Landau-Kleffner Syndrome: A Rare Childhood Language Disorder

Imagine waking up one morning and no longer being able to understand or properly communicate through speech. You can hear just fine, but you can no longer understand what other people are saying, and you can't express yourself in words either! Landau-Kleffner Syndrome is a rare childhood disorder in which children, ages three through nine, lose their ability to understand and use speech.

Although it is not yet understood exactly how the syndrome works, it is known that all children with Landau-Kleffner (LKS) experience some seizure activity in their sleep involving both hemispheres of the brain. It is believed that this seizure activity destroys the parts of the brain used for speech communication and damages the language centers and some other areas. Most children experience other symptoms as well including hyperactivity, depression, and loss of some control of the facial muscles. Seizures are usually limited to sleep but occasionally children experience seizures while awake also.

Parents, educators, and health care professionals often think initially that children with LKS are just goofing off as they don't follow directions or talk weirdly. This leads to children trying to hide the problems, thinking that it is something that will just fix itself. When the regression of language skills is well established, it is often thought that the child has lost his ability to hear. Diagnosis is difficult as children with Landau-Kleffner Syndrome are not able to explain what is wrong or list their symptoms.

There is currently no cure for Landau-Kleffner Syndrome. Anticonvulsants are prescribed for the seizures, and speech therapy as well as sign language instruction are given to help limit language deterioration. Some children respond well to sign language instruction, and those who acquire LKS after becoming fluent in reading and writing are still frequently able to use those means for communication. Some children have their language centers more severely damaged and make less progress in any language areas.

There is also a controversial experimental treatment for LKS involving making tiny incisions over the surface of the brain area where the seizures originate. Theoretically this keeps the seizure activity from spreading to other areas of the brain. This procedure is called multiple subpial transaction.

The seizure activity of Landau-Kleffner Syndrome clears up on its own during puberty. After this, the prognosis varies with each individual. Some patients are able to relearn speech communication and make a total recovery. Most have some degree of language difficulty for life which can vary from no verbal language at all to fluent verbal expressive language but the inability to follow verbal directions.