Long-term evolution of neuropsychological competences in encephalopathy with status epilepticus during sleep: a variable prognosis

Epilepsia. 2013 Oct:54 Suppl 7:77-85. doi: 10.1111/epi.12313.

Abstract

Purpose: The aim of this study was to evaluate the long-term cognitive outcome in children with continuous spikes and waves during slow wave sleep (CSWS syndrome).

Methods: We reviewed the neuropsychological tests of 25 children diagnosed with CSWS between 1987 and 2010 and with a mean follow-up of 13.5 years.

Key findings: Cognitive performances worsened during CSWS in virtually all patients. Seven patients (28%) with nonlesional epilepsy had a positive outcome; three patients (12%) showed persistence of motor deficit without involvement of cognitive functions; and seven patients (28%) who presented a long duration of CSWS (mean = 28.1 months) had a negative cognitive outcome. In 6 patients (24%) with structural or metabolic disorders before CSWS onset cognitive outcomes did not change; 2 patients (8%) had a negative outcome irrespective of the duration or presence of other neurologic disorders before CSWS onset. Forty-four percent of children with CSWS demonstrated permanent cognitive impairment.

Significance: The long-term outcome of CSWS syndrome is variable and seems to depend on treatment response, disease duration, and underlying etiology.

Keywords: CSWS; ESES; Epilepsy; Neuropsychological evaluation; Outcome.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Cognition Disorders / diagnosis
  • Cognition Disorders / epidemiology
  • Cognition Disorders / psychology*
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Neuropsychological Tests*
  • Prognosis
  • Sleep Wake Disorders / diagnosis
  • Sleep Wake Disorders / epidemiology
  • Sleep Wake Disorders / psychology*
  • Status Epilepticus / diagnosis
  • Status Epilepticus / epidemiology
  • Status Epilepticus / psychology*
  • Time Factors
  • Treatment Outcome
  • Young Adult