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(Abst. 1.154), 2017

Clinical profile of NORSE Syndrome in children from a multicenter US registry (The pSERG cohort)
Authors: Claudine Sculier, Université Libre de Bruxelles, Hôpital Erasme, Brussels, Belgium; Nicolas Gaspard, Université Libre de Bruxelles, Hôpital Erasme, Brussels, Belgium; Marina Gaínza-Lein, Boston Children’s Hospital, Harvard University Medical School, Boston, MA, United States; Universidad Austral de Chile, Valdivia, Chile; Iván Sánchez Fernández, Boston Children’s Hospital, Harvard University Medical School, Boston, MA, United States;Hospital Sant Joan de Déu, Universidad de Barcelona, Barcelona, Spain; Anne Anderson, Baylor College of Medicine, Texas Children's Hospital; Ravindra Arya, Cincinnati Children’s Hospital Medical Center, University of Cincinnati, Cincinnati, OH, United States; Brian T. Burrows, 8 Barrows Neurological Institute, Phoenix Children’s Hospital, Department of Pediatrics, University of Arizona School of Medicine, Phoenix, AZ; Department of Neurology, Mayo Clinic, Scottsdale, AZ, USA; J. Nicholas Brenton, University of Virginia Health System, Charlottesville, VA, United States.; Jessica L. Carpenter, Children’s National Medical Center, George Washington University School of Medicine and Health Sciences. Washington, DC, United States; Kevin Chapman, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, United States; William D. Gaillard, Children’s National Medical Center, George Washington University School of Medicine and Health Sciences, Washington, DC, United States.; Tracy A. Glauser, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, United States; Howard P. Goodkin, The University of Virginia Health System, Charlottesville, VA, USA; Yi-Chen Lai, Baylor College of Medicine. Houston, TX, United States.; Tiffani L. McDonough, Columbia University Medical Center, Columbia University. New York, NY, United States.; Mohamad A. Mikati, Duke University Medical Center, Duke University, Durham, NC, United States; Anuranjita Nayak, Baylor College of Medicine. Houston, TX, United States.; Katrina Peariso, Cincinnati Children’s Hospital Medical Center, University of Cincinnati, Cincinnati, OH, United States; James Riviello, Columbia University Medical Center, Columbia University, New York City, NY, United States.; Allison Rusie, Northwestern University Feinberg School of Medicine, Chicago, IL, USA; Katherine Sperberg, Children's National Health System, George Washington University School of Medicine and Health Sciences; Dmitry Tchapyjnikov, Duke University Medical Center, Duke University. Durham, NC, United States; Mark S. Wainwright, Northwestern University Feinberg School of Medicine, Chicago, IL, United States; Korwyn Williams, Barrow Neurological Institute, Phoenix Children’s Hospital, University of Arizona School of Medicine, Phoenix, AZ, United States; Angus Wilfong, Phoenix Children’s Hospital, University of Arizona College of Medicine; and Tobias Loddenkemper, Boston Children’s Hospital, Harvard University Medical School, Boston, MA, USA
Content: Rationale: New-onset refractory status epilepticus (NORSE) was recently defined in children.  We aimed to outline the clinical profile of NORSE in a pediatric population, compare it with the febrile illness related epileptic syndrome (FIRES) and identify features associated with outcome.  Methods: We enrolled patients within the pediatric Status Epilepticus Research Group (pSERG) cohort with the following inclusion criteria: 1) age 1month-18years; 2) first episode of refractory status epilepticus (RSE) requiring more than 2 anti-seizure medications (ASM); 3) RSE episode between January 2011 and October 2016. We excluded patients with a history of epilepsy or a readily identifiable cause of status epilepticus (SE), according to the consensus NORSE definition: patients without history of epilepsy and new onset of RSE without readily identifiable cause. FIRES is a subgroup of  NORSE that requires a prior febrile illness. Since this was an observational study, there was no standardized protocol regarding the management of RSE. The primary outcome was no return to baseline function at hospital discharge or death (worse outcome). Bonferroni’s correction was applied for multiple comparisons. Adjusted p-values are provided and considered significant if less than 0.05. Results: Of 244 patients with a first episode of RSE, 46 patients met criteria for NORSE. Median age was 2.4 years and 25 (54%) were female. A definitive etiology was found in 6 patients (13%): genetic (2.2%), auto-immune (4.3%) or infectious (6.5%). Twenty-one (45%) patients were diagnosed with febrile SE (FSE) and 19 (41%) patients remained with an unknown cause (Table 1). Fourteen (30%) patients correspond to FIRES. CSF results were abnormal in 16 (39%) cases, including 10 FIRES cases. Twenty eight (60%) patients returned to baseline function and 18 (40%) did not, including 12 (86%) FIRES. Three (6.5%) patients died. Patients with worse outcome were older (5.8 vs 1.2 years; p=0.0072), more often presented with a preceding febrile illness (13[72%] vs 6[39%]; p=0.016), longer ICU stay (45 vs 3 days; p=0.0016) and longer electroclinical SE duration (264 vs 12 hours; p=0.0063). They required more ASM (2.5 vs 2; adjusted p=0.048), anesthetics in continuous infusion (2 vs 0; p=0.023), ketogenic diet (8 vs 1; p=0.018) or immunotherapies (10 vs 2; p=0.0072). Patients with FSE had better outcome (20 vs. 1; p < 0.001) (Table 2). Conclusions: The etiology of NORSE in children is unknown in almost half of the cases, with FIRES and FSE constituting the largest subgroups. Our study shows outcomes better than adult NORSE patients. Consistent with previous literature, cases of NORSE attributed to FIRES (preceding febrile illness) and prolonged super-refractory cases were associated with worse outcome. Funding: Epilepsy Foundation of America (EF-213583, Targeted Initiative for Health Outcomes), American Epilepsy Society/Epilepsy Foundation of America Infrastructure Awards, Pediatric Epilepsy Research Foundation, International Federation of Clinical Neurophysiology (IFCN), Epilepsy Research Fund.
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