J Korean Child Neurol Soc.  2003 Nov;11(2):283-289.

Clinical Reviews of Landau-Kleffner Syndrome

• Kang HC
• Lim IS
• Hwang YS
• Park SK
• Kim HD

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.

Keyword

Landau-Kleffner syndrome

MeSH Terms

Anticonvulsants
Aphasia, Broca
Female
Humans
Ketogenic Diet
Landau-Kleffner Syndrome*
Male
Retrospective Studies
Seizures
Anticonvulsants