Landau-Kleffner 증후군의 임상적 고찰

Abstract

Purpose : We intended to describe the clinical features including characteristic etiologies, therapeutic approaches and outcomes for Landau-Kleffner syndrome(LKS).

Methods : A retrospective chart reviews were done to reveal the clinical and electrophysiological features in 5 patients who were diagnosed as LKS and undergone extensive diagnostic work-up and various therapeutic interventions.

Results : Among five LKS patients, 2 patients were males and 3 were females. All patients showed well controlled seizure outcomes but cognitive function including auditory and/or expressive aphasia were not improved in spite of using various anticonvulsants. Only two patients responded to steroid therapy but one patient showed repeated deterioration after discontinuation of the durg. Among 3 patients who were on ketogenic diet(KD), one patient showed a dramatic improvement in liguistic and cognitive functions in spite of underlying mitochondrial complex I deficiency. Multiple subpial transections (MST) were done in the other two patients because of incomplete recovery from ketogenic diet, and only one patient showed fairly successful improvement.

Conclusion : Cognitive regression induced by LKS could be successfully improved by various therapeutic modalities including steroid, KD and palliative MST in most patients.