Information on Landau-Kleffner Syndrome

Landau-Kleffner syndrome (LKS) is a infancy disorder. Landau-Kleffner syndrome is characterized by the sudden or slow development of aphasia (the inability to understand or express language) and an abnormal electroencephalogram (EEG). The cause of Landau-Kleffner Syndrome is dysfunctional immune system, exposure to a virus, and brain trauma. LKS affects the parts of the brain that control comprehension and speech. The disorder usually occurs in children between the ages of 5 and 7 years.

Just about 80 percent of the child with LKS has one or extra epileptic seizures that generally happen at night. The syndrome affects both males and females. In addition, as many as 80 percent of infant with Landau-Kleffner syndrome have behavioral problems, such as hyperactivity or autistic-like behaviors. LKS may also be called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder. For no apparent reason, these children begin having trouble understanding what is said to them. Children may also appear to be autistic or developmentally delayed.

The inability to understand language ultimately affects the child's spoken language which may progress to a complete loss of the skill to speak. Infants who have learned to read and write before the start of auditory agnosia can frequently continue communicating through written language. At several time, 80 percent of children with LKS have one or more seizures. The seizures usually end by the time the child becomes a teenager. Most children outgrow the seizures, and electrical brain activity on the EEG usually returns to normal by age 15. All LKS children have abnormal electrical brain activity on both the right and left sides of their brains.

People with Landau-Kleffner Syndrome have abnormal EEG patterns in the temporal lobe (located on the sides of the brain) and in the temporo-parieto-occipital regions during sleep. Treatment for Landau-Kleffner syndrome usually consists of medications, such as anticonvulsants and corticosteroids, and speech therapy, which should be started early. High dose diazepam given at bedtime has resulted in excellent improvement in some children. A controversial treatment option involves a surgical technique called multiple subpial transections in which the pathways of abnormal electrical brain activity are severed.