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Children with ESES: variability in the syndrome
Michele Van Hirtum-Das1, Eliot A Licht, Susan Koh
1Department of Pediatrics and Neurology, David Geffen School of Medicine at UCLA, Mattel Children's Hospital at UCLA, Los Angeles, CA, USA.
Epilepsy Research
|June 30, 2006
Summary
Electrical status epilepticus in sleep (ESES) in children can present with varying severity. While neuroimaging is uncommon in Landau-Kleffner syndrome (LKS), higher spike-wave index and generalized discharges correlate with developmental issues.
Area of Science:
- Pediatric Neurology
- Clinical Neurophysiology
Background:
- Electrical status epilepticus in sleep (ESES) is an EEG pattern with continuous spike-wave discharges during sleep.
- ESES is associated with neurocognitive and behavioral problems in children.
Purpose of the Study:
- To investigate the clinical spectrum of disorders presenting with significant paroxysmal discharges during sleep in children.
- To analyze the relationship between EEG findings and clinical outcomes in pediatric patients with ESES.
Main Methods:
- Retrospective review of 1497 overnight video-EEG monitoring records from UCLA.
- Evaluation of demographic, clinical, and electroencephalographic variables in 102 identified patients.
Main Results:
- 18 of 90 evaluated patients were diagnosed with Landau-Kleffner syndrome (LKS).
- Neuroimaging abnormalities were infrequent in LKS patients.
- A higher spike-wave index (>50%) in non-LKS patients correlated with global developmental disturbances (p<0.05).
- Generalized discharges were associated with severe developmental disturbance, though not statistically significant (p=0.07).
Conclusions:
- ESES severity varies and may not directly correlate with spike-wave index (SWI).
- Landau-Kleffner syndrome (LKS) has a better prognosis than Continuous Spike-Wave Status Epilepticus (CSWS).
- LKS and CSWS may represent distinct disorders rather than a continuum.