Review
Biochemistry & Molecular Biology
Tzu-Hsin Huang, Ming-Chi Lai, Yu-Shiue Chen, Chin-Wei Huang
Summary: Status epilepticus (SE) is a neurological emergency with high mortality rate. The downregulation of GABAA receptors and upregulation of NMDA and AMPA receptors are the most widely accepted hypothesis underlying BZD resistance.
Article
Clinical Neurology
Fedele Dono, Claudia Carrarini, Mirella Russo, Maria Vittoria De Angelis, Francesca Anzellotti, Marco Onofrj, Laura Bonanni
Summary: The 2019 novel coronavirus has spread globally, with neurological manifestations being increasingly recognized in addition to respiratory symptoms. This report presents a case of post-SARS-CoV-2 autoimmune encephalitis associated with new-onset refractory status epilepticus.
NEUROLOGICAL SCIENCES
(2021)
Review
Clinical Neurology
Aurelie Hanin, Jorge Cespedes, Anita Huttner, David Strelnikov, Margaret Gopaul, Marcello DiStasio, Annamaria Vezzani, Lawrence J. Hirsch, Eleonora Aronica
Summary: New-Onset Refractory Status Epilepticus (NORSE) is a severe form of status epilepticus and its subtype with a preceding febrile illness, known as FIRES, remains largely unexplained. Understanding the pathophysiological mechanisms and long-term consequences of cryptogenic NORSE is crucial for improving patient management and preventing secondary neuronal injury and drug-resistant post-NORSE epilepsy. Neuropathological evaluations have been helpful in identifying the etiologies of some previously unknown cases. This study summarizes the findings of neuropathology studies in NORSE patients, highlighting cases where neuropathology findings aided in diagnosis or treatment selection.
JOURNAL OF 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
Immunology
Tomoya Wadayama, Mikito Shimizu, Tomohiro Yata, Teruyuki Ishikura, Yuta Kajiyama, Daisuke Hirozawa, Tatsusada Okuno, Hideki Mochizuki
Summary: Anti-IL-6 treatment may be an effective method to stop status epilepticus in C-NORSE cases.
JOURNAL OF NEUROIMMUNOLOGY
(2022)
Review
Clinical Neurology
Ioannis Stavropoulos, Jin Han Khaw, Antonio Valentin
Summary: Background: New-onset refractory status epilepticus (NORSE) and its subset of febrile infection-related epilepsy syndrome (FIRES) are devastating clinical presentations with high rates of mortality and morbidity. The recently published consensus on the treatment of these conditions includes anesthetics, antiseizure drugs, antivirals, antibiotics, and immune therapies. Despite the internationally accepted treatment, the outcome remains poor for a significant percentage of patients. Methods: We conducted a systematic review of the use of neuromodulation techniques in the treatment of the acute phase of NORSE/FIRES using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Results: Our search strategy brought up 74 articles of which 15 met our inclusion criteria. A total of 20 patients were treated with neuromodulation. Thirteen cases represented FIRES and in 17 cases the NORSE remained cryptogenic. Ten had electroconvulsive therapy (ECT), seven had vagal nerve stimulation (VNS), and four had deep brain stimulation (DBS); one patient had initially VNS and later DBS. Eight patients were female and nine were children. In 17 out of 20 patients, the status epilepticus was resolved after neuromodulation, while three patients died. Conclusion: NORSE can have a catastrophic course and the first treatment goal should be the fastest possible termination of status epilepticus. The data presented are limited by the small number of published cases and the variability of neuromodulation protocols used. However, they show some potential clinical benefits of early neuromodulation therapy, suggesting that these techniques could be considered within the course of FIRES/NORSE.
FRONTIERS IN NEUROLOGY
(2023)
Article
Neurosciences
Ruochen Dang, Tao Yu, Bingliang Hu, Yuqi Wang, Zhibin Pan, Rong Luo, Quan Wang
Summary: This study aimed to develop an intelligent diagnostic model for encephalitis based on EEG signals. The proposed model, called TT-SGCN, achieved high accuracy and precision in classifying and pairwise-classifying patients with encephalitis. The results suggest that this model has the potential to be an intelligent auxiliary diagnostic tool for encephalitis.
FRONTIERS IN NEUROSCIENCE
(2023)
Article
Clinical Neurology
B. Perin, W. Szurhaj
Summary: NORSE is a clinical presentation characterized by refractory status epilepticus without a clear cause in patients without active epilepsy or other neurological disorders. It can lead to long-term neurological sequelae, cognitive impairment, epilepsy, and functional disability. The cause of NORSE can be autoimmune, infectious, genetic, toxic, etc., and in some cases, the cause cannot be determined. Good communication with the family and rapid consideration of immunotherapy are important in the management of NORSE.
