Lennart Walger, Sophie Adler, Konrad Wagstyl, Leonie Henschel, Bastian David, Valeri Borger, Elke Hattingen, Hartmut Vatter, Christian E. Elger, Torsten Baldeweg, Felix Rosenow, Horst Urbach, Albert Becker, Alexander Radbruch, Rainer Surges, Martin Reuter, Fernando Cendes, Zhong Irene Wang, Hans-Jürgen Huppertz, Theodor Rüber
doi : 10.1111/epi.17522
Focal cortical dysplasias (FCDs) are malformations of cortical development and one of the most common pathologies causing pharmacoresistant focal epilepsy. Resective neurosurgery yields high success rates, especially if the full extent of the lesion is correctly identified and completely removed.
Thahesh Tharmaraja, Jamie Sin Ying Ho, Aidan Neligan, Sanjeev Rajakulendran
doi : 10.1111/epi.17523
New-onset refractory status epilepticus (NORSE) is a devastating neurological presentation. There is a paucity of large studies on NORSE as it is a relatively new clinical syndrome. The aim of this review was to summarize the etiologies and establish a mortality rate for NORSE.
Ashley Reynolds, Michaela Vranic-Peters, Alan Lai, David B. Grayden, Mark J. Cook, Andre Peterson
doi : 10.1111/epi.17548
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.
Giampaolo Milior, Mélanie Morin-Brureau, Johan Pallud, Richard Miles, Gilles Huberfeld
doi : 10.1111/epi.17552
Animal models of human brain disorders permit researchers to explore disease mechanisms and to test potential therapies. However, therapeutic molecules derived from animal models often translate poorly to the clinic.
Daniel M. Goldenholz
doi : 10.1111/epi.17559
Christian M. Boßelmann, Costin Leu, Dennis Lal
doi : 10.1111/epi.17570
Kathryn M. Snyder, Kiefer J. Forseth, Cristian Donos, Patrick S. Rollo, Simon Fischer-Baum, Joshua Breier, Nitin Tandon
doi : 10.1111/epi.17555
Lexical retrieval deficits are characteristic of a variety of different neurological disorders. However, the exact substrates responsible for this are not known. We studied a large cohort of patients undergoing surgery in the dominant temporal lobe for medically intractable epilepsy (n = 95) to localize brain regions that were associated with anomia.
Georgia Ramantani, Christine Bulteau, Dorottya Cserpan, Willem M. Otte, Georg Dorfmüller, J. Helen Cross, Josef Zentner, Martin Tisdall, Kees P. J. Braun
doi : 10.1111/epi.17574
We aimed to assess determinants of seizure outcome following pediatric hemispherotomy in a contemporary cohort.
Javier Peña-Ceballos, Patrick B. Moloney, Tudor Munteanu, Michael Doyle, Niamh Colleran, Brenda Liggan, Annette Breen, Sinead Murphy, Hany El-Naggar, Peter Widdess-Walsh, Norman Delanty
doi : 10.1111/epi.17549
Recent clinical trials have shown that cenobamate substantially improves seizure control in focal-onset drug-resistant epilepsy (DRE). However, little is known about cenobamate's performance in highly active (≥20 seizures/month) and ultra-refractory focal epilepsy (≥6 failed epilepsy treatments, including antiseizure medications [ASMs], epilepsy surgery, and vagus nerve stimulation). Here, we studied cenobamate's efficacy and tolerability in a “real-world� severe DRE cohort.
Nina J. Ghosn, Kevin Xie, Akash R. Pattnaik, James J. Gugger, Colin A. Ellis, Elizabeth Sweeney, Emily Fox, John M. Bernabei, Jenaye Johnson, Jacqueline Boccanfuso, Brian Litt, Erin C. Conrad
doi : 10.1111/epi.17558
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.
Haris Hakeem, Bshra Ali A. Alsfouk, Patrick Kwan, Martin J. Brodie, Zhibin Chen
doi : 10.1111/epi.17573
To assess the temporal trends in the use of second antiseizure (ASM) regimens and compare the efficacy of substitution monotherapy and combination therapy after failure of initial monotherapy in people with epilepsy.
Sophie Diederich, Ulrich Hemmeter, Maren Paulmann, Maja Mockenhaupt
doi : 10.1111/epi.17563
This study was undertaken to determine the impact of dosage in new users of lamotrigine (LTG) and the concomitant intake of valproic acid (VPA) on epidermal necrolysis (EN).
