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Children with ESES: variability in the syndrome.
Epilepsy Res. 2006 Aug; 70 Suppl 1:S248-58.ER

Abstract

OBJECTIVE

We undertook a retrospective study of children who present with significant activation of paroxysmal discharges during sleep to examine the clinical spectrum of disorders that present with such an EEG abnormality.

BACKGROUND

Electrical status epilepticus in sleep (ESES) is an electrographic pattern characterized by nearly continuous spike-wave discharges in slow wave sleep, usually with a frequency of 1.5-3 Hz and usually diffuse and bilateral in distribution. A variety of neurocognitive and behavioral problems have been associated with this EEG pattern.

METHODS

We conducted a retrospective review of 1497 EEG records of patients admitted to University of California, Los Angeles (UCLA) for overnight video-EEG monitoring during a 5 year interval. Demographic, clinical and electroencephalographic variables were evaluated.

RESULTS

EEG records for 102 patients meeting criteria were identified. Clinical information was available for 90 of those patients. Eighteen of these patients could be diagnosed with Landau-Kleffner syndrome (LKS). Key findings include: (1) neuroimaging abnormalities were uncommon in our LKS patients; (2) among children who do not fit the specific diagnostic criteria for LKS, a spike-wave index (SWI) >50% was more likely to be associated with global developmental disturbances than SWI < or =50% (p<0.05); (3) Children with generalized discharges were more likely to experience severe or global developmental disturbance than those with focal abnormalities, without reaching statistical significance (p=0.07).

CONCLUSIONS

Severity of ESES can vary over time between and within patients and clinical status does not always directly correlate with SWI. However, the prognosis of LKS is substantially better than CSWS and these two disorders could be classified in a dichotomous manner rather than be seen as two points along a continuum.

Authors+Show Affiliations

Department of Pediatrics and Neurology, David Geffen School of Medicine at UCLA, Mattel Children's Hospital at UCLA, Los Angeles, CA, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

16806829

Citation

Van Hirtum-Das, Michele, et al. "Children With ESES: Variability in the Syndrome." Epilepsy Research, vol. 70 Suppl 1, 2006, pp. S248-58.
Van Hirtum-Das M, Licht EA, Koh S, et al. Children with ESES: variability in the syndrome. Epilepsy Res. 2006;70 Suppl 1:S248-58.
Van Hirtum-Das, M., Licht, E. A., Koh, S., Wu, J. Y., Shields, W. D., & Sankar, R. (2006). Children with ESES: variability in the syndrome. Epilepsy Research, 70 Suppl 1, S248-58.
Van Hirtum-Das M, et al. Children With ESES: Variability in the Syndrome. Epilepsy Res. 2006;70 Suppl 1:S248-58. PubMed PMID: 16806829.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Children with ESES: variability in the syndrome. AU - Van Hirtum-Das,Michele, AU - Licht,Eliot A, AU - Koh,Susan, AU - Wu,Joyce Y, AU - Shields,W Donald, AU - Sankar,Raman, Y1 - 2006/06/23/ PY - 2005/11/30/received PY - 2006/01/12/revised PY - 2006/01/19/accepted PY - 2006/6/30/pubmed PY - 2006/10/27/medline PY - 2006/6/30/entrez SP - S248 EP - 58 JF - Epilepsy research JO - Epilepsy Res. VL - 70 Suppl 1 N2 - OBJECTIVE: We undertook a retrospective study of children who present with significant activation of paroxysmal discharges during sleep to examine the clinical spectrum of disorders that present with such an EEG abnormality. BACKGROUND: Electrical status epilepticus in sleep (ESES) is an electrographic pattern characterized by nearly continuous spike-wave discharges in slow wave sleep, usually with a frequency of 1.5-3 Hz and usually diffuse and bilateral in distribution. A variety of neurocognitive and behavioral problems have been associated with this EEG pattern. METHODS: We conducted a retrospective review of 1497 EEG records of patients admitted to University of California, Los Angeles (UCLA) for overnight video-EEG monitoring during a 5 year interval. Demographic, clinical and electroencephalographic variables were evaluated. RESULTS: EEG records for 102 patients meeting criteria were identified. Clinical information was available for 90 of those patients. Eighteen of these patients could be diagnosed with Landau-Kleffner syndrome (LKS). Key findings include: (1) neuroimaging abnormalities were uncommon in our LKS patients; (2) among children who do not fit the specific diagnostic criteria for LKS, a spike-wave index (SWI) >50% was more likely to be associated with global developmental disturbances than SWI < or =50% (p<0.05); (3) Children with generalized discharges were more likely to experience severe or global developmental disturbance than those with focal abnormalities, without reaching statistical significance (p=0.07). CONCLUSIONS: Severity of ESES can vary over time between and within patients and clinical status does not always directly correlate with SWI. However, the prognosis of LKS is substantially better than CSWS and these two disorders could be classified in a dichotomous manner rather than be seen as two points along a continuum. SN - 0920-1211 UR - https://www.unboundmedicine.com/medline/citation/16806829/Children_with_ESES:_variability_in_the_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0920-1211(06)00203-8 DB - PRIME DP - Unbound Medicine ER -