Claudine Sculier1, Nicolas Gaspard2. 1. Département de Neurologie, Université Libre de Bruxelles, Hôpital Erasme, Brussels, Belgium; Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA. Electronic address: claudine.sculier@erasme.ulb.ac.be. 2. Département de Neurologie, Université Libre de Bruxelles, Hôpital Erasme, Brussels, Belgium; Comprehensive Epilepsy Center, Neurology Department, Yale University School of Medicine, New Haven, CT, USA.
Abstract
PURPOSE: To summarize the clinical features, suggested work-up, treatment and prognosis of new-onset refractory status epilepticus (NORSE), a condition recently defined as the occurrence of refractory status epilepticus (RSE) in a patient without active epilepsy, and without a clear acute or active structural, toxic or metabolic cause; and of the related syndrome of febrile infection-related epilepsy syndrome (FIRES), also recently defined as a subgroup of NORSE preceded by a febrile illness between 2 weeks and 24 h prior to the onset of RSE. METHOD: Narrative review of the medical literature about NORSE and FIRES. RESULTS: NORSE and FIRES mainly affect school-age children and young adults. A prodromal phase with flu-like symptoms precedes the SE onset in two third of NORSE cases, and by definition in all FIRES. Status epilepticus usually starts with repeated focal seizures with secondary bilateralization. Most cases evolve to super RSE (SRSE) and have unfavorable outcome, with short-term mortality of 12-27%, long-term disability and epilepsy. No specific imaging or laboratory abnormalities have been identified so far that allows an early diagnosis and half of adult cases remain of unknown etiology. A standardized diagnostic algorithm is provided and. Autoimmune encephalitis is the most frequent identified cause. In the absence of specific diagnosis, immunotherapy could be tried in addition to antiepileptic treatment. CONCLUSIONS: This review presents the rare but devastating syndrome of NORSE, including the subcategory of FIRES. Early recognition with complete work-up is primordial to identify the underlying cause and promptly start appropriate treatment.
PURPOSE: To summarize the clinical features, suggested work-up, treatment and prognosis of new-onset refractory status epilepticus (NORSE), a condition recently defined as the occurrence of refractory status epilepticus (RSE) in a patient without active epilepsy, and without a clear acute or active structural, toxic or metabolic cause; and of the related syndrome of febrile infection-related epilepsy syndrome (FIRES), also recently defined as a subgroup of NORSE preceded by a febrile illness between 2 weeks and 24 h prior to the onset of RSE. METHOD: Narrative review of the medical literature about NORSE and FIRES. RESULTS: NORSE and FIRES mainly affect school-age children and young adults. A prodromal phase with flu-like symptoms precedes the SE onset in two third of NORSE cases, and by definition in all FIRES. Status epilepticus usually starts with repeated focal seizures with secondary bilateralization. Most cases evolve to super RSE (SRSE) and have unfavorable outcome, with short-term mortality of 12-27%, long-term disability and epilepsy. No specific imaging or laboratory abnormalities have been identified so far that allows an early diagnosis and half of adult cases remain of unknown etiology. A standardized diagnostic algorithm is provided and. Autoimmune encephalitis is the most frequent identified cause. In the absence of specific diagnosis, immunotherapy could be tried in addition to antiepileptic treatment. CONCLUSIONS: This review presents the rare but devastating syndrome of NORSE, including the subcategory of FIRES. Early recognition with complete work-up is primordial to identify the underlying cause and promptly start appropriate treatment.
Authors: Alberto Cossu; Tommaso Lo Barco; Francesca Darra; Elena Fontana; Elena Fiorini; Martina Marangone; Paolo Biban; Bernardo Dalla Bernardina; Gaetano Cantalupo Journal: Neurol Clin Pract Date: 2021-04
Authors: Lisa Langenbruch; Christine Strippel; Dennis Görlich; Christian E Elger; Gabriel Möddel; Sven G Meuth; Christoph Kellinghaus; Heinz Wiendl; Stjepana Kovac Journal: J Neurol Date: 2021-05-21 Impact factor: 4.849