REVUE NEUROLOGIQUE
(2022)
Article
Immunology
Robyn Howarth, Laura Blackwell, Grace Gombolay
Summary: This study examines severity and cognitive status in pediatric NMDARE by comparing objective measures: modified Rankin score (mRS), CASE, and CALS. The results showed improvement in scores from admission to discharge, with variability in individual recovery trajectories. CALS identified three clusters of patients with differential rates of early recovery, and CALS <30 was associated with minimal improvement and poor outcomes.
JOURNAL OF NEUROIMMUNOLOGY
(2023)
Article
Clinical Neurology
Margaret T. Gopaul, Aurelie Hanin, Jorge Cespedes, Yashwanth Pulluru, Karnig Kazazian, Andreas van Baalen, Teneille E. Gofton, Nicolas Gaspard, Lawrence J. Hirsch
Summary: The etiology of new-onset refractory status epilepticus (NORSE), including its subtype with prior fever known as FIRES (febrile infection-related epilepsy syndrome), remains uncertain. This study investigated the seasonality of NORSE cases and found that the incidence of NORSE is highest in the summer and lowest in the spring.
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
Paolo Manganotti, Giovanni Furlanis, Milos Ajcevic, Cristina Moras, Lucia Bonzi, Valentina Pesavento, Alex Buoite Stella
Summary: Neurological manifestations of COVID-19, such as autoimmune encephalitis and new-onset refractory status epilepticus, may require prompt recognition and appropriate treatment, including early EEG evaluation and intravenous immunoglobulin therapy for suspected autoimmune etiology triggered by SARS-CoV-2 infection.
JOURNAL OF NEUROLOGY
(2021)
Article
Clinical Neurology
Laura Mantoan Ritter, Richard Selway
Summary: This article discusses the resistance to drug therapy in the treatment of new-onset refractory status epilepticus (NORSE) and the need for new treatment approaches. Non-drug approaches like neuromodulation, particularly vagal nerve stimulation (VNS), are explored as potential adjunct treatment modalities for NORSE patients. The article summarizes cases treated with VNS, discusses mechanisms of action, timing and protocols, and proposes avenues for future research.
FRONTIERS IN NEUROLOGY
(2023)
Article
Psychiatry
Kasumi Yasuda, Shinya Uenishi, Hiroshi Sakamoto, Kohei Hori, Jinsoo Koh, Shun Takahashi
Summary: This article presents a case of a woman diagnosed with schizophrenia without antiNMDAR antibodies after experiencing NMDAR encephalitis. The study suggests that NMDAR dysfunction may be associated with the onset of schizophrenia.
ASIAN JOURNAL OF PSYCHIATRY
(2022)
Article
Clinical Neurology
Claudine Sculier, Cristina Barcia Aguilar, Nicolas Gaspard, Marina Gainza-Lein, Ivan Sanchez Fernandez, Marta Amengual-Gual, Anne Anderson, Ravindra Arya, Brian T. Burrows, James N. Brenton, Jessica L. Carpenter, Kevin E. Chapman, Justice Clark, William D. Gaillard, Tracy A. Glauser, Joshua L. Goldstein, Howard P. Goodkin, Mark Gorman, Yi-Chen Lai, Tiffani L. McDonough, Mohamad A. Mikati, Anuranjita Nayak, Katrina Peariso, James Riviello, Allison Rusie, Katherine Sperberg, Coral M. Stredny, Robert C. Tasker, Dmitry Tchapyjnikov, Alejandra Vasquez, Mark S. Wainwright, Angus A. Wilfong, Korwyn Williams, Tobias Loddenkemper
Summary: This study characterized the clinical profile and outcomes of new onset refractory status epilepticus (NORSE) in children, and found a relationship between fever onset and status epilepticus (SE). It showed that patients with fever at onset were younger and had shorter SE episodes with better outcomes, while patients with preceding fever had more prolonged SE and worse outcomes. This highlights the importance of fever timing in predicting clinical characteristics and outcomes of NORSE in pediatric patients.
