Landau Kleffner Syndrome is a rare form of childhood epilepsy which results in a severe language disorder. All children with LKS have abnormal electrical activity in one, sometimes both temporal lobes, the area of the brain responsible among other functions for processing language.
The language disorder in most children affects comprehension or understanding. Many LKS children become unable to understand their own name. They are also likely to have difficulty recognizing environmental sounds such as the telephone ringing. These children may appear
to be deaf. Expressive language - the ability to speak is often seriously affected; some children lose their speech completely. Rarely, a child may be able to understand language, but have difficulty with speaking.
Behavioral problems are common, especially hyper-activity, poor attention, depression and irritability. Some children have episodes of very abnormal "autistic type behavior" with symptoms such as avoidance of contact with family and friends (avoidance of eye contact is common) extreme pickiness over food, very disturbed sleep, attacks of rage and aggression, insensitivity to pain, bizarre and inappropriate and repetitive play.
There may be temporary other neurological problems such as loss of bladder and bowel control, episodes of visual disturbances may occur, the child can see but does not understand what he sees. He may have difficulty recognizing family and friends or common objects, such as food and clothes.
* onset occurs at the time of language acquisition
* major symptom is failure of speech development
* seizures occur in 75% but are infrequent and get less with time
* generalized seizures
* multifocal EEG spikes
* etiology unknown at present
* no imaging abnormalities
* seizures are generally controllable and abate with time but the outcome for speech is less good
* treatment with steroids and anti-epileptic drugs is presently undertaken
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