Landau-Kleffner syndrome
Overview
Landau-Kleffner syndrome (LKS) is a childhood disorder. A major feature of LKS is the gradual or sudden loss of the ability to understand and use spoken language. All children with LKS have abnormal electrical brain waves that can be documented by an electroencephalogram (EEG), a recording of the electric activity of the brain. Approximately 80 percent of the children with LKS have one or more epileptic seizures that usually occur at night. Behavioral disorders such as hyperactivity, aggressiveness and depression can also accompany this disorder. LKS may also be called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder. This syndrome was first described in 1957 by Dr. William M. Landau and Dr. Frank R. Kleffner, who identified six children with the disorder.
Symptoms
* Aphasia * Abnormal electro-encephalogram (EEG) * Seizures * Comprehension difficulties * Hyperkinesia * Attention disorders * Behavioral changes * Aphasia
Causes
The cause of LKS is unknown. Some experts think there is more than one cause for this disorder. All of the children with LKS appear to be perfectly normal until their first seizure or the start of language problems. There have been no reports of children who have a family history of LKS. Therefore, LKS is not likely to be an inherited disorder.
Prognosis
Prognosis of Landau-Kleffner Syndrome: some cases may spontaneously improve Prognosis for Landau-Kleffner Syndrome: The prognosis for children with LKS varies. Some affected children may have a permanent severe language disorder, while others may regain much of their language abilities (although it may take months or years). In some cases, remissions and relapse may occur. The prognosis is improved when the onset of the disorder is after age 6 and when speech therapy is started early. Seizures generally disappear by adulthood. (Source: excerpt from NINDS Landau-Kleffner Syndrome Information Page: NINDS)
Treatment
Medication to control the seizures and abnormal brain wave activity (anticonvulsants) usually has very little effect on language ability. Corticosteroid therapy has improved the language ability of some children. Sign language instruction has benefited others.