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Journal of the Korean Child Neurology Society 2003;11(2):283-289.
Published online November 30, 2003.
Clinical Reviews of Landau-Kleffner Syndrome.
Hoon Chul Kang, In Suk Lim, Yong Soon Hwang, Sang Keun Park, Heung Dong Kim
1Epilepsy Center, Inje University College of Medicine, Sang-gye Paik Hospital, Seoul, Korea.
2Department of Pediatrics, Inje University College of Medicine, Sang-gye Paik Hospital, Seoul, Korea.
3Department of Neurosurgery, Inje University College of Medicine, Sang-gye Paik Hospital, Seoul, Korea.
4Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea. hdkimmd@yumc.yonsei.ac.kr
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.
Key Words: Landau-Kleffner syndrome


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