal‐glial changes with the presence and hemispheric‐side of hippocampal sclerosis (HS) and its potential role in predicting pharmacoresistance in temporal lobe epilepsy (TLE).

Methods

We included structural magnetic resonance imaging (MRI) and proton magnetic resonance spectroscopy (¹H‐MRS) metabolic data for 91 patients with unilateral TLE and 50 healthy controls. We measured the values of total N‐acetyl aspartate/total creatine (tNAA/tCr), glutamate/tCr (Glu/tCr), and myo‐inositol/tCr (mIns/tCr). To assess the influence of the pharmacoresponse and hemispheric‐side of HS on ...

Summary

Our aim was to clarify the incidence and risk of acute symptomatic seizures in people with coronavirus disease 2019 (COVID‐19). This multicenter retrospective study enrolled people with COVID‐19 from 18 January to 18 February 2020 at 42 government‐designated hospitals in Hubei province, the epicenter of the epidemic in China; Sichuan province; and Chongqing municipality. Data were collected from medical records by 11 neurologists using a standard case report form. A total of 304 people were enrolled, of whom 108 had ...

Abstract

The aim of this study was to investigate the influence of concomitant antiepileptic drugs (AEDs) on brivaracetam (BRV) trough serum concentrations. A total number of 368 routinely collected blood samples from 148 inpatients from Mara Hospital (Bethel Epilepsy Center) and von Bodelschwingh Foundation Bethel were retrospectively evaluated. Generalized estimation equations (GEEs) were used for statistical analysis. GEE analyses showed that BRV trough serum concentrations were significantly lower in patients with strong enzyme‐inducing AEDs (carbamazepine, phenytoin, and/or phenobarbital/primidone, −49%), but were ...

Abstract

Objective

Tuberous sclerosis complex (TSC) is one of the most common genetic causes of epilepsy. Seizures in TSC typically first present in infancy or early childhood, including focal seizures and infantile spasms. Infantile spasms in TSC are particularly characteristic in its strong responsiveness to vigabatrin. Although a number of mouse models of epilepsy in TSC have been described, there are very limited electroencephalographic (EEG) or seizure data during the preweanling neonatal and infantile‐equivalent mouse periods. Tsc1
^GFAPCKO mice are a well‐characterized ...

Abstract

Objective

To delineate the epileptic phenotype of LAMA2‐related muscular dystrophy (MD) and correlate it with the neuroradiological and muscle biopsy findings, as well as the functional motor phenotype.

Methods

Clinical, electrophysiological, neuroradiological, and histopathological data of 25 patients with diagnosis of LAMA2‐related MD were analyzed.

Results

Epilepsy occurred in 36% of patients with LAMA2‐related MD. Mean age at first seizure was 8 years. The most common presenting seizure type was focal‐onset seizures with or without impaired awareness. Visual aura and autonomic signs, including vomiting, were frequently ...

Abstract

Objective

The “adenosine hypothesis of SUDEP” (sudden unexpected death in epilepsy) predicts that a seizure‐induced adenosine surge combined with impaired metabolic clearance can foster lethal apnea or cardiac arrest. Changes in adenosine receptor density and adenosine kinase (ADK) occur in surgical epilepsy patients. Our aim was to correlate the distribution of ADK and adenosine A_2A and A₁ receptors (A_2AR and A₁R) in surgical tissue from patients with temporal lobe epilepsy and hippocampal sclerosis (TLE/HS) with SUDEP risk factors.

Methods

In 75 cases, patients ...

Abstract

Objective

The present study tested whether ictal onset sites are regions of more severe interneuron loss in epileptic pilocarpine‐treated rats, a model of human temporal lobe epilepsy.

Methods

Local field potential recordings were evaluated to identify ictal onset sites. Electrode sites were visualized in Nissl‐stained sections. Adjacent sections were processed with proximity ligation in situ hybridization for glutamic acid decarboxylase 2 (Gad2). Gad2 neuron profile numbers at ictal onset sites were compared to contralateral regions. Other sections were processed with immunocytochemistry for reelin ...

Abstract

Objective

Early onset epileptic encephalopathy with suppression‐burst is one of the most severe epilepsy phenotypes in human patients. A significant proportion of cases have a genetic origin, and the most frequently mutated gene is KCNQ2, encoding Kv7.2, a voltage‐dependent potassium channel subunit, leading to so‐called KCNQ2‐related epileptic encephalopathy (KCNQ2‐REE). To study the pathophysiology of KCNQ2‐REE in detail and to provide a relevant preclinical model, we generated and described a knock‐in mouse model carrying the recurrent p.(Thr274Met) variant.

Methods

We introduced the p.(Thr274Met) variant ...

Abstract

This article emphasizes the role of the technological progress in changing the landscape of epilepsy surgery and provides a critical appraisal of robotic applications, laser interstitial thermal therapy, intraoperative imaging, wireless recording, new neuromodulation techniques, and high‐intensity focused ultrasound. Specifically, (a) it relativizes the current hype in using robots for stereo‐electroencephalography (SEEG) to increase the accuracy of depth electrode placement and save operating time; (b) discusses the drawback of laser interstitial thermal therapy (LITT) when it comes to the need ...