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(Abst. 1.214), 2017

Long-Term Outcomes in Pediatric Refractory Status Epilepticus (the pSERG cohort)
Authors: Juan A. Piantino, Doernbecher Children’s Hospital, Oregon Health & Science University, Portland, OR, United States; Marina Gaínza-Lein, Boston Children’s Hospital, Harvard University Medical School, Boston, MA, United States, Universidad Austral de Chile, Valdivia, Chile.; Kevin Chapman, Children’s Hospital Colorado, University of Colorado School of Medicine, Aurora, CO, United States; 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; 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; William Gaillard, Children’s National Medical Center, George Washington University School of Medicine and Health Sciences, Washington, DC, United States.; Tracy Glausser, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, United States; Joshua L. Goldstein, Northwestern University Feinberg School of Medicine, Chicago, IL, United States; Howard P. Goodkin, University of Virginia Health System, Charlottesville, VA, 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 J. Riviello, Columbia University Medical Center; 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; Robert C. Tasker, Boston Children’s Hospital, Harvard University Medical School, Boston, MA, United States; 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; Angus Wilfong, Barrow Neurological Institute, Phoenix Children’s Hospital; Korwyn Williams, Barrow Neurological Institute, Phoenix Children’s Hospital, University of Arizona School of Medicine, Phoenix, AZ, United States; and Tobias Loddenkemper, Boston Children’s Hospital, Harvard University Medical School, Boston, MA, USA
Content: Rationale: Describe long term outcomes in refractory convulsive status epilepticus (RSE) and identify predictors of new neurological deficits after RSE. Methods: This multicenter prospective observational study included pediatric patients who presented with RSE defined as failure of =2 anti-seizure medications (ASM) to terminate SE, or initiation of a continuous infusion for seizure control. Patients were admitted to the hospital from 2011-2016 followed-up for at least 1 year by chart review. Our primary outcome was the development of any new neurological deficits (dichotomous: yes/no) in the latest follow-up compared to before the RSE. We excluded patients who died during the RSE hospitalization. Results: One hundred and sixty-three patients presented with RSE and had follow-up for at least 1 year. Median (p25-p75) follow-up time was 2.1 (1.2– 3.3) years, 86 (52.8%) were males, and median (p25-p75) age was 4.5 (1.17 – 10) years. The RSE etiology was unknown in 61 patients (37.4%), structural in 49 patients (30.1%), genetic in 32 patients (19.6%), metabolic in 5 patients (3.1%), and other in 16 patients (9.8%). Forty-one (25.2%) patients had prior developmental delay.Eleven patients (6.8%) died between hospital discharge and follow-up. The cause of death was structural in 2, due to complications of genetic/metabolic disorders in 6, and unknown in 3. Two patients (3.3%) died of probable sudden unexpected death in epilepsy (SUDEP). At follow-up, 142 patients (87.1%) were on ASM, and 109 (67.7%) continued to have unprovoked seizures. Patients were on a median (p25-p75) number of 2 (1-4) ASM. Out of 77 patients with no prior history of epilepsy, 60 (77.9%) were prescribed ASM and 42 (54.5%) presented with additional unprovoked seizures. At follow-up, 26 (16.15%) patients presented with additional RSE episodes, and 84 (53.9%) patients required a rescue medication on at least one occasion. Sixteen (12.6%) of the 127 patients with no prior history of RSE had repeated episodes of RSE at follow-up, and 60 (47.2%) required rescue medications on at least one occasion during follow-up.Thirty-six (34.6%) patients developed new neurological deficits during follow-up. In multivariate analysis, the only predictor for new deficit was RSE duration (p=0.04) (Table 1). Patients with new deficits had a median (p25-p75) RSE seizure duration of 27.75 (1.75-144) hours (versus 3.68 (2-13) hours in patients without new deficits) (p=0.023) (Table 1). Conclusions: One third of patients with RSE developed new neurological deficits during long term follow-up, and the only identified predictor for new neurological deficits was RSE seizure duration. More than half of the patients with no prior epilepsy before RSE presented with recurrent unprovoked seizures. Funding: Supported by Pediatric Epilepsy Research Foundation and Epilepsy Research Fund.
Figure 1