Abstracts

Rationale: Report on outcomes of three patients who underwent focal cortical resection for Electrical Status Epilepticus in Sleep (ESES). Methods: Through a comprehensive search of the Medical Index and EEG reports, all patients with ESES seen at the Mayo Clinic from 2007-2009 were identified. Their charts were reviewed to identify patients who underwent focal cortical resection and the outcome thereof. Results: Three patients were identified. Patient #1 had a history of perinatal intraventricular hemorrhage, shunted hydrocephalus, right hemiparesis and global delay. She developed focal and secondarily generalized epilepsy at age 2 years. Overnight EEG revealed ESES with multifocal discharges maximal over the parieto-occipital regions. Regression occurred at age 5 years and she was diagnosed with Continuous Spike Wave in Sleep Syndrome (CSWS). She did not respond to numerous medication trials and deep brain stimulation. She also had seizures recorded from the left frontotemporal region. She underwent left temporal lobectomy at age 7. She continues to take levetiracetam and diazepam with remission of seizures, but she is still in ESES. Patient #2 had a history of perinatal infarct, hydrocephalus, VP shunt, right mesial temporal sclerosis, and global developmental delay. He developed focal and secondarily generalized epilepsy at age 2 years arising from the right mesial temporal lobe. Developmental regression occurred at age 10 years. Overnight EEG revealed ESES with generalized and focal right frontal discharges and he was diagnosed with CSWS. He did not respond to diazepam. He underwent right temporal lobectomy at age 11 with remission of ESES. He is on lamotrigine with rare breakthrough seizures. Patient #3 had no prior epilepsy risk factors. She developed focal epilepsy including focal status epilepticus at age 3 years arising from the right temporal region. Developmental regression occurred at age 4 years. Overnight EEG revealed ESES with generalized discharges maximal over the right temporal region and she was diagnosed with Landau Kleffner Syndrome (LKS). She did not respond to multiple medical therapies. SISCOM and intracranial monitoring revealed right temporal ictal focus. She underwent right parietal resection at age 6. She is currently in remission of ESES and seizures on levetiracetam. Conclusions: CSWS and LKS are rare but treatable seizure syndromes of childhood characterized by developmental regression and electrical status epilepticus in slow wave sleep (ESES). At present, the pathophysiology of ESES is unknown. However, in some cases, ESES may be caused by a focal cortical lesion. Focal cortical resection should be considered in CSWS or LKS patients with focal seizures of known or unknown etiology who have not responded to medications.