Article
Clinical Neurology
Dong Ah Lee, Joonwon Lee, Hyung Chan Kim, Kang Min Park, Sung Eun Kim
Summary: This study evaluates the differences in hippocampal structural volumes and intra-hippocampal networks between patients with status epilepticus and healthy controls. Significant alterations were found in structural volumes and intra-hippocampal structural covariance networks in patients with status epilepticus compared to healthy controls, despite the absence of evident hippocampal atrophy on visual analysis.
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY
(2022)
Article
Clinical Neurology
Michelle L. Kloc, Jennifer M. Daglian, Gregory L. Holmes, Tallie Z. Baram, Jeremy M. Barry
Summary: The study found that although recurrent febrile seizures did not lead to spatial cognitive deficits in the active avoidance task, they did alter the brain structure and resulted in long-lasting changes in the temporal organization of the hippocampus.
Article
Clinical Neurology
Kevin D. Chen, Alicia M. Hall, Megan M. Garcia-Curran, Gissell A. Sanchez, Jennifer Daglian, Renhao Luo, Tallie Z. Baram
Summary: The study successfully induced eFSE in mice and found that eFSE increased susceptibility to the epileptic agent KA in adult mice, leading to shortened latency to seizure onset and increased severity. 16.5% of the mice developed epilepsy after experiencing eFSE, suggesting that eFSE promotes pro-epileptogenic network changes in some mice, resembling temporal lobe epilepsy.
Article
Chemistry, Medicinal
Summya Rashid, Adil Farooq Wali, Shahzada Mudasir Rashid, Rana M. Alsaffar, Ajaz Ahmad, Basit L. Jan, Bilal Ahmad Paray, Saeed M. A. Alqahtani, Azher Arafah, Muneeb U. Rehman
Summary: The study reveals that zingerone has neuroprotective effects by inhibiting cognitive impairment, neural damage, and inflammation induced by seizures.
Article
Behavioral Sciences
Rita Asdikian, Helene Hajjar, Sana Alturk, Jean-Pierre Asdikian, Fatima Jaafar, Houssein Salah, Yara Mrad, Farah Issa, Michel Almardini, Fatima Nourelddine, Ronza Abdel Rassoul, Houssam Al-Koussa, Makram Obeid
Summary: The study found that non-convulsive status epilepticus in periadolescent rats can lead to seizure burden dependent hippocampal injury and cognitive deficits, emphasizing the importance of prompt diagnosis and treatment of NCSE.
EPILEPSY & BEHAVIOR
(2021)
Article
Biology
Pablo M. Casillas-Espinosa, Alison Anderson, Anna Harutyunyan, Crystal Li, Jiyoon Lee, Emma L. Braine, Rhys D. Brady, Mujun Sun, Cheng Huang, Christopher K. Barlow, Anup D. Shah, Ralf B. Schittenhelm, Richelle Mychasiuk, Nigel C. Jones, Sandy R. Shultz, Terence J. O'Brien
Summary: This study found that sodium selenate treatment provides a sustained disease-modifying effect in chronically epileptic rats in the post-status epilepticus (SE) model of drug-resistant temporal lobe epilepsy (TLE). The treatment reduces the number of spontaneous seizures, cognitive dysfunction, and sensorimotor deficits. It is also associated with increased protein phosphatase 2A (PP2A) expression, reduced hyperphosphorylated tau, and reversed telomere length shortening.
Article
Neurosciences
Michelle L. Kloc, Yuncai Chen, Jennifer M. Daglian, Gregory L. Holmes, Tallie Z. Baram, Jeremy M. Barry
Summary: This study aimed to investigate the effects of febrile status epilepticus (FSE) on neural circuitry governing learning and memory. The results show that FSE leads to physiological changes in the hippocampal circuit and cognitive impairment. Specifically, FSE induces decoupling of cortical synaptic pathways and altered signal phase coherence, and increased DG synaptic activity is predictive of poor cognitive outcomes. These alterations interfere with the ability of hippocampal dendrites to receive, decode, and propagate neocortical inputs, potentially contributing to FSE cognitive comorbidities.