Elaine W. Pang, Nicholas D. Lawn, Judy Lee, John W. Dunne
doi : 10.1111/epi.17567
Although increased mortality associated with epilepsy is well understood, data in patients after their first-ever seizure are limited. We aimed to assess mortality after a first-ever unprovoked seizure and identify causes of death (CODs) and risk factors.
Gian Marco Duma, Alberto Danieli, Giovanni Mento, Valerio Vitale, Raffaella Scotto Opipari, Viktor Jirsa, Paolo Bonanni, Pierpaolo Sorrentino
doi : 10.1111/epi.17551
Large aperiodic bursts of activations named neuronal avalanches have been used to characterize whole-brain activity, as their presence typically relates to optimal dynamics. Epilepsy is characterized by alterations in large-scale brain network dynamics.
Ayushe A. Sharma, Rodolphe Nenert, Adam Goodman, Jerzy P. Szaflarski
doi : 10.1111/epi.17538
Neuroinflammation (NI) is a pathophysiological factor in many neurological disorders, including epilepsy. Because NI causes microstructural tissue damage that worsens with increasing brain temperature, abnormally elevated brain temperatures may be a surrogate measure of the biochemical consequences of NI.
Alfredo Lucas, Eli J. Cornblath, Nishant Sinha, Peter Hadar, Lorenzo Caciagli, Simon S. Keller, Leonardo Bonilha, Russell T. Shinohara, Joel M. Stein, Sandhitsu Das, Ezequiel Gleichgerrcht, Kathryn A. Davis
doi : 10.1111/epi.17565
Temporal lobe epilepsy (TLE) is the most common type of focal epilepsy. An increasingly identified subset of patients with TLE consists of those who show bilaterally independent temporal lobe seizures. The purpose of this study was to leverage network neuroscience to better understand the interictal whole brain network of bilateral TLE (BiTLE).
Jérôme Clatot, Shridhar Parthasarathy, Stacey Cohen, Jillian L. McKee, Shavonne Massey, Ala Somarowthu, Ethan M. Goldberg, Ingo Helbig
doi : 10.1111/epi.17444
Loss-of-function variants in SCN1A cause Dravet syndrome, the most common genetic developmental and epileptic encephalopathy (DEE). However, emerging evidence suggests separate entities of SCN1A-related disorders due to gain-of-function variants. Here, we aim to refine the clinical, genetic, and functional electrophysiological features of a recurrent p.R1636Q gain-of-function variant, identified in four individuals at a single center.
Sara Matricardi, Sandrine Cestèle, Marina Trivisano, Benedetta Kassabian, Nathalie Leroudier, Roberta Vittorini, Margherita Nosadini, Elisabetta Cesaroni, Sabrina Siliquini, Cristina Marinaccio, Francesca Longaretti, Barbara Podestà, Francesca Felicia Operto, Concetta Luisi, Stefano Sartori, Clementina Boniver, Nicola Specchio, Federico Vigevano, Carla Marini, Massimo Mantegazza
doi : 10.1111/epi.17509
This study was undertaken to refine the spectrum of SCN1A epileptic disorders other than Dravet syndrome (DS) and genetic epilepsy with febrile seizures plus (GEFS+) and optimize antiseizure management by correlating phenotype–genotype relationship and functional consequences of SCN1A variants in a cohort of patients.
Ingrid E. Scheffer
doi : 10.1111/epi.17562
Karen L. Oliver, Marina Trivisano, Simone A. Mandelstam, Angela De Dominicis, David I. Francis, Timothy E. Green, Alison M. Muir, Apoorva Chowdhary, Christoph Hertzberg, Klaus Goldhahn, Julia Metreau, Christine Prager, Jason Pinner, Michael Cardamone, Kenneth A. Myers, Richard J. Leventer, Gaetan Lesca, Melanie Bahlo, Michael S. Hildebrand, Heather C. Mefford, Angela M. Kaindl, Nicola Specchio, Ingrid E. Scheffer
doi : 10.1111/epi.17542
WWOX is an autosomal recessive cause of early infantile developmental and epileptic encephalopathy (WWOX-DEE), also known as WOREE (WWOX-related epileptic encephalopathy). We analyzed the epileptology and imaging features of WWOX-DEE, and investigated genotype–phenotype correlations, particularly with regard to survival.
