Electrical Status Epilepticus During Sleep: a Case Report of Postmorbid Baseline Evaluation

Objective An 8-year-old, Caucasian, and right-handed female presented for a baseline neuropsychological evaluation due to diagnosis of electrical status epilepticus during sleep (ESES) preceded by postictal, late-night discovery of the patient on the floor and subsequent overnight electroencephalography. This first recognized seizure was tonic-clonic. Method The patient was born at 39 weeks gestation weighing approximately 5 lb, subsequently spending 48 h in NICU due to low birth weight and hypoglycemia. Developmental milestones were recalled within normal limits (WNL). The patient is prescribed levetiracetam, clobazam, and acetazolamide and has undergone multiple EEGs since diagnosis. Remaining medical history unremarkable including absence of known head trauma. Family history is remarkable for anxiety and developmental disability. Results Tests suggest global impairment, particularly in motor abilities and processing speed. Academic achievement is average across basic reading and writing, but verbal learning and memory are significantly diminished unless in story form. Evidence of impulsivity and inattention were present along with deficits in mathematics. Affect recognition was intact and tests of cognitive flexibility and planning were discontinued. Conclusion ESES generally presents early and results in neuropsychological deficits that are dependent upon length of condition and frequency and intensity of seizures. While deficits may improve when ESES subsides in pre-pubescence, some may persist into adulthood with high outcome variability. Baseline and ongoing neuropsychological testing are critical to early, targeted interventions and ongoing care. When early baselines are not feasible, it is important to construct a detailed, data-driven conceptualization to accommodate potential factors involved in notable deficits.