Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions

被引:377
作者
Hirsch, Lawrence J. [1 ]
Gaspard, Nicolas [2 ]
van Baalen, Andreas [3 ]
Nabbout, Rima [4 ]
Demeret, Sophie [5 ]
Loddenkemper, Tobias [6 ,7 ]
Navarro, Vincent [8 ]
Specchio, Nicola [9 ]
Lagae, Lieven [10 ]
Rossetti, Andrea O. [11 ,12 ]
Hocker, Sara [13 ]
Gofton, Teneille E. [14 ]
Abend, Nicholas S. [15 ,16 ,17 ]
Gilmore, Emily J. [1 ]
Hahn, Cecil [18 ,19 ]
Khosravani, Houman [20 ,21 ,22 ]
Rosenow, Felix [23 ]
Trinka, Eugen [24 ,25 ]
机构
[1] Yale Univ, Comprehens Epilepsy Ctr, Dept Neurol, New Haven, CT USA
[2] Univ Libre Bruxelles, Dept Neurol, Hop Erasme, Brussels, Belgium
[3] Univ Kiel, Univ Med Ctr Schleswig Holstein, Dept Neuropediat, Kiel, Germany
[4] Paris Descartes Univ, Necker Enfants Malades Hosp, AP HP, Reference Ctr Rare Epilepsies,Dept Child Neurol, Paris, France
[5] Hop La Pitie Salpetriere, AP HP, Neurol Intens Care Unit, Paris, France
[6] Boston Childrens Hosp, Dept Neurol, Div Epilepsy & Clin Neurophysiol, Boston, MA USA
[7] Harvard Med Sch, Boston, MA USA
[8] UPMC Univ, Sorbonne Univ, Brain & Spine Inst, AP HP,GH Pitie Salpetriere Charles Foix,Epilepsy, Paris, France
[9] IRCCS, Dept Neurosci, Bambino Gesu Childrens Hosp, Rome, Italy
[10] Univ Hosp Leuven, Sect Pediat Neurol, Dept Dev & Regenerat, Leuven, Belgium
[11] CHU Vaudois, Dept Neurol, Lausanne, Switzerland
[12] Univ Lausanne, Lausanne, Switzerland
[13] Mayo Clin, Dept Neurol, Div Crit Care Neurol, Rochester, MN USA
[14] Western Univ, Schulich Sch Med & Dent, Dept Clin Neurol Sci, London, ON, Canada
[15] Childrens Hosp Philadelphia, Dept Neurol, Philadelphia, PA 19104 USA
[16] Childrens Hosp Philadelphia, Dept Pediat, Philadelphia, PA 19104 USA
[17] Univ Penn, Philadelphia, PA 19104 USA
[18] Univ Toronto, Hosp Sick Children, Div Neurol, Toronto, ON, Canada
[19] Univ Toronto, Dept Paediat, Toronto, ON, Canada
[20] Univ Toronto, Sunnybrook Hlth Sci Ctr, Dept Med, Div Neurol, Toronto, ON, Canada
[21] Univ Toronto, Sunnybrook Hlth Sci Ctr, Dept Med, Interdept Div Crit Care, Toronto, ON, Canada
[22] Univ Toronto, Dept Med, Interdept Div Crit Care, Toronto, ON, Canada
[23] Goethe Univ Frankfurt, Ctr Neurol & Neurosurg, Epilepsy Ctr Frankfurt Rhine Main, Frankfurt, Germany
[24] Paracelsus Med Univ, Ctr Cognit Neurosci, Christian Doppler Med Ctr, Dept Neurol, Salzburg, Austria
[25] UMIT, Dept Publ Hlth Hlth Serv & HTA, Inst Publ Hlth, Hall In Tirol, Austria
关键词
autoimmune; cryptogenic; encephalitis; febrile infection-related epilepsy syndrome; febrile status epilepticus; infantile hemiconvulsion-hemiplegia and epilepsy syndrome; new-onset refractory status epilepticus; prolonged febrile convulsions; refractory status epilepticus; super-refractory status epilepticus; PLASMA-EXCHANGE; ENCEPHALOPATHY; IMMUNOTHERAPY; ENCEPHALITIS; INFLAMMATION; ENTITIES; ETIOLOGY; CHILDREN; ADULTS; FEVER;
D O I
10.1111/epi.14016
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
We convened an international group of experts to standardize definitions of New-Onset Refractory Status Epilepticus (NORSE), Febrile Infection-Related Epilepsy Syndrome (FIRES), and related conditions. This was done to enable improved communication for investigators, physicians, families, patients, and other caregivers. Consensus definitions were achieved via email messages, phone calls, an in-person consensus conference, and collaborative manuscript preparation. Panel members were from 8 countries and included adult and pediatric experts in epilepsy, electroencephalography (EEG), and neurocritical care. The proposed consensus definitions are as follows: NORSE is a clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurological disorder, with new onset of refractory status epilepticus without a clear acute or active structural, toxic or metabolic cause. FIRES is a subcategory of NORSE, applicable for all ages, that requires a prior febrile infection starting between 2 weeks and 24 hours prior to onset of refractory status epilepticus, with or without fever at onset of status epilepticus. Proposed consensus definitions are also provided for Infantile Hemiconvulsion-Hemiplegia and Epilepsy syndrome (IHHE) and for prolonged, refractory and super-refractory status epilepticus. This document has been endorsed by the Critical Care EEG Monitoring Research Consortium. We hope these consensus definitions will promote improved communication, permit multicenter research, and ultimately improve understanding and treatment of these conditions.
引用
收藏
页码:739 / 744
页数:6
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