Focal epilepsy with sensory seizures associated with arginine:glycine amidinotransferase deficiency: A clinical and advanced magnetic resonance imaging study

Abstract

We aim to determine whether epilepsy can be considered part of the arginine:glycine amidinotransferase (AGAT) deficiency syndrome phenotype and to identify its associated electroclinical signatures. We reviewed clinical data from our center, identifying individuals with AGAT deficiency. Each individual underwent a dedicated epilepsy assessment with electroencephalography and 3-T brain magnetic resonance imaging (MRI). Additionally, 30 age- and sex-matched healthy controls (18 females, 28.2 ± 3.7 years old) were recruited for advanced MRI analysis. A family with four affected members carrying homozygous AGAT c.446>A:p.Trp149* ...

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Predictors of atypical language lateralization in focal epilepsy: A mega‐analysis of fMRI evidence

Abstract

Objective

To identify predictors of language lateralization derived from functional magnetic resonance imaging (fMRI) in children and adults with left- and right-sided focal epilepsy.

Methods

We conducted a mega-analysis of data from 914 individuals from 24 samples. We used multilevel models to identify predictors of language lateralization in left and right hemisphere epilepsy groups. We assigned each participant a clinical predictor score to explore whether there was a cumulative influence of predictors on increasing atypical language lateralization.

Results

Left hemisphere epilepsy was a predictor of ...

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Development and validation of International Classification of Diseases, 9th and 10th Revision, Clinical Modification‐based algorithms to identify adult epilepsy in electronic health records

Abstract

Objective

Electronic health records (EHRs) are increasingly used to conduct research and evaluate epilepsy quality of care. We examined the accuracy of International Classification of Diseases, 9th and 10th Revision, Clinical Modification (ICD-9-CM, ICD-10-CM)- and antiseizure medicine (ASM)-based algorithms for adult epilepsy.

Methods

Data from a diverse New York multicenter EHR were queried to identify encounters between January 1, 2012 and September 20, 2018 coded with an epilepsy/seizure ICD-CM code or an ASM. Eight hundred adults were randomly selected (350 with epilepsy-related codes, ...

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Automated detection of interictal epileptiform discharges with few electroencephalographic channels

Abstract

Interictal epileptiform discharges (IEDs) are crucial for epilepsy diagnosis and management. New electroencephalographic (EEG) devices with fewer electrodes are more accessible, but their ability to detect IEDs is uncertain. The aim of this study is to determine whether IEDs can be reliably detected in reduced-channel EEG data, enabling broader epilepsy diagnosis. Using EEG samples from 3378 patients and an external validation set of 51 patients, we trained Cyclops, a deep neural network designed to function across various channel configurations. Performance ...

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Feasibility of and patient satisfaction with Readiness Brain Operation Optimization Training, a manualized cognitive prehabilitation program for epilepsy surgery

Abstract

Objective

Readiness Brain Operation Optimization Training (ReBOOT) is a manualized cognitive prehabilitation program for patients considering epilepsy surgery designed to improve presurgical preparedness through education and reduce the impact of postsurgical cognitive changes with preemptive cognitive compensatory training. The purpose of this study was to understand the feasibility of cognitive prehabilitation, examine patient satisfaction with ReBOOT, and assess changes in compensatory strategy use following ReBOOT.

Methods

Seventeen participants (65% female, mean age = 41 years) considering epilepsy surgery were enrolled in ReBOOT, which includes two one-on-one ...

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On‐scalp magnetoencephalography based on optically pumped magnetometers to investigate temporal lobe epilepsy

Abstract

Cryogenic magnetoencephalography (MEG) has a lower yield in temporal lobe epilepsy (TLE) than in extratemporal lobe epilepsy. The advent of optically pumped magnetometers (OPMs) might change this situation thanks to on-scalp MEG. This study aims to investigate the detection/localization accuracy of interictal epileptiform discharges (IEDs) using on-scalp MEG in TLE and the added value of face-OPMs. Eleven patients underwent a 1-h MEG recording with OPMs placed both on the scalp (flexible cap, scalp-OPMs) and on the face (glasseslike structure, face-OPMs). ...

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Appropriate selection of participants in pediatric developmental and epileptic encephalopathy trials: Lessons learned and future opportunities

Abstract

We evaluate the impact of independent, central review of epilepsy syndromes and seizure classification in randomized controlled treatment trials of Lennox–Gastaut syndrome (LGS) and Dravet syndrome (DS). Central review of two recent trials determined that 5.6% of enrolled DS participants and 3.4% of enrolled LGS participants pending randomization did not meet criteria for the epilepsy syndrome under study. Furthermore, 23% of DS participants and 32% of LGS participants had at least one seizure reclassified; 63.8% of DS sites and 78% ...

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Electroconvulsive therapy for super refractory status epilepticus: A scoping review

Abstract

Objective

Status epilepticus (SE) is a neurologic emergency. Although evidence-based treatments exist for SE, treatment of refractory status epilepticus (RSE) and super refractory status epilepticus (SRSE) lacks evidence. Electroconvulsive therapy (ECT) is safe and efficacious when used to treat psychiatric disorders. Seizure control attributed to ECT in the setting of SRSE has been reported in case reports and case series. The objective of this scoping review was to understand the current knowledge regarding the safety and efficacy of ECT in aborting ...

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Performance of cryptogenic new onset refractory status epilepticus score in a Brazilian cohort after testing for antineuronal antibodies with tissue‐based and cell‐based assays

Abstract

Objective

This study was undertaken to describe a case series of Brazilian new onset refractory status epilepticus (NORSE) patients and evaluate the sensitivity and specificity of a clinical score to predict cryptogenic etiology (c-NORSE score) after autoimmune encephalitis (AE) testing.

Methods

Thirty-seven patients with NORSE from the Brazilian Autoimmune Encephalitis Network were investigated with brain magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) analysis, and complementary testing with tissue-based assays and cell-based assays for antineuronal and antiglial antibodies (abs) in serum/CSF. Final diagnoses were ...

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Patterns of phosphorylated tau accumulation in a spectrum of acquired and developmental brain lesions associated with refractory epilepsy

Abstract

Objective

Phosphorylated tau (pTau) has been reported in surgical resections in refractory epilepsy. It is unclear whether this is activity-driven physiological pTau or signifies the advent of neurodegenerative cascades, relevant to memory decline. To date, primarily hippocampal sclerosis and focal cortical dysplasia (FCD) type II have been studied. We aimed to explore pTau in a range of acquired and developmental epileptogenic pathologies to assess its prevalence and identify potential drivers.

Method

A total of 104 cases were studied representing FCD IA (n = 11), ...

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