Encephalopathy with electrical status epilepticus in sleep (ESES) syndrome is characterized by a near-continuous spike-and-wave discharges during sleep with marked developmental regression, mainly in speech, and the presence of clinical seizures. Although the etiology ofESES is generally unknown, its resistance to antiseizure medication (ASM), and favorable responses to oral corticosteroids (OCS), support a role for inflammation. However, the prolonged use of OCS results in undesirable side effects and alternative treatment measures are needed. Herein, we present a patient with ESES who revealed responsed to a combination of immunomodulating agents other than OCS. The patient revealed 30, 50, and 100%, reduction in the ESES pattern on EEG with the sequential addition of anakinra (interleukin-1ß inhibitor), intravenous immunoglobulin (IVIg), and sirolimus, an inhibitor of mammalian target of rapamycin (mTOR) respectively, after discontinuation of OCS due to side effects. This combination of immune-modulating agents, that were selected based on monocyte cytokine profiles, also resulted in a gradual improvement of speech and behavioral symptoms. This case indicates a possible use of immunomodulating agents other than OCS for ESES syndrome.
Keywords: ESES; IL-1ß; Oral corticosteroid; mTOR inhibitor.
© 2020 The Authors.