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Landau-Kleffner syndrome

What are the symptoms?

Although characteristically the children lose language understanding (auditory agnosia) which limits speech, a wide range of additional impairments are seen. These include problems with social communication such as seen in Autism Spectrum disorders, some global reduction in learning abilities, and in more than half, difficulties with co-ordination of walking, feeding and hand function. The lack of response to speech, and even to familiar sounds in some cases, may suggest hearing loss but audiological testing is normal. Many of the children have behaviour problems including impulsivity, hyperactivity, distractibility and challenging behaviour.

Three quarters have epileptic seizures. These are commonly partial motor seizures, or accompanied by loss of responsiveness but a significant minority do not have obvious seizures. All, however, show epileptic discharges on electroencephalogram (EEG) in the central region of the brain with a characteristic increase in the rate of discharges in sleep which are often continuous (non-convulsive status). Magnetic resonance imaging (MRI) scans are usually normal.

This situation of loss of skills associated with a high rate of subclinical epileptiform activity (resembling epilepsy but not detectable by the usual clinical tests) is an example of an epileptic encephalopathy (brain disease) in which it is assumed that the epileptic activity stops normal functioning of that part of the brain. It is, however, a poorly understood phenomenon.

View Background Background  |  How is it treated? View How is it treated?

Medical text written November 2004 by Professor B Neville, Professor of Paediatric Neurology, Institute of Child Health, London, UK.

 

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