Landau-Kleffner syndrome evolving to electrical status epilepticus: a case report illustrating clinical heterogeniety

Abstract

A child with Landau-Kleffner syndrome who changed to continuous spike wave discharges both during sleep as well as awake is reported in this communication. A 4.5-year-old boy developed rapidly progressive mixed aphasia with relative preservation of writing and drawing skills. Electroencephalogram showed 2-2.5 Hz spike wave discharges more marked during slow wave sleep. Single photon emission tomography revealed hypoperfusion of the left frontotemporoparietal area. The patient did not respond to methylprednisolone, intravenous immunoglobulin and a number of antiepileptic drugs. At 2 year follow up there was no clinical improvement and the EEG showed continuous spike wave discharges not only during sleep but also during waking. He was aphasic but had preservation of spatial intelligence. This case report highlights the severe and resistant form of Landau-Kleffner syndrome and the heterogeniety of the condition. (J Pediatr Neurol 2004; 2(3): 157-160).