Landau-Kleffner syndrome

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Acquired aphasia with epilepsy (Landau-Kleffner syndrome)

In this disorder, a child whose language has so far developed normally loses both receptive and expressive language but retains general intelligence. There are associated EEG abnormalities, nearly always bilateral and temporal, and often with more widespread disturbances. Most of the affected children develop seizures either before or after the change in expressive language. The disorder starts usually between 3 and 9 years of age. In most cases the loss of language occurs over several months but it may be more .rapid. In the early stages the severity of the impairment may fluctuate.

Causes of Landau-Kleffner syndrome

The cause is unknown but an inflammatory encephalitis has been proposed. The prognosis is variable: about two-thirds are left with a receptive language deficit, but the other third recover completely.

Assessment of Landau-Kleffner syndrome

Early investigation is essential both to determine the nature and severity of the speech and language disorder and to exclude mental retardation, deafness, cerebral palsy, and pervasive developmental disorder. The speech-producing organs should be examined. It is particularly important to detect deafness at an early stage. Parents can give some indication of the child's speech and language skills, especially if they complete a standardized inventory. With younger children it may be necessary to rely on this information, but children from about the age of 3 years can be tested by a standard test of language appropriate to the child's age. If possible, such a test should be carried out by a speech therapist or a psychologist specializing in the subject.

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