Article
Clinical Neurology
Julien Hebert, Gregory S. Day, Claude Steriade, Richard A. Wennberg, David F. Tang-Wai
CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES
(2018)
Article
Clinical Neurology
Claude Steriade, William Martins, Juan Bulacio, Marcia E. Morita-Sherman, Dileep Nair, Ajay Gupta, William Bingaman, Jorge Gonzalez-Martinez, Imad Najm, Lara Jehi
Article
Clinical Neurology
T. Strohm, C. Steriade, G. Wu, S. Hantus, A. Rae-Grant, M. Larvie
AMERICAN JOURNAL OF NEURORADIOLOGY
(2019)
Article
Clinical Neurology
Claude Steriade, Lara Jehi, Balu Krishnan, Marcia Morita-Sherman, Ahsan N. Moosa, Stephen Hantus, Patrick Chauvel
CLINICAL NEUROPHYSIOLOGY
(2020)
Review
Pharmacology & Pharmacy
Claude Steriade, Jacqueline French, Orrin Devinsky
EXPERT OPINION ON INVESTIGATIONAL DRUGS
(2020)
Review
Clinical Neurology
Claude Steriade, Jeffrey Britton, Russell C. Dale, Avi Gadoth, Sarosh R. Irani, Jenny Linnoila, Andrew McKeon, Xiao-Qiu Shao, Viviana Venegas, Christian G. Bien
Article
Immunology
Julien Hebert, Priti Gros, Sarah Lapointe, Fatima S. Amtashar, Claude Steriade, Catherine Maurice, Richard A. Wennberg, Gregory S. Day, David F. Tang-Wai
JOURNAL OF NEUROIMMUNOLOGY
(2020)
Review
Clinical Neurology
Claude Steriade, Maarten J. Titulaer, Annamaria Vezzani, Josemir W. Sander, Roland D. Thijs
Summary: Systemic autoimmune disorders are more common in patients with epilepsy, especially in systemic lupus erythematosus and type 1 diabetes mellitus. Vascular and metabolic factors play important roles in the relationship between systemic autoimmune disorders and epilepsy. Dysfunction in the immune system can affect neuronal excitability in various ways.
Editorial Material
Clinical Neurology
Claude Steriade
Editorial Material
Clinical Neurology
Claude Steriade
Editorial Material
Clinical Neurology
Claude Steriade
Article
Clinical Neurology
Claude Steriade, Michael R. Sperling, Bree DiVentura, Meryl Lozano, Renee A. Shellhaas, Sudha Kilaru Kessler, Dennis Dlugos, Jacqueline French
Summary: This article presents a pragmatic seizure classification based on the International League Against Epilepsy (ILAE) scheme, designed for use in clinical trials. It also proposes simplifications to the current scheme for the intended use of investigators, industry sponsors, and regulatory agencies.
Editorial Material
Clinical Neurology
Claude Steriade
Summary: In drug-resistant temporal lobe epilepsy, unsupervised machine learning analysis of MRI features identified four latent disease factors that are associated with pathological changes. These factors can accurately predict drug response, surgical outcome, and cognitive dysfunction.
Editorial Material
Clinical Neurology
Claude Steriade
Summary: This study investigated seizure characteristics in antibody-associated autoimmune encephalitis and found that different types of antibodies were associated with different seizure patterns. NMDAR-positive and GAD-positive patients were more likely to have bilateral tonic-clonic seizures, while LGI1-positive patients were more likely to have focal seizures. Furthermore, LGI1-positive patients uniquely experienced faciobrachial dystonic seizures, and seizure occurrence was associated with disease severity in NMDAR-positive patients. These findings highlight the importance of seizures as a diagnostic and therapeutic consideration in antibody-associated autoimmune encephalitis.
Article
Clinical Neurology
Gregory S. Day, Melanie Y. Yarbrough, Peter Kortvelyessy, Harald Pruss, Robert C. Bucelli, Marvin J. Fritzler, Warren Mason, David F. Tang-Wai, Claude Steriade, Julien Hebert, Rachel L. Henson, Elizabeth M. Herries, Jack H. Ladenson, A. Sebastian Lopez-Chiriboga, Neill R. Graff-Radford, John C. Morris, Anne Fagan
Summary: CSF biomarkers indicate that despite neuroaxonal compromise, neuronal integrity is acutely maintained in AME. Low levels of synaptic function biomarkers may reflect antibody-mediated internalization of cell surface receptors and could serve as an acute indicator of antibody-mediated synaptic dysfunction, potentially influencing disease severity and outcomes.
Article
Clinical Neurology
Jaakko Vallinoja, Timo Nurmi, Julia Jaatela, Vincent Wens, Mathieu Bourguignon, Helena Maenpaa, Harri Piitulainen
Summary: The study aimed to assess the effects of lesions related to spastic diplegic cerebral palsy on functional connectivity. Using multiple imaging modalities, the researchers found enhanced functional connectivity in the sensorimotor network of individuals with spastic diplegic cerebral palsy, which was not correlated with hand coordination performance.
CLINICAL NEUROPHYSIOLOGY
(2024)
Article
Clinical Neurology
Francesca Ginatempo, Nicola Loi, John C. Rothwell, Franca Deriu
Summary: This study comprehensively investigated sensorimotor integration in the cranial-cervical muscles of healthy adults and found that the integration of sensory inputs with motor output is profoundly influenced by the type of sensory afferent involved and the functional role played by the target muscle.
CLINICAL NEUROPHYSIOLOGY
(2024)