Review
Clinical Neurology
Claudine Sculier, Nicolas Gaspard
Summary: This review summarizes the recent developments in the pathophysiology, diagnosis, and management of new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES). NORSE and FIRES can have various causes, with autoimmune encephalitis and genetic disorders being the most common. However, the majority of cases remain cryptogenic, and recent studies suggest that autoinflammation plays a key role in these conditions. Immunomodulatory treatment is supported by these findings. Consensus recommendations for the management of NORSE and FIRES have also been published recently.
CURRENT OPINION IN NEUROLOGY
(2023)
Article
Clinical Neurology
Robert Daniel Nass, Julia Taube, Tobias Bauer, Theodor Ruber, Rainer Surges, Christoph Helmstaedter
Summary: The study conducted a long-term follow-up of epilepsy patients, finding that although some patients showed improvement after discharge from the intensive care unit, the severity of the disease remained high during follow-up. Most patients experienced varying degrees of impairment in functional outcomes, leading to a decline in independence and cognitive function.
EUROPEAN JOURNAL OF NEUROLOGY
(2021)
Article
Biochemistry & Molecular Biology
Sereen Sandouka, Aseel Saadi, Prince Kumar Singh, Rhoda Olowe, Tawfeeq Shekh-Ahmad
Summary: Nrf2 activation plays a crucial role in protecting against oxidative stress-induced damage in temporal lobe epilepsy. The activation of Nrf2 is time-controlled, region-specific, and cell-type dependent. Targeted activation of the Nrf2 pathway is necessary to modify the development of epilepsy.
CELL AND BIOSCIENCE
(2023)
Article
Immunology
Sree Lalitha Bojja, Bikash Medhi, Shashi Anand, Alka Bhatia, Rupa Joshi, Ranjana W. Minz
Summary: The study found that metformin can alleviate seizure-induced inflammation and neuronal degeneration, potentially mediated by the mTOR pathway.
INFLAMMOPHARMACOLOGY
(2021)
Article
Clinical Neurology
Danielle deCampo, Julie Xian, Alexis Karlin, Katie R. Sullivan, Sarah M. Ruggiero, Peter Galer, Mark Ramos, Nicholas S. Abend, Alex Gonzalez, Ingo Helbig
Summary: In this study, the genetic testing strategies and clinical characteristics of individuals with FIRES were reviewed. The results showed that the etiology of FIRES is still unknown and current treatments are often ineffective. Further studies are needed to develop novel diagnostic and treatment approaches.
FRONTIERS IN NEUROLOGY
(2023)
Review
Clinical Neurology
Zubeda Sheikh, Lawrence J. Hirsch
Summary: New-onset refractory status epilepticus (NORSE) is a clinical presentation characterized by refractory status epilepticus without a clear cause in a patient without active epilepsy or relevant neurological disorder. Febrile infection related epilepsy syndrome (FIRES) is a subcategory of NORSE, which requires a prior febrile infection. Extensive testing may reveal the etiology in some patients, while others remain unexplained.
FRONTIERS IN NEUROLOGY
(2023)
Article
Clinical Neurology
Ronny Wickstrom, Olga Taraschenko, Robertino Dilena, Eric T. Payne, Nicola Specchio, Rima Nabbout, Sookyong Koh, Nicolas Gaspard, Lawrence J. Hirsch
Summary: This study aims to develop consensus-based recommendations for the management of adult and pediatric patients with new onset refractory status epilepticus (NORSE)/febrile infection-related epilepsy syndrome (FIRES) based on best evidence and experience. Through the Delphi methodology, 85 recommendation statements were generated and divided into five sections: disease characteristics, diagnostic testing and sampling, acute treatment, treatment in the postacute phase, and research, registries, and future directions in NORSE/FIRES. The analysis offers insight into supporting evidence, current gaps in the literature, and provides guidance for diagnosis, evaluation, and management of patients with these conditions.
