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Articles » NINDS Landau-Kleffner Syndrome Information Page: NINDS
 

NINDS Landau-Kleffner Syndrome Information Page: NINDS

Article title: NINDS Landau-Kleffner Syndrome Information Page: NINDS

Main condition: Landau-Kleffner Syndrome

Conditions: Landau-Kleffner Syndrome


What is Landau-Kleffner Syndrome?
Landau-Kleffner syndrome (LKS), also called acquired epileptiform aphasia, is a rare, childhood neurological disorder characterized by the sudden or gradual development of aphasia (loss of language) and an abnormal electro-encephalogram (EEG). LKS affects the parts of the brain that control speech and comprehension. The disorder usually occurs in children between the ages of 3 and 7. Typically, these children develop normally and then, for no apparent reason, they lose the ability to understand others and to speak. While many of the affected individuals have seizures, some do not. The disorder is difficult to diagnose and may be misdiagnosed as autism, pervasive developmental disorder, hearing impairment, learning disability, auditory/verbal processing disorder, attention deficit disorder, mental retardation, childhood schizophrenia, or emotional/behavioral problems.

Is there any treatment?
Treatment for LKS usually consists of medications, such as anticonvulsants and corticosteroids, and speech therapy, which should be started early. Another controversial treatment option involves a surgical technique called multiple subpial transection in which the pathways of abnormal electrical brain activity are severed.

What is the prognosis?
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.

What research is being done?
The NINDS supports broad and varied programs of research on epilepsy and developmental disorders. This research is aimed at discovering new ways to prevent, diagnose, and treat epilepsy and developmental disorders and, ultimately, to find cures for them.

Selected references

Deonna, T.
Acquired Epileptiform Aphasia in Children (Landau-Kleffner Syndrome). Journal of Clinical Neurophysiology, 8:3; 288-298 (1991).

Gordon, N.
Acquired Aphasia in Childhood: the Landau-Kleffner Syndrome. Developmental Medicine and Child Neurology, 32:3; 270-274 (1990).

Landau, W.
Landau-Kleffner Syndrome: An Eponymic Badge of Ignorance. Archives of Neurology, 49; 353 (April 1992).

Lerman, P, Lerman-Sagie, T, and Kivity, S.
Effect of Early Corticosteroid Therapy for Landau-Kleffner Syndrome. Developmental Medicine and Child Neurology, 33:3; 257-266 (1991).

Paquier, P, Van Dongen, H, and Loonen, M.
The Landau-Kleffner Syndrome or Acquired Aphasia with Convulsive Disorder. Archives of Neurology, 49; 354-359 (April 1992).

Tharpe, A, Johnson, G, and Glasscock III, M.
Diagnostic and Management Consideration of Acquired Epileptic Aphasia or Landau-Kleffner Syndrome The American Journal of Otology, 13:3, 210-214 (May 1991)

 Organizations

Epilepsy Foundation
4351 Garden City Drive
Suite 500
Landover, MD 20785-7223
postmaster@efa.org
http://www.epilepsyfoundation.org/
Tel: 301-459-3700 800-EFA-1000 (332-1000)
Fax: 301-577-2684

This fact sheet is in the public domain. You may copy it.Provided by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892


 

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