Abstracts

To better define the difference between Landau-Kleffner Syndrome (LKS) and Malignant Rolandic Epilepsy (MRE). We retrospectively studied our patients who presented with centrotemporal spikes and electrical status epilepticus during slow wave sleep. We analyzed their clinical courses, electroencephalogram (EEG), magnetoencephalography (MEG), magnetic resonance imaging (MRI), speech evaluations, neuropsychological evaluations, treatments, and long term outcomes. Eight patients presented with centrotemporal spikes and electrical status epilepticus during slow wave sleep. Six patients developed clinical aphasia, consistent with LKS (3 females, 3 males). Two patients never showed any clinical evidence of aphasia and may be better named as MRE (1 female, 1 male). However, both groups had similar clinical neurophysiology findings (EEG, MEG) and normal MRI. Both had some cognitive and learning problems at the clinical onset. The difference between them were age of onset (LKS- 5.78 yr, MRE -8.67 yr), speech findings (LKS- aphasia, MRE- normal speech), clinical response to steroid (4/5 of LKS- steroid responder, MRE-none) and long term outcome (LKS steroid responder- good outcome, none of MRE improved cognitively). Lamotrigine caused exacerbation of seizures in both MRE patients and produced non-convulsive electrical status epilepticus (NCES) both awake and sleep. We propose that LKS and MRE are biologically different based on age of onset, clinical manifestation, clinical response, and long term outcome. Even though the two groups share the same clinical neurophysiological findings, LKS never develops NCES during awake like the MRE. MRE also does not have any aphasia and steroid treatment may not be indicated.