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Favorable response to ketogenic diet therapy in a patient with -related epilepsy.
Epilepsy Behav Rep
March 2025
Section of Pediatric Neurology, Department of Pediatrics, The University of Chicago, Chicago, IL, United States.
Dynein Cytoplasmic 1 Heavy chain 1 (-related disorders are a spectrum of conditions including neurodevelopmental disorders, congenital brain malformations, and neuromuscular diseases. These clinical features may co-occur, with four main disease entities including epilepsy with developmental epileptic encephalopathy such as infantile epileptic spasms syndrome (IESS) and Lennox-Gastaut syndrome (LGS), axonal Charcot-Marie-Tooth disease type 2O, spinal muscular atrophy with lower extremity-predominance (SMALED), and congenital cortical malformations. Epilepsy associated with this disorder often becomes drug-resistant and requires multiple medications and, in some cases, non-pharmacological treatments.
View Article and Find Full Text PDFOutcomes following resective and disconnective strategies in the treatment of epileptic spasms: a systematic review of the literature and individual patient data meta-analysis.
Front Neurol
December 2024
Brain and Development Research Axis, Azrieli CHU Ste-Justine Research Center, Montreal, QC, Canada.
Epileptic spasms (ES) are a unique seizure type typically presenting in the form of infantile epileptic spasms syndrome (IESS) with characteristic hypsarrhythmia on scalp EEG and a preponderance with developmental delay or regression. While pharmacotherapy is the mainstay of treatment, surgical options, including disconnective or resective procedures, are increasingly recognized as viable therapeutic options for recurrent or persistent ES. However, limited data on safety, effectiveness, and prognostic factors hinder informed decision-making regarding surgery indications, timing, and intervention type.
View Article and Find Full Text PDFEtiology-specific subgroup analysis of initial pharmacotherapy in infantile epileptic spasm syndrome: A single-center cohort study.
Eur J Paediatr Neurol
January 2025
Division of Pediatric Neurology, Department of Pediatrics, Faculty of Medicine, Ege University, İzmir, Turkey. Electronic address:
Aim: To evaluate the efficacy of initial pharmacotherapy for infantile epileptic spasm syndrome (IESS) with electro-clinical outcome characteristics.
Method: A retrospective comparative cohort study with 280 IESS patients was designed; I. vigabatrin monotherapy (n = 129, 46 %); II.
Home video review in a pediatric clinical neurophysiology led multidisciplinary team meeting.
Epileptic Disord
December 2024
Department of Clinical Neurophysiology, King's College Hospital NHS Foundation Trust, London, UK.
Objective: Assessment of the value of review of home videos by a pediatric multidisciplinary team (MDT) in a pediatric neurophysiology department.
Methods: We describe and evaluate the review of home videos alongside clinical history and previous investigations from patients referred to the Evelina pediatric EEG department at a twice-monthly MDT meeting between 01/2021 and 09/2022. We retrospectively analyzed measures of video quality, quantity and duration, time taken from referral to MDT meeting, pre-MDT and post-MDT meeting proposed diagnosis and clinical outcomes.
Infantile epileptic spasms syndrome: When spasms come out of the blue.
Epilepsy Behav
December 2024
Child Neurology, Epilepsy and Movement Disorders, Bambino Gesù, IRCCS Children's Hospital, Full Member of European Reference Network EpiCARE, Rome, Italy.
Background: This study evaluates the electroclinical features of infantile epileptic spasms syndrome (IESS) suddenly appearing in previously normal patients, aiming to describe clinical outcomes and independent predictors.
Method: We retrospectively selected a homogeneous group of patients with IESS from two Italian centers. All patients had normal development prior to IESS onset and a follow-up period lasting at least one year.
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