[Source: The Irish Medical Times]

Landau-Kleffner syndrome (LKS) is an acquired epileptic aphasia of childhood and is a rare, childhood neurological syndrome. It is accompanied by abnormal electroencephalogram (EEG) and behaviour symptoms of autism.

LKS may also be called infantile ‘acquired aphasia’, ‘acquired epileptic aphasia’ or ‘acquired aphasia with convulsive disorder’, but Rapin et al (1977) called it ‘auditory verbal agnosia’. It was first described by Landau and Kleffner, who identified six children with LKS (1957).

Auditory verbal agnosia or ‘pure word deafness’ is involved — in other words, a disturbance in comprehension of spoken language in the presence of otherwise intact auditory functioning and, essentially, normal performance in other language modalities.

Aphasia itself is a defect or loss of the power of expression by speech, writing or signs, or a defect or loss of the power of comprehension of spoken or written language (the Greek word for ‘speechless’ is aphatos).

In terms of incidence, LKS usually starts before the age of six years and affects twice as many boys as girls (Beaumanoir et al, 1985). More than 198 cases have been reported from 1957 to 1992 (Beaumanoir, 1992).

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