Combination of intravenous immunoglobulin and steroid pulse therapy improves outcomes of febrile refractory status epilepticus

•Patients of febrile status epilepticus have high mortality and morbidity.•IVIG combined steroid pulse therapy is associated with better outcomes of febrile status epilepticus.•We suggest early and aggressive immunotherapy for febrile status epilepticus. 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. 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. 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. 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.