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Electrical status epilepticus in sleep (ESES): Clinical and EEG characteristics and response to treatments.

Electrical status epilepticus in sleep (ESES) is defined as an age related, self-limited epileptic encephalopathy. It is characterized by heterogeneous clinical manifestations and a specific electroencephalographic (EEG) pattern of continuous spikes and waves during slow sleep (CSWS). Objective The aim of this study was to describe the electroclinical spectrum in children with electrical status epilepticus in sleep (ESES), and assessment of treatment pattern. Methods Clinical data of 16 patients with ESES/CSWS syndrome who were treated and followed at least two years were analyzed. Inclusion criteria were as follows: (1) Determination of the ESES pattern on the EEG and a (2) follow-up period of two to four years. All patients underwent a clinical evaluation including history, physical and neurological examinations, sleep and awake EEGs, and brain MRI. Patients with an underlying etiology were classified as symptomatic while others were classified as idiopathic. Records of EEGs of patients were reevaluated to determine two aspects of ESES: (1) The spike–wave index (SWI) on the NREM sleep EEG and (2) the area of maximum amplitude of continuous epileptic activity. The SWI on the NREM sleep EEG during the ESES period was visually calculated. The ranges of the SWI considered were as follows: N85– 100% (typical ESES) and 50–85% (atypical ESES). We also defined the ESES pattern as anterior if the maximum amplitude of spike–waves was in the frontal, frontocentral, or frontotemporal areas and as posterior if the maximum amplitude of spike–waves was in the posterior temporal, temporo-occipital, or occipital areas on the EEG. Results Complete data were available in 16 children. Age at ESES diagnosis ranged from 36 to 84 months. Antiepileptic drugs were used as first treatment for ESES in 16/16 (100%). Electrical status epilepticus in sleep initially resolved in 87%, but 56% had subsequent relapse. At last follow-up, ESES resolved in 56%. And those children was seizures free. Conclusion We found high failure rate of first line AEDs inpreventing ESES, and high relapse rate. There are no standardizations of managment of ESES.We were managed by ourexperiences, relevant medical records and clinical trials.

electrical status epilepticus in sleep, ESES, EEG, treatment

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