Neuropathology of New-Onset Refractory Status Epilepticus (NORSE).

Autor: Hanin A; Department of Neurology and Immunobiology, Yale University School of Medicine, New Haven, CT, USA. aurelie.hanin@icm-institute.org.; Institut du Cerveau, Paris Brain Institute, ICM, Inserm, CNRS, AP-HP, Hôpital de La Pitié-Salpêtrière, Sorbonne Université, DMU Neurosciences 6, Paris, France. aurelie.hanin@icm-institute.org.; Epilepsy Unit and Department of Clinical Neurophysiology, AP-HP, Hôpital de La Pitié-Salpêtrière, DMU Neurosciences 6, Paris, France. aurelie.hanin@icm-institute.org., Cespedes J; Comprehensive Epilepsy Center, Department of Neurology, Yale University School of Medicine, New Haven, CT, USA.; School of Medicine, Universidad Autonoma de Centro America, San Jose, Costa Rica., Huttner A; Department of Pathology, Yale University School of Medicine, New Haven, CT, USA., Strelnikov D; Comprehensive Epilepsy Center, Department of Neurology, Yale University School of Medicine, New Haven, CT, USA., Gopaul M; Comprehensive Epilepsy Center, Department of Neurology, Yale University School of Medicine, New Haven, CT, USA., DiStasio M; Department of Neurology and Immunobiology, Yale University School of Medicine, New Haven, CT, USA.; Department of Pathology, Yale University School of Medicine, New Haven, CT, USA., Vezzani A; Department of Acute Brain Injury, Istituto di Recerche Farmacologiche Mario Negri IRCCS, Milan, Italy., Hirsch LJ; Comprehensive Epilepsy Center, Department of Neurology, Yale University School of Medicine, New Haven, CT, USA., Aronica E; Department of (Neuro)Pathology, Amsterdam UMC, University of Amsterdam, Amsterdam Neuroscience, Meibergdreef 9, 1105, Amsterdam, The Netherlands.; Stichting Epilepsie Instellingen Nederland (SEIN), Heemstede, The Netherlands.
Jazyk: angličtina
Zdroj: Journal of neurology [J Neurol] 2023 Aug; Vol. 270 (8), pp. 3688-3702. Date of Electronic Publication: 2023 Apr 20.
DOI: 10.1007/s00415-023-11726-x
Abstrakt: New-Onset Refractory Status Epilepticus (NORSE), including its subtype with a preceding febrile illness known as FIRES (Febrile Infection-Related Epilepsy Syndrome), is one of the most severe forms of status epilepticus. Despite an extensive workup (clinical evaluation, EEG, imaging, biological tests), the majority of NORSE cases remain unexplained (i.e., "cryptogenic NORSE"). Understanding the pathophysiological mechanisms underlying cryptogenic NORSE and the related long-term consequences is crucial to improve patient management and preventing secondary neuronal injury and drug-resistant post-NORSE epilepsy. Previously, neuropathological evaluations conducted on biopsies or autopsies have been found helpful for identifying the etiologies of some cases that were previously of unknown cause. Here, we summarize the findings of studies reporting neuropathology findings in patients with NORSE, including FIRES. We identified 64 cryptogenic cases and 66 neuropathology tissue samples, including 37 biopsies, 18 autopsies, and seven epilepsy surgeries (the type of tissue sample was not detailed for 4 cases). We describe the main neuropathology findings and place a particular emphasis on cases for which neuropathology findings helped establish a diagnosis or elucidate the pathophysiology of cryptogenic NORSE, or on described cases in which neuropathology findings supported the selection of specific treatments for patients with NORSE.
(© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)
Databáze: MEDLINE
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