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Objective: Lennox-Gastaut Syndrome (LGS) is a severe, often treatment-resistant epilepsy syndrome typically diagnosed in early childhood. Many have seizures before diagnosis. Some have periods of seizure freedom before treatment resistance, i.
View Article and Find Full Text PDFEpilepsia
December 2024
Department of Pediatric Neurology, University Medical Center Utrecht Brain Center, Utrecht, The Netherlands.
Objective: Previous retrospective studies have reported vigabatrin-associated brain abnormalities on magnetic resonance imaging (VABAM), although clinical impact is unknown. We evaluated the association between vigabatrin and predefined brain magnetic resonance imaging (MRI) changes in a large homogenous tuberous sclerosis complex (TSC) cohort and assessed to what extent VABAM-related symptoms were reported in TSC infants.
Methods: The Dutch TSC Registry and the EPISTOP cohort provided retrospective and prospective data from 80 TSC patients treated with vigabatrin (VGB) before the age of 2 years and 23 TSC patients without VGB.
Epilepsia Open
November 2024
Division of Pediatric Neurology, University of California, Los Angeles, California, USA.
Vigabatrin-associated brain abnormalities on MRI (VABAM) are observed in approximately 20% of children who receive vigabatrin for treatment of infantile epileptic spasms syndrome. Although usually reversible and asymptomatic, VABAM is occasionally symptomatic. Whereas asymptomatic VABAM appears to be dose-dependent, symptomatic VABAM is possibly associated with co-administration of vigabatrin and hormonal therapy (i.
View Article and Find Full Text PDFFront Neurol
October 2024
The Division of Neurology, Department of Pediatrics, McMaster Children's Hospital, Hamilton, ON, Canada.
Purpose: Infantile epileptic spasms syndrome (IESS) is associated with abnormal neuronal networks during a critical period of synaptogenesis and brain plasticity. Hypsarrhythmia is a visual EEG biomarker used to diagnose IESS, assess response to treatment, and monitor relapse. Computational EEG biomarkers hold promise in providing unbiased, reliable, and objective criteria for clinical management.
View Article and Find Full Text PDFEpilepsy Behav
November 2024
Department of Neurology, Division of Neurocritical Care, University of Florida College of Medicine, Gainesville, FL 32611, USA.
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