Review
Pediatrics
Piero Pavone, Giovanni Corsello, Umberto Raucci, Riccardo Lubrano, Enrico Parano, Martino Ruggieri, Filippo Greco, Silvia Marino, Raffaele Falsaperla
Summary: FIRES is a rare but severe disorder characterized by refractory status epilepticus following a febrile infection, with persistent seizures and cognitive impairment. Its pathogenesis may involve inflammation or autoimmune mechanisms, and various treatment approaches have been proposed. Prognosis is generally poor.
ITALIAN JOURNAL OF PEDIATRICS
(2022)
Article
Clinical Neurology
Mathieu Milh, Pierre Roubertoux, Najoua Biba, Julie Chavany, Adeline Spiga Ghata, Camille Fulachier, Stephan Christopher Collins, Christel Wagner, Jean-Christophe Roux, Binnaz Yalcin, Marie-Solenne Felix, Florence Molinari, Pierre-Pascal Lenck-Santini, Laurent Villard
Article
Neurosciences
Amanda E. Hernan, J. Matthew Mahoney, Willie Curry, Seamus Mawe, Rod C. Scott
Summary: The study reveals that neurons in the hippocampus and prefrontal cortex form a functional network during spatial working memory tasks and are well modulated in time. However, animals with cortical malformation show poorly modulated neurons in time, leading to difficulties in encoding task parameters and integrating into a functional network.
Article
Clinical Neurology
Rod C. Scott
Summary: Treating epilepsy remains challenging for patients who do not achieve seizure freedom, despite the introduction of new drugs over the past decades. Viewing the brain as a complex adaptive system and exploring new therapeutic approaches could open up opportunities to search for novel therapies. By embracing these concepts and applying them to different levels of brain complexity, the epilepsy community may potentially reap significant rewards.
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY
(2021)
Editorial Material
Pediatrics
Suresh Pujar, Rod C. Scott
ARCHIVES OF DISEASE IN CHILDHOOD
(2021)
Article
Clinical Neurology
Pierre-Pascal Lenck-Santini
Summary: The precise coordination of neuronal activity is crucial for optimal brain function, and failure in this coordination could lead to seizures and cognitive deficits in epilepsy. Restoring the dynamic coordination of epileptic networks may improve both seizure and cognitive outcomes.
Article
Clinical Neurology
Aswin Chari
Summary: SEEG-guided surgery in children with epilepsy can achieve high rates of seizure freedom. Factors such as non-motor seizures and the number of seizures recorded during SEEG are crucial in identifying a putative SOZ. The indication for SEEG also plays a significant role in postoperative outcomes.
Article
Biochemistry & Molecular Biology
Enora Moutin, Sophie Sakkaki, Vincent Compan, Nathalie Bouquier, Federica Giona, Julie Areias, Elise Goyet, Anne-Laure Hemonnot-Girard, Vincent Seube, Bastien Glasson, Nathan Benac, Yan Chastagnier, Fabrice Raynaud, Etienne Audinat, Laurent Groc, Tangui Maurice, Carlo Sala, Chiara Verpelli, Julie Perroy
Summary: Mutations in the Shank3 gene lead to autism spectrum disorders through disrupting the integrity of the glutamate receptosome, affecting synaptic plasticity and behaviors. Fine-tuning protein-protein interactions within the glutamate receptosome controlled by changes in neuronal activity play a crucial role in regulating plasticity. Restoring the integrity of the glutamate receptosome and its sensitivity to neuronal activity could rescue synaptic transmission, plasticity, and behaviors associated with Shank3 mutations.
MOLECULAR PSYCHIATRY
(2021)
Article
Neurosciences
Najoua Biba-Maazou, Helene Becq, Emilie Pallesi-Pocachard, Stefania Sarno, Samuel Granjeaud, Aurelie Montheil, Marie Kurz, Laurent Villard, Mathieu Milh, Pierre-Pascal Lenck Santini, Laurent Aniksztejn
Summary: Mutations in the KCNQ2 gene can cause developmental and epileptic encephalopathy, affecting the electrophysiological properties of brain cells at different developmental stages, but the effects vary during the course of development.
