2,115 results match your criteria: "Comprehensive Epilepsy Center[Affiliation]"

Cortical network hyperexcitability related to synaptic dysfunction in Alzheimer's disease (AD) is a potential target for therapeutic intervention. In recent years, there has been increased interest in the prevalence of silent seizures and interictal epileptiform discharges (IEDs, or seizure tendency), with both entities collectively termed "subclinical epileptiform activity" (SEA), on neurophysiologic studies in AD patients. SEA has been demonstrated to be common in AD, with prevalence estimates ranging between 22-54%.

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Background: Genetic diversity in human leukocyte antigen (HLA) alleles across populations is a significant risk factor for drug-induced severe cutaneous adverse reactions (SCARs), e.g., carbamazepine (CBZ)- and lamotrigine (LTG)-induced Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN).

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The main causes of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis are ovarian teratoma and herpes simplex virus (HSV) encephalitis. We present a rare case of suspected anti-NMDAR encephalitis caused by corpus callosotomy (CC). An 18-year-old woman with Lennox-Gastaut syndrome underwent CC.

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Objective: The evaluation to determine candidacy and treatment for epilepsy surgery in persons with drug-resistant epilepsy (DRE) is not uniform. Many non-invasive and invasive tests are available to ascertain an appropriate treatment strategy. This study examines expert response to clinical vignettes of magnetic resonance imaging (MRI)-positive lesional focal cortical dysplasia in both temporal and extratemporal epilepsy to identify associations in evaluations and treatment choice.

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Objectives: The purpose of the current study was to apply Two-step cluster analysis on a large dataset of patients with juvenile myoclonic epilepsy (JME). We hypothesized that there are distinct subgroups of patients with similar clinical characteristics. We also hypothesized that the seizure outcome is different between these clusters.

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Malformations of cortical development (MCD) are neurological conditions involving focal disruptions of cortical architecture and cellular organization that arise during embryogenesis, largely from somatic mosaic mutations, and cause intractable epilepsy. Identifying the genetic causes of MCD has been a challenge, as mutations remain at low allelic fractions in brain tissue resected to treat condition-related epilepsy. Here we report a genetic landscape from 283 brain resections, identifying 69 mutated genes through intensive profiling of somatic mutations, combining whole-exome and targeted-amplicon sequencing with functional validation including in utero electroporation of mice and single-nucleus RNA sequencing.

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Objective: This study was undertaken to evaluate the cross-cultural application of the International Classification of Cognitive Disorders in Epilepsy (IC-CoDE) to a cohort of Spanish-speaking patients with temporal lobe epilepsy (TLE) living in the United States.

Methods: Eighty-four Spanish-speaking patients with TLE completed neuropsychological measures of memory, language, executive function, visuospatial functioning, and attention/processing speed as part of the Neuropsychological Screening Battery for Hispanics. The contribution of demographic and clinical variables to cognitive performance was evaluated.

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Article Synopsis
  • * Studied 100 patients, finding that 77% had motor FS, but no significant relationship between seizure behaviors and specific brain abnormality locations.
  • * Concluded that there are no clear associations between FS behaviors and MRI-detected brain abnormalities, suggesting a need for more research into FS behavior variations.
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Putative roles for homeostatic plasticity in epileptogenesis.

Epilepsia

March 2023

Comprehensive Epilepsy Center, Department of Neurology, University of Chicago, Chicago, Illinois, USA.

Homeostatic plasticity allows neural circuits to maintain an average activity level while preserving the ability to learn new associations and efficiently transmit information. This dynamic process usually protects the brain from excessive activity, like seizures. However, in certain contexts, homeostatic plasticity might produce seizures, either in response to an acute provocation or more chronically as a driver of epileptogenesis.

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Bi-allelic variants in NAE1 cause intellectual disability, ischiopubic hypoplasia, stress-mediated lymphopenia and neurodegeneration.

Am J Hum Genet

January 2023

Department of Metabolic Diseases, Division Pediatrics, Wilhelmina Children's Hospital University Medical Center Utrecht, Utrecht University, 3584 EA Utrecht, the Netherlands. Electronic address:

Neddylation has been implicated in various cellular pathways and in the pathophysiology of numerous diseases. We identified four individuals with bi-allelic variants in NAE1, which encodes the neddylation E1 enzyme. Pathogenicity was supported by decreased NAE1 abundance and overlapping clinical and cellular phenotypes.

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To promote health equity within the United States (US), randomized clinical trials should strive for unbiased representation. Thus, there is impetus to identify demographic disparities overall and by disease category in US clinical trial recruitment, by trial phase, level of masking, and multi-center status, relative to national demographics. A systematic review and meta-analysis were conducted using MEDLINE, Embase, CENTRAL, and ClinicalTrials.

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Stiripentol for Drug-Resistant Epilepsy Treatment in Tuberous Sclerosis Complex.

Pediatr Neurol

February 2023

Division of Neurology, Comprehensive Epilepsy Center, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.

Background: Drug-resistant epilepsy (DRE) is common in tuberous sclerosis complex (TSC). The role of stiripentol (STP) in seizure treatment in this population is not well understood. This study evaluates the efficacy and tolerability of STP in patients with TSC with DRE.

