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Status epilepticus is one of the most common neurological emergencies in children and adults. Febrile status epilepticus cases are often associated with inflammatory neurological diseases caused by specific pathogens or antineuronal autoimmunity. In addition, there is a subgroup of super-refractory status epilepticus triggered by fever and having no known cause.1 This condition is designated as either acute encephalitis with refractory, repetitive partial seizures (AERRPS)2 or febrile infection-related epilepsy syndrome (FIRES).3 The pathogenesis of AERRPS/FIRES is currently unknown. A close relationship between febrile illness and status epilepticus suggests deleterious effects of inflammation and autoimmunity on the onset and progression of seizure. However, immune mechanisms in human status epilepticus associated with isolated fever have not been fully elucidated.
We report a comprehensive study of the inflammatory mediators in paediatric cases of AERRPS. We show a marked upregulation of proinflammatory cytokines and chemokines in the cerebrospinal fluid (CSF) of patients with this condition.
We defined AERRPS using the criteria shown in online supplementary table S1. Between April 2010 and July 2013, 14 patients with AERRPS and 14 patients with other inflammatory neurological diseases (OIND) were enrolled in the study. Serum and CSF specimens from patients with AERRPS and OIND were collected between 0 and 39 days from the onset of neurological symptoms. Eighteen patients with non-inflammatory neurological diseases (NIND) served as a control group. Additionally, the Shizuoka Institute of Epilepsy and Neurological Disorders provided us with 13 conserved CSF specimens from patients with AERRPS who fulfilled the same criteria, which we also included in our analysis. …