Abstract

Objective: Electrical status epilepticus of sleep (ESES) is a rare childhood epilepsy associated with global neuropsychological regression. Typically, ESES consists of bi-hemispheric epileptiform activity, however we present two patients (P1, age 8 & P2, age 11) with focal right hemisphere ESES. Similarities and differences between their neuropsychological profiles are highlighted. Method: Language milestones developed within normal limits for P1, but were delayed for P2. Both P1 and P2 were delayed in motor skills. Seizure onset occurred at age four (P1) and age five (P2). EEGs for both patients repeatedly show frequent right hemisphere epileptiform discharges. Historically, epileptiform activity has remained treatment resistant for both patients. Results: Intellectual functioning declined for both patients over time. At age five, P1's overall IQ was 116 and was 85 at age 8. At age 9, P2's overall IQ was 99 and was 86 at age 11. P2's IQ decline is characteristic of a dramatic loss in verbal skills (2013 VCI = 97; 2014 VCI = 83), whereas verbal skills remain a relative strength for P1. Attention difficulties are the most prominent areas of concern across patients. For P1, working memory, executive functioning, and processing speed were consistent areas of weakness. For P2, executive functioning and visuospatial skills were consistent areas of weakness. Conclusion(s): There is evidence of normative neurocognitive declines in both patients over time. Within these cases of focal ESES, global decline was observed. However, P2 showed a deterioration in language out of proportion to other cognitive abilities, whereas P1 retained verbal skills as a relative strength.