Atypical absence seizures and gene variants: A gene-based review of etiology, electro-clinical features, and associated epilepsy syndrome
Epilepsy Behav. 2024 Feb;151:109636. doi: 10.1016/j.yebeh.2024.109636. Epub 2024 Jan 16.ABSTRACTAtypical absence seizures are generalized non-convulsive seizures that often occur in children with cognitive impairment. They are common in refractory epilepsy and have been recognized as one of the hallmarks of developmental epileptic encephalopathies. Notably, pathogenic variants associated with AAS, such as GABRG2, GABRG3, SLC6A1, CACNB4, SCN8A, and SYNGAP1, are also linked to developmental epileptic encephalopathies. Atypical absences differ from typical absences in that they are frequently drug-resistant and the prognosis ...
Source: Epilepsy and Behaviour - January 17, 2024 Category: Neurology Authors: Xiaoyu Zhao Zimeng He Yumei Li Xiaofan Yang Baomin Li Source Type: research
Adaptive functioning and neurodevelopment in patients with Dravet syndrome: 12-month interim analysis of the BUTTERFLY observational study
We present the 12-month interim analysis of the study.MATERIALS AND METHODS: Patients with a genetically confirmed diagnosis of DS were enrolled in the study. Adaptive functioning and neurodevelopmental status were measured using the Vineland Adaptive Behavior Scale, Third Edition (Vineland-III), Bayley Scales of Infant Development, Third Edition (BSID-III), and Wechsler Preschool and Primary Scale of Intelligence, Fourth Edition (WPPSI-IV). Executive function, ambulatory function and locomotor activities, and overall clinical status were measured using the Behavior Rating Inventory of Executive Function - Preschool Versio...
Source: Epilepsy and Behaviour - January 14, 2024 Category: Neurology Authors: Joseph Sullivan Elaine Wirrell Kelly G Knupp Dillon Chen Robert Flamini Muhammad Zafar Pam Ventola Javier Avenda ño Fei Wang Kimberly A Parkerson Barry Ticho Source Type: research
Transition in epilepsy - A pilot study with patients in and outside of academic centers
CONCLUSIONS: This study highlights the lack of appropriate transition to adult healthcare system (AHCS) amongst an unselected group of patients with epilepsy in Canada and United States. An overwhelming majority of patients followed in the community and in academy centers were simply "transferred" to an adult health practitioner, or they remained under the care of pediatricians. Finally, most patients lack access to significant social and medical support after moving to the AHCS.PMID:38219605 | DOI:10.1016/j.yebeh.2024.109624 (Source: Epilepsy and Behaviour)
Source: Epilepsy and Behaviour - January 14, 2024 Category: Neurology Authors: Quratulain Zulfiqar Ali Paula Marques Puja Patel Jaime Carrizosa Rima Nabbout Danielle M Andrade Source Type: research
Effects of valproic acid and levetiracetam monotherapy on balance functions in patients with generalized epilepsy
CONCLUSION: Valproic acid and levetiracetam may affect the vestibulocular and vestibulocolic reflex pathways negatively. In this cohort, valproic acid had more pronounced adverse effects on balance functions as compared to levetiracetam.PMID:38219606 | DOI:10.1016/j.yebeh.2024.109622 (Source: Epilepsy and Behaviour)
Source: Epilepsy and Behaviour - January 14, 2024 Category: Neurology Authors: Deniz U ğur Cengiz Sanem Can Çolak Emre Akg ün Özdemir Ahmet Ad ıgüzel Source Type: research
Health care access, psychosocial outcomes and mental health in adults living with epilepsy during the COVID-19 pandemic
CONCLUSION: Our cohort of PLWE experienced some challenges during the COVID-19 pandemic including poorer mental health and financial and employment-related stressors. Encouragingly, healthcare access was relatively spared during the COVID-19 crisis, with some patients even reporting a reduction in seizure burden. However, PLWE require ongoing psychosocial support with particular attention to decompensation of mental health and social stressors that may be exacerbated by the COVID-19 pandemic.PMID:38219607 | DOI:10.1016/j.yebeh.2023.109617 (Source: Epilepsy and Behaviour)
Source: Epilepsy and Behaviour - January 14, 2024 Category: Neurology Authors: L Mc Carthy B Mathew L J Blank G Van Hyfte E Gotlieb J Goldstein P Agarwal C S Kwon A Singh M Fields L Marcuse J Y Yoo E Sivarak K Gururangan A Navis N Jett é Source Type: research
The longitudinal effects of cannabidiol on brain temperature in patients with treatment-resistant epilepsy
Epilepsy Behav. 2024 Feb;151:109606. doi: 10.1016/j.yebeh.2023.109606. Epub 2024 Jan 9.ABSTRACTNeuroinflammation (NI) is a key pathophysiological contributor to treatment-resistant epilepsy (TRE) that remains challenging to observe in vivo. Magnetic resonance spectroscopic imaging and thermometry (MRSI-t) is an emerging technique that can be used to non-invasively map brain temperature, whereby brain temperature elevations serve as a surrogate for the cellular and biochemical processes associated with NI. In a previous multimodal imaging study of focal epilepsy patients, we observed MRSI-t-based brain temperature elevation...
