Role of neuropsychology in identification of CSWS in a school-aged child with a remote neurological insult

Abstract

Electrical status epilepticus of slow-wave sleep (ESES) is characterized by excessive interictal spike-wave discharges on EEG during sleep and can occur in the absence of overt clinical seizures. Continuous spike-wave during slow wave sleep (CSWS), an epilepsy syndrome associated with ESES, is associated with a plateau/decline in cognitive development and increases in behavioral and emotional dysregulation. Here we present a case in which neuropsychological (NP) evaluation initially ordered based on memory and attention concerns led to the identification of subclinical seizure activity and an evolving epileptic encephalopathy in an 11-year-old child with a history of remote neurological insult. The patient was referred for an initial NP evaluation at age 8 which revealed weaknesses in functions typically mediated by the dominant (usually left) hemisphere juxtaposed with her left hemiparesis. EEG was recommended which showed independent, multifocal spike and sharp wave discharges exacerbated by sleep. Follow-up NP evaluations over the following 26 months, during which time aggressive treatment was initiated, coincided with EEG findings of an evolving epileptic encephalopathy in the patient who continued to remain free from clinical seizures. This case highlights the importance of comprehensive epilepsy care and routine involvement of neuropsychology in the management of complex epilepsy patients.

Keywords

• CSWS
• ESES
• Neuropsychological assessment
• Pediatric epilepsy
• Pediatric stroke