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Objective: Cannabidiol (CBD) expanded access program, initiated in 2014, provided add-on CBD to patients with treatment-resistant epilepsies (TREs) at 35 US epilepsy centers. Prior publications reported results through December 2016; herein, we present efficacy and safety results through January 2019.

Methods: Patients received plant-derived highly purified CBD (Epidiolex®; 100 mg/ml oral solution), increasing from 2 to 10 mg/kg/day to tolerance or maximum 25-50 mg/kg/day dose, depending on the study site.

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Rare Genetic Variation and Outcome of Surgery for Mesial Temporal Lobe Epilepsy.

Ann Neurol

December 2022

Department of Neuroscience, Central Clinical School, Monash University, Melbourne, Victoria, Australia.

Objective: Genetic factors have long been debated as a cause of failure of surgery for mesial temporal lobe epilepsy (MTLE). We investigated whether rare genetic variation influences seizure outcomes of MTLE surgery.

Methods: We performed an international, multicenter, whole exome sequencing study of patients who underwent surgery for drug-resistant, unilateral MTLE with normal magnetic resonance imaging (MRI) or MRI evidence of hippocampal sclerosis and ≥2-year postsurgical follow-up.

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Background: For patients with anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) and ovarian teratoma, "conservative" surgical approaches (complete or partial unilateral oophorectomy or bilateral partial oophorectomies) are associated with clinical improvement. "Aggressive" ovarian resections (complete bilateral oophorectomy or "blind" ovarian resections without pre-operative evidence of teratoma) are also reported, although the evidence supporting these approaches is unclear.

Objective: To compare the one-year functional outcomes of patients with NMDARE who underwent conservative vs.

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Objective: The significance of ictal magnetoencephalography (MEG) is not well appreciated. We evaluated the relationships between ictal MEG, MRI, intracranial electroencephalography (ICEEG), surgery and postoperative seizure outcome.

Methods: A total of 45 patients (46 cases) with ictal MEG who underwent epilepsy surgery was included.

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Coronavirus 19 (COVID-19) has infected over 400 million people worldwide. Although COVID-19 causes predominantly respiratory symptoms, it can affect other organs including the brain, producing neurological symptoms. People with epilepsy (PWE) have been particularly impacted during the pandemic with decreased access to care, increased stress, and worsening seizures in up to 22% of them probably due to multiple factors.

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Objective: We hypothesized that the international economic sanctions have affected various groups of Iranian people and have compromised their right to access medicines.

Methods: Scopus and MEDLINE from their inception to 15 May 2022 were systematically searched for related published original manuscripts. The following keywords were used (title/abstract): "sanction(s)" AND "Iran".

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Background: A Core Outcome Set (COS) is a standardised list of outcomes that should be reported as a minimum in all clinical trials. In epilepsy, the choice of outcomes varies widely among existing studies, particularly in clinical trials. This diminishes opportunities for informed decision-making, contributes to research waste and is a barrier to integrating findings in systematic reviews and meta-analyses.

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Purpose: To investigate the characteristics of patients with MRI-negative temporal lobe epilepsy (TLE) (1.5 T brain MRI) in comparison with: (i) patients with hippocampal sclerosis (HS)-TLE; (ii) persons with non-HS structural TLE; and (iii) patients with dual pathology.

Methods: This was a retrospective study.

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Background: Previous studies have shown that patients with epilepsy (PWE) perceived significant disruption in the quality and provision of care due to the coronavirus disease 2019 (COVID-19) pandemic. The present study aimed to investigate the effect of this pandemic on seizure control status and changes in seizure frequency in PWE.

Methods: A consecutive sample of adult PWE registered in the database of Shiraz Epilepsy Center (Shiraz, Iran) was included in the study.

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Objective: We aimed to inquire whether any seizure rescue medications are included in the in-flight medical emergency kits of the main airlines in the world. This data could help the airline authorities update their strategies in light of any shortcomings.

Methods: First, we identified ten major airlines in the world.

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Objective: This study was undertaken to identify molecular mechanisms in brain tissue of Rasmussen encephalitis (RE) when compared to people with non-RE epilepsy (PWE) and control cases using whole exome sequencing (WES), RNAseq, and proteomics.

Methods: Frozen brain tissue (ages = 2-19 years) was obtained from control autopsy (n = 14), surgical PWE (n = 10), and surgical RE cases (n = 27). We evaluated WES variants in RE associated with epilepsy, seizures, RE, and human leukocyte antigens (HLAs).

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The anterior nucleus of the thalamus plays a role in the epileptic network.

Ann Clin Transl Neurol

December 2022

Department of Neurology, Comprehensive Epilepsy Center of Beijing, Beijing Key Laboratory of Neuromodulation, Xuanwu Hospital, Capital Medical University, Beijing, China.

Objectives: We investigated both the metabolic differences and interictal/ictal discharges of the anterior nucleus of the thalamus (ANT) in patients with epilepsy to clarify the relationship between the ANT and the epileptic network.

Methods: Nineteen patients with drug-resistant epilepsy who underwent stereoelectroencephalography were studied. Metabolic differences in ANT were analyzed using [18F] fluorodeoxyglucose-positron emission tomography with three-dimensional (3D) visual and quantitative analyses.

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