Nicolas Gaspard1, Brandon P Foreman2, Vincent Alvarez2, Christian Cabrera Kang2, John C Probasco2, Amy C Jongeling2, Emma Meyers2, Alyssa Espinera2, Kevin F Haas2, Sarah E Schmitt2, Elizabeth E Gerard2, Teneille Gofton2, Peter W Kaplan2, Jong W Lee2, Benjamin Legros2, Jerzy P Szaflarski2, Brandon M Westover2, Suzette M LaRoche2, Lawrence J Hirsch2. 1. From the Yale University School of Medicine (N.G., L.J.H.), Department of Neurology, Division of Epilepsy and Clinical Neurophysiology and Comprehensive Epilepsy Center, New Haven, CT; Université Libre de Bruxelles-Hôpital Erasme (N.G., B.L.), Brussels, Belgium; University of Cincinnati Department of Neurology and Rehabilitation Medicine (B.P.F.), OH; Department of Neurology (V.A.), Hôpital de Sion; Department of Clinical Neurosciences (V.A.), Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland; Department of Neurology (V.A., J.W.L.), Brigham and Women's Hospital, Harvard Medical School, Boston MA; Emory University School of Medicine (C.C.K., S.M.L.), Atlanta, GA; Johns Hopkins Bayview Medical Center (J.C.P., P.W.K.), Department of Neurology, and Johns Hopkins University School of Medicine, Baltimore MD; Department of Neurology (A.C.J., E.M.), Columbia University, New York, NY; Vanderbilt University Medical Center (K.F.H.), Nashville, TN; Hospital of the University of Pennsylvania (S.E.S.), Philadelphia; Feinberg School of Medicine (A.E., E.E.G.), Northwestern University, Chicago, IL; University of Western Ontario (T.G.), Canada; University of Alabama at Birmingham (J.P.S.); and Department of Neurology (B.M.W.), Massachusetts General Hospital, Boston. ngaspard@ulb.ac.be. 2. From the Yale University School of Medicine (N.G., L.J.H.), Department of Neurology, Division of Epilepsy and Clinical Neurophysiology and Comprehensive Epilepsy Center, New Haven, CT; Université Libre de Bruxelles-Hôpital Erasme (N.G., B.L.), Brussels, Belgium; University of Cincinnati Department of Neurology and Rehabilitation Medicine (B.P.F.), OH; Department of Neurology (V.A.), Hôpital de Sion; Department of Clinical Neurosciences (V.A.), Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland; Department of Neurology (V.A., J.W.L.), Brigham and Women's Hospital, Harvard Medical School, Boston MA; Emory University School of Medicine (C.C.K., S.M.L.), Atlanta, GA; Johns Hopkins Bayview Medical Center (J.C.P., P.W.K.), Department of Neurology, and Johns Hopkins University School of Medicine, Baltimore MD; Department of Neurology (A.C.J., E.M.), Columbia University, New York, NY; Vanderbilt University Medical Center (K.F.H.), Nashville, TN; Hospital of the University of Pennsylvania (S.E.S.), Philadelphia; Feinberg School of Medicine (A.E., E.E.G.), Northwestern University, Chicago, IL; University of Western Ontario (T.G.), Canada; University of Alabama at Birmingham (J.P.S.); and Department of Neurology (B.M.W.), Massachusetts General Hospital, Boston.
Abstract
OBJECTIVES: The aims of this study were to determine the etiology, clinical features, and predictors of outcome of new-onset refractory status epilepticus. METHODS: Retrospective review of patients with refractory status epilepticus without etiology identified within 48 hours of admission between January 1, 2008, and December 31, 2013, in 13 academic medical centers. The primary outcome measure was poor functional outcome at discharge (defined as a score >3 on the modified Rankin Scale). RESULTS: Of 130 cases, 67 (52%) remained cryptogenic. The most common identified etiologies were autoimmune (19%) and paraneoplastic (18%) encephalitis. Full data were available in 125 cases (62 cryptogenic). Poor outcome occurred in 77 of 125 cases (62%), and 28 (22%) died. Predictors of poor outcome included duration of status epilepticus, use of anesthetics, and medical complications. Among the 63 patients with available follow-up data (median 9 months), functional status improved in 36 (57%); 79% had good or fair outcome at last follow-up, but epilepsy developed in 37% with most survivors (92%) remaining on antiseizure medications. Immune therapies were used less frequently in cryptogenic cases, despite a comparable prevalence of inflammatory CSF changes. CONCLUSIONS: Autoimmune encephalitis is the most commonly identified cause of new-onset refractory status epilepticus, but half remain cryptogenic. Outcome at discharge is poor but improves during follow-up. Epilepsy develops in most cases. The role of anesthetics and immune therapies warrants further investigation.
OBJECTIVES: The aims of this study were to determine the etiology, clinical features, and predictors of outcome of new-onset refractory status epilepticus. METHODS: Retrospective review of patients with refractory status epilepticus without etiology identified within 48 hours of admission between January 1, 2008, and December 31, 2013, in 13 academic medical centers. The primary outcome measure was poor functional outcome at discharge (defined as a score >3 on the modified Rankin Scale). RESULTS: Of 130 cases, 67 (52%) remained cryptogenic. The most common identified etiologies were autoimmune (19%) and paraneoplastic (18%) encephalitis. Full data were available in 125 cases (62 cryptogenic). Poor outcome occurred in 77 of 125 cases (62%), and 28 (22%) died. Predictors of poor outcome included duration of status epilepticus, use of anesthetics, and medical complications. Among the 63 patients with available follow-up data (median 9 months), functional status improved in 36 (57%); 79% had good or fair outcome at last follow-up, but epilepsy developed in 37% with most survivors (92%) remaining on antiseizure medications. Immune therapies were used less frequently in cryptogenic cases, despite a comparable prevalence of inflammatory CSF changes. CONCLUSIONS:Autoimmune encephalitis is the most commonly identified cause of new-onset refractory status epilepticus, but half remain cryptogenic. Outcome at discharge is poor but improves during follow-up. Epilepsy develops in most cases. The role of anesthetics and immune therapies warrants further investigation.
Authors: Sarosh R Irani; Katarzyna Bera; Patrick Waters; Luigi Zuliani; Susan Maxwell; Michael S Zandi; Manuel A Friese; Ian Galea; Dimitri M Kullmann; David Beeson; Bethan Lang; Christian G Bien; Angela Vincent Journal: Brain Date: 2010-06 Impact factor: 13.501
Authors: Carol A Glaser; Sabrina Gilliam; Somayeh Honarmand; Jay H Tureen; Daniel H Lowenstein; Larry J Anderson; Andrew W Bollen; Marylou V Solbrig Journal: Neurocrit Care Date: 2008 Impact factor: 3.532
Authors: Olga Taraschenko; Howard S Fox; Sean J Pittock; Anastasia Zekeridou; Maftuna Gafurova; Ember Eldridge; Jinxu Liu; Shashank M Dravid; Raymond Dingledine Journal: Epilepsia Date: 2019-02-11 Impact factor: 5.864
Authors: Raoul Sutter; Gian Marco De Marchis; Saskia Semmlack; Peter Fuhr; Stephan Rüegg; Stephan Marsch; Wendy C Ziai; Peter W Kaplan Journal: CNS Drugs Date: 2017-01 Impact factor: 5.749
Authors: Vincent Alvarez; Jong Woo Lee; M Brandon Westover; Frank W Drislane; Jan Novy; Mohamed Faouzi; Nicola A Marchi; Barbara A Dworetzky; Andrea O Rossetti Journal: Neurology Date: 2016-09-24 Impact factor: 9.910