Clinical Reviews of Landau-Kleffner Syndrome.
- Author:
Hoon Chul KANG
1
;
In Suk LIM
;
Yong Soon HWANG
;
Sang Keun PARK
;
Heung Dong KIM
Author Information
1. Epilepsy Center, Inje University College of Medicine, Sang-gye Paik Hospital, Seoul, Korea.
- Publication Type:Original Article
- Keywords:
Landau-Kleffner syndrome
- MeSH:
Anticonvulsants;
Aphasia, Broca;
Female;
Humans;
Ketogenic Diet;
Landau-Kleffner Syndrome*;
Male;
Retrospective Studies;
Seizures
- From:
Journal of the Korean Child Neurology Society
2003;11(2):283-289
- CountryRepublic of Korea
- Language:Korean
-
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.
