A Review of Children with Electrical Status Epilepticus in Slow Wave Sleep (ESES) (P04.174)

Abstract

Objective: To determine characteristics, treatment and outcome of children with electrical status epilepticus in slow wave sleep (ESES). Background ESES is a rare EEG finding associated with Landau Kleffner syndrome (LKS) and Continuous Spike Wave in Sleep syndrome (CSWS). These syndromes are potentially treatable causes of developmental regression. Design/Methods: All patients with ESES seen at Mayo Clinic 2007-2011 were retrospectively identified. All patients underwent detailed evaluation, neurological examination, and prolonged EEG monitoring during evaluation and follow up. Medical records, EEGs, and neuroimaging were reviewed to determine clinical presentation, treatment, and outcome. Results: 30 patients were identified. Average age at onset of regression was 5.4 years (range 13 months to 11 years). 6/30 patients met criteria for LKS. 24/30 patients met criteria for CSWS. Etiologies included unknown cause (14/30), symptomatic (16/30). Specific etiologies included thalamic injury (3), hydrocephalus (4), cortical dysplasia (3). EEG abnormalities in LKS were maximally present over the frontotemporal head regions; in CSWS, EEG abnormalities were more diffuse. Presenting symptoms included: regression of language, regression of motor skills, frontal lobe syndrome (inattention, impulsivity, difficulty with planning and sequencing activities), behavioral difficulties, autistic features, and decreased level of consciousness. 26/30 patients underwent treatment with followup at Mayo Clinic. Successful therapies include diazepam, corticosteroids, IVIG, ethosuximide, zonisamide, focal parietal resection, temporal lobectomy, although relapses did occur. Unsuccessful treatments include valproic acid, lamotrigine, levetiracetam, sulthiame, felbamate, deep brain stimulation, mycophenolate mofetil. 24/26 patients responded to treatment. 6/24 patients relapsed. 17/26 are currently in remission. Follow up time was 1.7 patient years. Conclusions: ESES is a rare but treatable cause of childhood developmental regression. It is important to screen children with seizures and regression for ESES as this is a treatable conditon. Effective treatments may include diazepam, prednisone, IVIG, ethosuximide, zonisamide, focal epilepsy surgery. Relapse is common, but terminal remission is possible. Disclosure: Dr. Martyanov has nothing to disclose. Dr. Wirrell has received research support from Mayo Foundation. Dr. Nickels has nothing to disclose.