Source: Epilepsy and Behaviour - January 10, 2024 Category: Neurology Authors: Ayushe A Sharma Jerzy P Szaflarski Source Type: research
'Knowledge exchange' workshops to optimise development of a risk prediction tool to assist conveyance decisions for suspected seizures - Part of the Risk of ADverse Outcomes after a Suspected Seizure (RADOSS) project
CONCLUSION: Collaborative stakeholder engagement provided crucial insights that can guide RADOSS to develop a user-aligned, optimized tool.PMID:38199055 | DOI:10.1016/j.yebeh.2023.109611 (Source: Epilepsy and Behaviour)
Source: Epilepsy and Behaviour - January 10, 2024 Category: Neurology Authors: Adam J Noble Beth Morris Laura J Bonnett Markus Reuber Suzanne Mason Jasmine Wright Richard Pilbery Fiona Bell Tom Shillito Anthony G Marson Jon M Dickson Source Type: research
Letter to the Editor: EEG-based seizure detection
Epilepsy Behav. 2024 Feb;151:109614. doi: 10.1016/j.yebeh.2023.109614. Epub 2024 Jan 9.NO ABSTRACTPMID:38199056 | DOI:10.1016/j.yebeh.2023.109614 (Source: Epilepsy and Behaviour)
Source: Epilepsy and Behaviour - January 10, 2024 Category: Neurology Authors: E E M Reus G H Visser F M E Cox Source Type: research
What have we learned from the real-world efficacy of FFA in DS and LGS? A post-marketing study in clinical practice
Epilepsy Behav. 2024 Feb;151:109620. doi: 10.1016/j.yebeh.2024.109620. Epub 2024 Jan 8.ABSTRACTOBJECTIVE: To evaluate the effectiveness and tolerability of fenfluramine (FFA) in routine clinical practice treating real-world populations with Dravet syndrome (DS) and Lennox-Gastaut syndrome (LGS).METHODS: This was a retrospective analysis of patients with DS or LGS who initiated FFA treatment from 2018 to 2022 at a single center. Patient demographics, medical history, seizure characteristics, and treatment outcomes were collected from electronic medical records. Duration of FFA treatment, dosage regimens, seizure frequency, ...
Source: Epilepsy and Behaviour - January 9, 2024 Category: Neurology Authors: Adri án Valls Carbó Álvaro Beltrán Irene S ánchez-Miranda Román Borja Cabal Pablo G ómez-Porro Ángel Aledo-Serrano Gloria L ópez Sobrino Fernando Ayuga Mar ía Gómez Eguilaz Antonio Gil-Nagel Source Type: research
How to handle a missed or delayed dose of lacosamide in pediatric patients with epilepsy? a mode-informed individual dosing
This study aims to investigate the effects on the pharmacokinetic (PK) of lacosamide (LCM), and to guide the individual dosing regimens for children and ones with poor medication adherence. Population PK research was performed based on 164 plasma samples of 113 pediatric patients aged from 1.75 to 14.42 years old. The PK characteristic of LCM was developed by a one-compartment model with first-order elimination. The typical value of apparent clearance (CL) and apparent volume of distribution (Vd) was 1.91 L·h-1 and 56.53 L respectively. In the final model, the variability of CL was significantly associated with the body s...
