MULTILOBAR POLYMICROGYRIA, INTRACTABLE DROP ATTACK SEIZURES, AND SLEEP-RELATED ELECTRICAL STATUS EPILEPTICUS

Background and Objective: Patients with cortical malformations often h ave intractable seizures and are candidates for epilepsy surgery. With in an unselected series of patients with various forms of cortical mal formation, nine patients with multilobar polymicrogyria had electrical status epilepticus during sleep (ESES) accompanied by infrequent foca l motor seizures. Eight patients also had intractable atonic drop atta ck seizures. Because ESES usually is accompanied by a good long-term s eizure prognosis, the objective of this study was to examine ESES outc ome among patients with a structural lesion that is usually highly epi leptogenic and has a low seizure remission trend. Methods: The nine pa tients had follow-up periods lasting 4 to 19 years. All underwent brai n MRI: serial sleep EEG recordings, and cognitive testing during and a fter ESES. Results: ESES and drop attack seizures appeared between the ages of 2 and 5 gears (mean, 4 years) and ceased between the ages of 5 and 12 years (mean, 8 years). At the last visit patients were 8 to 2 3 years of age (mean, 14.5 years) and were either seizure free or had very infrequent focal motor seizures during sleep. Three patients were free from antiepileptic drugs. In no patient was definite cognitive d eterioration apparent after ESES in comparison with earlier evaluation s. Conclusions: Age-related secondary bilateral synchrony underlying E SES may be facilitated in multilobar polymicrogyria, The good seizure outcome contrasts with that usually found in the presence of cortical malformations. For children with polymicrogyria and drop attack seizur es, surgical treatment of the epilepsy should be considered cautiously , and sleep EEG recordings should be performed systematically.