Disadvantage and Neurocognitive Comorbidities in Childhood Idiopathic Epilepsies

Abstract

Objective

To characterize the relationship between neighborhood disadvantage on cognitive function as well as clinical, sociodemographic, and family factors in children with new onset idiopathic epilepsy and healthy controls.

Methods

Research participants were 288 children aged 8-18 years with recent onset epilepsy (CWE, n = 182; mean age = 12.2 ±3.2 years), healthy first-degree cousin controls (HC, n = 106; mean age = 12.5 ±3.0), and one biological or adopted parent per child (n=279). All participants were administered a comprehensive neuropsychological battery (reasoning, language, memory, executive function, motor function, and academic achievement). Family ...

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The Presence and Severity of Epilepsy Coincide with Reduced GABA and Cortical Excitatory Markers in SSADH Deficiency

Abstract

Objective

Succinic semialdehyde dehydrogenase deficiency (SSADHD) is a rare inherited metabolic disorder caused by a defect of ɣ-aminobutyrate (GABA) catabolism. Despite the resultant hyperGABAergic environment facilitated by the metabolic defect, individuals with this disorder have a paradoxically high prevalence of epilepsy. We aimed to study the characteristics of epilepsy in SSADHD and its concordance with GABA-related metabolites and neurophysiologic markers of cortical excitation.

Methods

Subjects in an international natural history study of SSADHD underwent clinical assessments, electroencephalography, transcranial magnetic stimulation (TMS), magnetic resonance ...

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Treatment of NORSE/FIRES with Tocilizumab in a child and a young adult

Abstract

New Onset Refractory Status Epilepticus (NORSE) is a rare and devastating condition occurring in a previously healthy patient. It is called Febrile Infection Related Epilepsy Syndrome (FIRES) when preceded by a febrile infection. It often leads to intensive care treatment, including antiseizure drugs in combination with anaesthetic agents, and sometimes ketogenic diet. The mortality rate is high, and severe epileptic and neuropsychiatric sequelae are usually observed.

Based on the possible role of neuroinflammation, intravenous immunoglobulin, corticosteroids and immunomodulatory treatment (anti-IL1, IL6) ...

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A pharmacokinetic model of antiseizure medication load to guide care in the epilepsy monitoring unit

Abstract

Objective

Evaluating patients with drug-resistant epilepsy often requires inducing seizures by tapering antiseizure medications (ASMs) in the epilepsy monitoring unit (EMU). The relationship between ASM taper strategy, seizure timing, and severity remains unclear. In this study, we developed and validated a pharmacokinetic model of total ASM load and tested its association with seizure occurrence and severity in the EMU.

Methods

We studied 80 patients who underwent intracranial electroencephalographic recording for epilepsy surgery planning. We developed a first order pharmacokinetic model of the ASMs ...

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Comparing electrical stimulation functional mapping with subdural electrodes and stereoelectroencephalography

Abstract

Objective

Electrical stimulation mapping (ESM) is the clinical standard for functional localization with subdural electrodes (SDE). As stereoelectroencephalography (SEEG) has emerged as an alternative option, we compared functional responses, afterdischarges (ADs), and unwanted ESM-induced seizures (EISs) between the two electrode types.

Methods

Incidence and current thresholds for functional responses (sensory, motor, speech/language), ADs, and EISs were compared between SDE and SEEG using mixed models incorporating relevant covariates.

Results

We identified 67 SEEG ESM and 106 SDE ESM patients (7207 and 4980 stimulated contacts, respectively). We ...

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A Comparison of Statistical versus Clinical Classifiers of Seizure Clustering in Women with Catamenial and Non‐Catamenial Epilepsy

Abstract

Objective

We assessed whether 1) women with statistical clustering of daily seizure counts (DSC) or seizure intervals (SI) also showed clinical clustering, defined separately by ≥2 (≥2-SC) and ≥3 (≥3-SC) seizures on any single day, and 2) how these classifiers might apply to catamenial epilepsy.

Methods

This is a retrospective case-control analysis of data from 50 women with epilepsy (WWE). We assessed the relationships of the 4 classifiers to each other and to catamenial versus non-catamenial epilepsy using chi-square, correlation, logistic regression and ...

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Scalp Ripple Rates for Rapid Epilepsy Differentiation and Seizure Activity Assessment: Applicability and Influential Factors

Abstract

Objective

We aim to determine whether automatically detected ripple rate (ADRR) of ten-minute scalp electroencephalography (EEG) during slow-wave sleep can be a useful tool for rapid epilepsy differentiation and seizure activity assessment, and analyze the clinical factors that may affect it.

Methods

We retrospectively included 336 patients who underwent long-term video EEG with a sampling rate ≥1000 Hz, and three groups were established based on their final clinical diagnosis (non-epilepsy; non-active epilepsy [epilepsy being seizure free for at least one year]; and active ...

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Reasons for ineligibility for clinical trials of patients with medication‐resistant epilepsy

Abstract

Selection criteria for clinical trials for medication-resistant epilepsy are used to limit variability and to ensure safety. However, it has become more challenging to recruit subjects for trials. This study investigated the impact of each inclusion and exclusion criterion on medication-resistant epilepsy clinical trial recruitment at a large academic epilepsy center. We retrospectively identified all patients with medication-resistant focal or generalized onset epilepsy who attended an outpatient clinic over a consecutive 3-month period. We assessed each patient’s eligibility for trials ...

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Primidone improves symptoms in TRPM3‐linked DEE‐SWAS

Abstract

Developmental and epileptic encephalopathy (DEE) with continuous spike-and-wave activation in sleep (CSWS) or DEE-SWAS is an age-dependent disease, often accompanied by a decline in cognitive abilities. Early successful treatment of CSWS is associated with a better cognitive outcome. We retrospectively analyzed the clinical, electrophysiological, radiological, and genetic data of children with DEE-SWAS associated with melastatin-related transient receptor type 3 gene (TRPM3) missense variants.

We report two unrelated children with pharmaco-resistant DEE-SWAS and developmental delay/regression and different heterozygous de novo missense variants ...

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Prognostic interictal electroencephalographic biomarkers and models to assess antiseizure medication efficacy for clinical practice: A scoping review

Abstract

Antiseizure medication (ASM) is the primary treatment for epilepsy. In clinical practice, methods to assess ASM efficacy (predict seizure freedom or seizure reduction), during any phase of the drug treatment lifecycle, are limited. This scoping review identifies and appraises prognostic electroencephalographic (EEG) biomarkers and prognostic models that use EEG features, which are associated with seizure outcomes following ASM initiation, dose adjustment, or withdrawal. We also aim to summarize the population and context in which these biomarkers and models were identified ...

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