Source: Epilepsy and Behaviour - January 9, 2024 Category: Neurology Authors: Jun Wang Yan Mei Song Liang Si-Chan Li Chen Chen Gang Nie Ya-Li Tuo Dan Sun Yang Wang Source Type: research
Cross-cultural validation and psychometric properties of the Spanish version of the quality of life in Childhood epilepsy Questionnaire (QOLCE-55)
CONCLUSIONS: The Spanish version of the QOLCE-55 has demonstrated adequate psychometric properties, indicating that it can be confidently used to measure the health-related quality-of-life (HRQoL) in children with epilepsy in a Spanish-speaking population. These results corroborate the instrument's cross-cultural validity.PMID:38194772 | DOI:10.1016/j.yebeh.2023.109619 (Source: Epilepsy and Behaviour)
Source: Epilepsy and Behaviour - January 9, 2024 Category: Neurology Authors: Salvador Iba ñez-Micó Jos é A López-Pina Antonia G ómez-Conesa Source Type: research
Transient anticonvulsant effects of time-restricted feeding in the 6-Hz mouse model
CONCLUSIONS: Both continuous and 2:5 TRF regimens transiently increased seizure thresholds in the 6-Hz model at around 2 weeks, which coincided with stability of blood glucose levels during the fed and fasted periods. Our findings suggest that the lack of prolonged anticonvulsant effects in the acute electrical seizure models employed may be attributed to only modest metabolic and antioxidant adaptations found in the brain and liver. Our findings underscore the potential therapeutic value of TRF in managing seizure-related conditions.PMID:38184948 | DOI:10.1016/j.yebeh.2023.109618 (Source: Epilepsy and Behaviour)
Source: Epilepsy and Behaviour - January 7, 2024 Category: Neurology Authors: Oliver J Armstrong Elliott S Neal Diana Vidovic Weizhi Xu Karin Borges Source Type: research
Cognitive and psychiatric adverse events during adjunctive cenobamate treatment in phase 2 and phase 3 clinical studies
CONCLUSIONS: Cognitive and psychiatric TEAEs were reported by similar numbers of cenobamate- and placebo-treated patients during double-blind adjunctive cenobamate treatment (< 4 % of patients), and exposure-adjusted incidence rates of these TEAEs remained low during open-label cenobamate treatment for up to 7 years. Treatment discontinuations due to these TEAEs were rare. The results of this post-hoc analysis indicate that adjunctive cenobamate treatment exhibits a low incidence of cognitive or psychiatric TEAEs in patients with uncontrolled focal seizures.PMID:38184949 | DOI:10.1016/j.yebeh.2023.109605 (Source: Epilepsy and Behaviour)
Source: Epilepsy and Behaviour - January 7, 2024 Category: Neurology Authors: Gregory L Krauss Steve S Chung Louis Ferrari Sean Stern William E Rosenfeld Source Type: research
Transient anticonvulsant effects of time-restricted feeding in the 6-Hz mouse model
CONCLUSIONS: Both continuous and 2:5 TRF regimens transiently increased seizure thresholds in the 6-Hz model at around 2 weeks, which coincided with stability of blood glucose levels during the fed and fasted periods. Our findings suggest that the lack of prolonged anticonvulsant effects in the acute electrical seizure models employed may be attributed to only modest metabolic and antioxidant adaptations found in the brain and liver. Our findings underscore the potential therapeutic value of TRF in managing seizure-related conditions.PMID:38184948 | DOI:10.1016/j.yebeh.2023.109618 (Source: Epilepsy and Behaviour)
Source: Epilepsy and Behaviour - January 7, 2024 Category: Neurology Authors: Oliver J Armstrong Elliott S Neal Diana Vidovic Weizhi Xu Karin Borges Source Type: research
Cognitive and psychiatric adverse events during adjunctive cenobamate treatment in phase 2 and phase 3 clinical studies
CONCLUSIONS: Cognitive and psychiatric TEAEs were reported by similar numbers of cenobamate- and placebo-treated patients during double-blind adjunctive cenobamate treatment (< 4 % of patients), and exposure-adjusted incidence rates of these TEAEs remained low during open-label cenobamate treatment for up to 7 years. Treatment discontinuations due to these TEAEs were rare. The results of this post-hoc analysis indicate that adjunctive cenobamate treatment exhibits a low incidence of cognitive or psychiatric TEAEs in patients with uncontrolled focal seizures.PMID:38184949 | DOI:10.1016/j.yebeh.2023.109605 (Source: Epilepsy and Behaviour)
Source: Epilepsy and Behaviour - January 7, 2024 Category: Neurology Authors: Gregory L Krauss Steve S Chung Louis Ferrari Sean Stern William E Rosenfeld Source Type: research
