Jainn-Jim Lin1, Yu Wang2, Shih-Yun Lan3, Oi-Wa Chan4, Shao-Hsuan Hsia4, Min-Liang Chou3, Po-Cheng Hung3, Meng-Ying Hsieh3, I-Jun Chou3, Huei-Shyong Wang3, Kuang-Lin Lin5. 1. Division of Pediatric Critical Care and Pediatric Neurocritical Care Center, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan; Molecular Infectious Disease Research Center, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan. 2. Division of Pediatric Critical Care and Pediatric Neurocritical Care Center, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Department of Pediatrics, Chang Gung Memorial Hospital, Chiayi, Taiwan. 3. Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan. 4. Division of Pediatric Critical Care and Pediatric Neurocritical Care Center, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan. 5. Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan. Electronic address: lincgh@cgmh.org.tw.
Abstract
BACKGROUND: Febrile infections are an important cause of paediatric refractory status epilepticus, and immune-mediated mechanisms and inflammatory processes have been associated with neurological manifestations in such patients. The aim of this study was to investigate the effects of immunotherapy as adjuvant treatment for febrile refractory status epilepticus. METHODS: We retrospectively reviewed cases of febrile refractory status epilepticus in a paediatric intensive care unit between January 2000 and December 2013 and analysed their clinical characteristics. Patients positive for antineuronal antibodies against surface antigens were excluded. RESULTS: We enrolled 63 patients (38 boys), aged 1-18 years, all of whom received multiple antiepileptic drugs. Twenty-nine (46%) of the patients received intravenous immunoglobulin alone, 16 (25.4%) received a combination of intravenous immunoglobulin and methylprednisolone pulse therapy, and 18 (28.6%) did not receive immunotherapy treatment. Overall, 12 (19%) patients died within 1 month. After 6 months, 12 (20%) patients had good neurological outcomes, including two who returned to baseline and 13 (29.5%) who had favourable seizure outcomes. We compared the outcomes of the different treatments, and found that a combination of intravenous immunoglobulin and methylprednisolone pulse therapy had the best neurological and seizure outcomes at 6 months compared to intravenous immunoglobulin alone and no immunotherapy. CONCLUSIONS: Our observational study showed that a combination of intravenous immunoglobulin and methylprednisolone pulse therapy as adjuvant treatment for febrile refractory status epilepticus was associated with better neurological and seizure outcomes. Further prospective studies are needed to confirm these findings.
BACKGROUND:Febrile infections are an important cause of paediatric refractory status epilepticus, and immune-mediated mechanisms and inflammatory processes have been associated with neurological manifestations in such patients. The aim of this study was to investigate the effects of immunotherapy as adjuvant treatment for febrile refractory status epilepticus. METHODS: We retrospectively reviewed cases of febrile refractory status epilepticus in a paediatric intensive care unit between January 2000 and December 2013 and analysed their clinical characteristics. Patients positive for antineuronal antibodies against surface antigens were excluded. RESULTS: We enrolled 63 patients (38 boys), aged 1-18 years, all of whom received multiple antiepileptic drugs. Twenty-nine (46%) of the patients received intravenous immunoglobulin alone, 16 (25.4%) received a combination of intravenous immunoglobulin and methylprednisolone pulse therapy, and 18 (28.6%) did not receive immunotherapy treatment. Overall, 12 (19%) patients died within 1 month. After 6 months, 12 (20%) patients had good neurological outcomes, including two who returned to baseline and 13 (29.5%) who had favourable seizure outcomes. We compared the outcomes of the different treatments, and found that a combination of intravenous immunoglobulin and methylprednisolone pulse therapy had the best neurological and seizure outcomes at 6 months compared to intravenous immunoglobulin alone and no immunotherapy. CONCLUSIONS: Our observational study showed that a combination of intravenous immunoglobulin and methylprednisolone pulse therapy as adjuvant treatment for febrile refractory status epilepticus was associated with better neurological and seizure outcomes. Further prospective studies are needed to confirm these findings.
Authors: Laurent M Willems; Sebastian Bauer; Kolja Jahnke; Martin Voss; Felix Rosenow; Adam Strzelczyk Journal: CNS Drugs Date: 2020-08 Impact factor: 5.749