Clinical Reviews of Landau-Kleffner Syndrome / 대한소아신경학회지
Journal of the Korean Child Neurology Society
; (4): 283-289, 2003.
Article
em Ko
| WPRIM
| ID: wpr-107771
Biblioteca responsável:
WPRO
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.
Palavras-chave
Texto completo:
1
Índice:
WPRIM
Assunto principal:
Afasia de Broca
/
Convulsões
/
Estudos Retrospectivos
/
Síndrome de Landau-Kleffner
/
Dieta Cetogênica
/
Anticonvulsivantes
Tipo de estudo:
Observational_studies
Limite:
Female
/
Humans
/
Male
Idioma:
Ko
Revista:
Journal of the Korean Child Neurology Society
Ano de publicação:
2003
Tipo de documento:
Article