JOURNAL OF PHYSIOLOGY-LONDON
(2022)
Review
Clinical Neurology
Mohamed R. Khalife, Rod C. Scott, Amanda E. Hernan
Summary: This article explores the role of seizures in causing cognitive impairments and presents an alternative hypothesis about these deficits. The article focuses on the mechanisms of information processing underlying healthy cognition and explains how the underlying etiology of epilepsy alters neural networks and potential treatments.
FRONTIERS IN NEUROLOGY
(2022)
Article
Biology
Aswin Chari, Kiran K. Seunarine, Xiaosong He, Martin M. Tisdall, Christopher A. Clark, Dani S. Bassett, Rod C. Scott, Richard E. Rosch
Summary: Network control theory is used to model the neurophysiological dynamics of the brain based on the structural connectome constructed from diffusion MRI. This study explores the changes in average and modal controllability in children with drug-resistant epilepsy compared to healthy controls. The results suggest that increased thalamocortical connectivity may be responsible for the observed changes in controllability. The findings also indicate that controllability changes can be used as a manifestation of brain network dysfunction in epilepsy and may help in understanding the pathophysiology of the disease.
COMMUNICATIONS BIOLOGY
(2022)
Article
Clinical Neurology
Andrew T. Hale, Aswin Chari, Rod C. Scott, J. Helen Cross, Curtis J. Rozzelle, Jeffrey P. Blount, Martin M. Tisdall
Summary: This article outlines the clinical and experimental evidence for considering epilepsy surgery prior to drug resistance. The authors argue that earlier surgery is safe and may lead to better outcomes, as well as reducing exposure to antiseizure medications and improving long-term neurocognitive outcomes and quality of life.
Article
Clinical Neurology
Felipe Castro-Villablanca, Friederike Moeller, Suresh Pujar, Felice D'Arco, Rod C. Scott, M. Zubair Tahir, Martin Tisdall, J. Helen Cross, Christin Eltze
Summary: This study found that an ictal EEG may not be mandatory for presurgical evaluation, particularly when a well-defined single unilateral MRI lesion has been identified and the interictal EEG is concordant.
Article
Neurosciences
Jeffrey L. Brabec, Mohamed Ouardouz, J. Matthew Mahoney, Rod C. Scott, Amanda E. Hernan
Summary: Early-life seizures are linked to cognitive deficits. Administration of adrenocorticotropic hormone (ACTH) improves learning and memory, while dexamethasone (Dex) does not. ACTH regulates gene expression, bringing it closer to the normal pattern in the brain.
NEUROBIOLOGY OF DISEASE
(2022)
Article
Environmental Sciences
Sophie Sakkaki, Noemie Cresto, Raphael Chancel, Emma Zub, Pierre Sicard, Tangui Maurice, Sandrine Ellero-Simatos, Laurence Gamet-Payrastre, Nicola Marchi, Julie Perroy
Summary: This study investigates the risk of continuous exposure to glyphosate from prenatal to adulthood and the impact of Shank3 synaptic gene mutation on neurological adaptations. The results show that prenatal exposure to glyphosate increases embryonic mortality rate and leads to neuroinflammation and behavioral adaptations in progenies of Shank3(Delta C/+) mice.
ENVIRONMENT INTERNATIONAL
(2023)
Article
Clinical Neurology
Matthew E. R. Butchbach, Rod C. Scott
Summary: Motor neuron diseases (MNDs) are neuromuscular disorders that primarily affect spinal motor neurons. There are numerous gene mutations associated with pediatric-onset MNDs, but effective treatments are currently lacking. Previous research focused on understanding single gene mutations and developing targeted therapies, but this approach has not been successful for multiple MNDs. We propose a systems biology approach that identifies common molecular and cellular pathways affected in early-onset MNDs, with the goal of expanding therapeutic options.
FRONTIERS IN NEUROLOGY
(2022)