Objectives: To evaluate clinical spectrum and therapeutic outcomes of electrical status epilepticus during sleep (ESES).

Methods: ESES cases were divided into 4 groups regarding cognition before treatment: Intellectual disability (ID) (ID before first seizure); Cognitive impairment (CI); Attention deficit (AD); Normal group (NG). The outcomes were classified into 3 groups: Good prognosis (GP): Recurrence group (RG); Seizure control (SC): seizure control only.

Results: 39 cases were included, with 3 benign childhood epilepsy with centro-temporal spikes (BECT), 26 BECT variant (BECTv), 8 symptomatic partial epilepsy (SPE) (2 focal cortical dysplasia (FCD)), and 2 aquired epileptic aphasia (LKS). The mean first seizure age (year) and initial occurrance of ESES age (year) were 4.14 and 6.14 in ID (n=7), 3.63 and 5.31 in CI (n=8), 4.61 and 6.13 in AD (n=19), 6.6 and 7.2 in NG (n=5), with significant defference on first seizure age between NG and CI (p=0.027). 18 in GP (13 BECTv, 1 SPE, 1 LKS) including 5 single antiepileptic drug (AED), 8 AED combined, 5 AED plus steroid and IVIG, 16 in RG (11 BECTv, 4 SPE, 1 LKS), 5 in SC (2 BECTv, 3 SPE) were classified. 2 FCD in RG had good prognosis through surgical treatment, whereas 13 from RG/SC refused AED combined or steroid plus IVIG therapies. There was significant prognostic correction among BECT, BECTv and SPE (p=0.041).

Conclusion: The earlier the first seizure is, the greater the risk of acquired cognitive impairment; standardized treatment is important; the prognosis is related to epileptic syndromes types.