Landau-Kleffner Syndrome

Abstract

In 1957, Landau and Kleffner described six children with symptoms, which they termed “acquired aphasia with convulsive disorder in children”. Since then, the syndrome (Landau-Kleffner Syndrome,-LKS) has been described in over 140 cases. The main problem of LKS is made up of three parts; an acquired aphasia (verbal auditory agnosia), paroxysmal electroencephalographic abnormalities with epileptic seizures, and behavioral disorders. The course and prognosis of LKS have been studied in the long term by Mantovani and Landau, Bishop, Holmes and Beamanoir et al. A review of their literature of LKS suggests the following characteristics; the association of acquired aphasia with EEG abnormalities (multifocal spikes and spike and wave discharges) is mentioned in all cases, but seizures occur in about 70 per cent of patients. Beaumanoir reports that aphasia commonly starts (except in less than 1/10) with an auditory verbal agnosia. The first symptoms appeared between the ages 2 to 11 years, with the peak of appearance around 3 years to 5 years of age. Seizures are mostly well controlled by antiepileptic therapy. In LKS, the most important problem is a therapeutic-education for speech disorder (verbal auditory agnosia), behavioral disorders and anosognosia. We emphasize the facilitation of psycho-motor development, and the education by sign language, lip (or speech) reading and reading and writing, i. e., a multi-sensory approach. © 1989, The Japan Society of Logopedics and Phoniatrics. All rights reserved.