Karen L. Oliver, Ingrid E. Scheffer, Mark F. Bennett, Bronwyn E. Grinton, Melanie Bahlo, Samuel F. Berkovic
doi : 10.1111/epi.17547
“How many epilepsy genes are there?� is a frequently asked question. We sought to (1) provide a curated list of genes that cause monogenic epilepsies, and (2) compare and contrast epilepsy gene panels from multiple sources.
Anna Maria Costa, Mohammad Gol, Chiara Lucchi, Giuseppe Biagini
doi : 10.1111/epi.17561
Epileptogenesis after status epilepticus (SE) has a faster onset in rats treated to reduce brain levels of the anticonvulsant neurosteroid allopregnanolone with the 5α-reductase inhibitor finasteride; however, it still has to be evaluated whether treatments aimed at increasing allopregnanolone levels could result in the opposite effect of delaying epileptogenesis.
Marek Pawlik, Anna Maria Czarnecka, Marcin KoÅ‚odziej, Katarzyna SkowroÅ„ska, MichaÅ‚ WÄ™grzynowicz, Martyna Podgajna, StanisÅ‚aw Jerzy Czuczwar, Jan Albrecht
doi : 10.1111/epi.17554
Initiation and development of early seizures by chemical stimuli is associated with brain cell swelling resulting in edema of seizure-vulnerable brain regions. We previously reported that pretreatment with a nonconvulsive dose of glutamine (Gln) synthetase inhibitor methionine sulfoximine (MSO) mitigates the intensity of initial pilocarpine (Pilo)-induced seizures in juvenile rats.
Andrea Cabassa Miskimen, Lilian L. Cohen, Elissa G. Yozawitz, Zachary M. Grinspan
doi : 10.1111/epi.17544
Shennan Aibel Weiss, Dawn Eliashiv, John Stern, Daniel Rubinstein, Itzhak Fried, Chengyuan Wu, Ashwini Sharan, Jerome Engel, Richard Staba, Michael R. Sperling
doi : 10.1111/epi.17582
How responsive neurostimulation (RNS) decreases seizure frequency is unclear. Stimulation may alter epileptic networks during inter-ictal epochs. Definitions of the epileptic network vary but fast ripples (FRs) may be an important substrate.
Wesley T. Kerr, Hai Chen, Mariana Figuera Losada, Christopher Cheng, Tiffany Liu, Jaqueline French
doi : 10.1111/epi.17568
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.
Lena-Luise Becker, Denise Horn, Felix Boschann, Evelien Van Hoeymissen, Thomas Voets, Joris Vriens, Christine Prager, Angela M. Kaindl
doi : 10.1111/epi.17586
Developmental and epileptic encephalopathy 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.
Magnhild Kverneland, Karl Otto Nakken, Dag Hofoss, Annette Holth Skogan, Per Ole Iversen, Kaja Kristine Selmer, Morten Ingvar Lossius
doi : 10.1111/epi.17585
Ketogenic diet, a high-fat, low-carbohydrate diet, is an established treatment for patients with severe epilepsy. We have previously reported a moderate reduction in seizure frequency after treatment with a modified Atkins diet.
Nebras M. Warsi, Simeon M. Wong, Carolina Gorodetsky, Hrishikesh Suresh, Olivia N. Arski, Mark Ebden, Elizabeth N. Kerr, Mary Lou Smith, Ivanna Yau, Ayako Ochi, Hiroshi Otsubo, Rohit Sharma, Puneet Jain, Shelly Weiss, Elizabeth J. Donner, O. Carter Snead, George M. Ibrahim
doi : 10.1111/epi.17556
Children with epilepsy commonly have comorbid neurocognitive impairments that severely affect their psychosocial well-being, education, and future career prospects. Although the provenance of these deficits is multifactorial, the effects of interictal epileptiform discharges (IEDs) and anti-seizure medications (ASMs) are thought to be particularly severe.
Remi Stevelink, Bobby P. C. Koeleman, Sanjay M. Sisodiya, International League Against Epilepsy Consortium on Complex Epilepsies
doi : 10.1111/epi.17529
Focal and generalized epilepsies are associated with robust differences in magnetic resonance imaging (MRI) measures of subcortical structures, gray matter, and white matter.
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