Oligodendrocyte lineage and myelination are compromised in the gray matter of focal cortical dysplasia type IIa

Abstract

Objectives

Focal cortical dysplasias (FCDs) are local malformations of the human neocortex and a leading cause of medically intractable epilepsy. FCDs are characterized by local architectural disturbances of the neocortex and often by a blurred gray‐white matter boundary indicating abnormal white matter myelination. We have recently shown that myelination is also compromised in the gray matter of dysplastic areas, since transcripts encoding factors for oligodendrocyte differentiation and myelination are downregulated and myelin fibers appear fractured and disorganized.

Methods

Here, we characterized the gray ...

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Hippocampal profiling: Localized magnetic resonance imaging volumetry and T2 relaxometry for hippocampal sclerosis

Abstract

Objective

Hippocampal sclerosis (HS) is the most common cause of drug‐resistant temporal lobe epilepsy, and its accurate detection is important to guide epilepsy surgery. Radiological features of HS include hippocampal volume loss and increased T2 signal, which can both be quantified to help improve detection. In this work, we extend these quantitative methods to generate cross‐sectional area and T2 profiles along the hippocampal long axis to improve the localization of hippocampal abnormalities.

Methods

T1‐weighted and T2 relaxometry data from 69 HS patients (32 ...

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Epileptogenicity in tuberous sclerosis complex: A stereoelectroencephalographic study

Abstract

Objective

In tuberous sclerosis complex (TSC)‐associated drug‐resistant epilepsy, the optimal invasive electroencephalographic (EEG) and operative approach remains unclear. We examined the role of stereo‐EEG in TSC and used stereo‐EEG data to investigate tuber and surrounding cortex epileptogenicity.

Methods

We analyzed 18 patients with TSC who underwent stereo‐EEG (seven adults). One hundred ten seizures were analyzed with the epileptogenicity index (EI). In 13 patients with adequate tuber sampling, five anatomical regions of interest (ROIs) were defined: dominant tuber (tuber with highest median EI), perituber ...

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Early detection rate changes from a brain‐responsive neurostimulation system predict efficacy of newly added antiseizure drugs

Abstract

Objective

Brain‐responsive neurostimulation (RNS System, NeuroPace) is used to treat medically refractory focal epilepsy and also provides long‐term ambulatory neurophysiologic data. We sought to determine whether these data could predict the clinical response to antiseizure drugs (ASDs).

Methods

First, newly added medications were identified in RNS System patients followed at a single epilepsy center. Daily detection rates including “episode starts” (predominantly interictal activity) and “long episodes” (often electrographic seizures) were compared before and after ASD initiation. Efficacy was determined from documentation of clinical ...

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Diagnosis of pyridoxine‐dependent epilepsy in an adult presenting with recurrent status epilepticus

Abstract

Pyridoxine‐dependent epilepsy (PDE) is a genetic metabolic disease caused by inborn errors affecting vitamin B6 metabolism, which typically presents with neonatal seizures resistant to antiepileptic drugs (AEDs). Treatment with pyridoxine terminates seizures and prevents neurological decline. We describe a case in which the diagnosis was established at the age of 22 years. Birth and development were normal, but there was a history of three isolated tonic‐clonic seizures during childhood and adolescence. At the age of 18 years, she developed frequent focal motor ...

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External validation and comparison of two prediction models for seizure recurrence after the withdrawal of antiepileptic drugs in adult patients

Abstract

Objective

The models currently available for predicting the risk of seizure recurrence after antiepileptic drug (AED) withdrawal in adult epilepsy patients include the prediction model developed by Lamberink et al (Lamberink model, 2017) and the Medical Research Council prediction model (MRC model, 1993). However, there was no external validation for the two models. The purpose of this study was to perform an independent external validation and a comparison of the Lamberink model and the MRC model in adult patients.

Methods

The study population ...

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Prospective validation study of an epilepsy seizure risk system for outpatient evaluation

Abstract

Objective

We conducted clinical testing of an automated Bayesian machine learning algorithm (Epilepsy Seizure Assessment Tool [EpiSAT]) for outpatient seizure risk assessment using seizure counting data, and validated performance against specialized epilepsy clinician experts.

Methods

We conducted a prospective longitudinal study of EpiSAT performance against 24 specialized clinician experts at three tertiary referral epilepsy centers in the United States. Accuracy, interrater reliability, and intra‐rater reliability of EpiSAT for correctly identifying changes in seizure risk (improvements, worsening, or no change) were evaluated using 120 seizures from four synthetic seizure diaries ...

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Readmission after neurosurgical intervention in epilepsy: A nationwide cohort analysis

Abstract

Objective

Hospital readmissions result in increased health care costs and are associated with worse outcomes after neurosurgical intervention. Understanding factors associated with readmissions will inform future studies aimed at improving quality of care in those with epilepsy.

Methods

Patients of all ages with epilepsy who underwent a neurosurgical intervention were identified in the 2014 Nationwide Readmissions Database, a nationally representative dataset containing data from roughly 17 million US hospital discharges. Diagnosis of epilepsy was based on International Classification of Diseases, Ninth Revision, Clinical ...

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