Overview of Landau–Kleffner syndrome: early treatment, tailored education and therapy improve outcome

Abstract

<h2>Abstract</h2> Landau–Kleffner syndrome (LKS) is a rare epilepsy occurring in children usually between the ages of three and nine years, characterised by dramatic loss of language skills in association with epileptiform activity over the language centres during sleep. It may be associated with convulsive seizures and additional difficulties with behaviour, social interaction, motor skills and cognition. Early treatment to suppress the EEG abnormalities can at least partially reverse this regression in 60–70% but usually needs to be continued for some years. Management focuses on restoring communication so that the child continues to engage and learn. Therapy and education support are essential and need to be very flexible as the child's profile can fluctuate dramatically and may include signing when they cannot access language and may require considerable opportunities for consolidation and overlearning. The epileptiform activity usually resolves by puberty, often with continuing recovery in skills for some years that will benefit from ongoing support. Many have persisting deficits as adults which are more severe with earlier age of onset and a longer epileptiform period. It is important to recognise LKS as early multi-disciplinary management may improve outcomes.