Lack of Prominent Cognitive Regression in the Long-term Outcome of Patients Having Electrical Status Epilepticus During Sleep With Different Types of Epilepsy Syndromes.

The purpose was to document 4 patients with different epilepsy syndromes, showing electrical status epilepticus during sleep (ESES), without marked cognitive and behavioral regression in the long-term follow-up. The mean age at onset of seizures was 8 years. Absences, myoclonic, focal motor, or generalized tonic - clonic seizures and drop attacks were the prominent seizure types. The neurological examination and neuroimaging findings revealed no abnormality. Focal epileptiform electroencephalographic (EEG) activity was seen in 3 cases, whereas generalized photosensitive epileptic discharges were detected in 1 patient with juvenile myoclonic epilepsy. Neuropsychological evaluations of all cases were within the normal range, and deterioration in mental status was not observed during their mean follow-up duration of 14 years. Our data support the view that ESES can emerge along with different types of childhood epilepsy syndromes, including idiopathic generalized epilepsies, and may not always be a poor prognostic factor.