25/11/2022
Landau-Kleffner syndrome (LKS) is a rare brain condition that causes children to lose their ability to speak and understand language. Children with LKS have abnormal brain activity during sleep, and many also experience seizures. Healthcare providers may treat LKS with medication, speech therapy and behavioral therapy.
Cause: the cause of Landau-Kleffner syndrome is unknown although a spectrum of epileptic conditions including LKS has been described in individuals with GRIN2A gene mutations and other candidate genes including RELN, BSN, EPHB2 and NID2 have been suggested.
LKS is a condition that shows up during childhood, causing difficulties with speech and seizures. Children with LKS lose the ability to talk and understand speech. This loss is called aphasia. Many children with LKS also have seizures, episodes of uncontrolled body movement.
Landau-Kleffner Syndrome is a rare form of epilepsy that manifests as a form of aphasia, (loss of language), which usually develops between 3 and 7 years. It is twice as common in males than females and is often diagnosed in conjunction with autism.
LKS is diagnosed when a doctor sees clinical features that are consistent with the syndrome such as a loss of speech and an electroencephalogram (EEG) that shows specific kinds of seizure activity. Genetic testing can be used to confirm if there is a genetic change in GRIN2A, but this testing is not done routinely.
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, remission and relapse may occur.
Healthcare providers use medication, speech therapy and behavioral therapy to treat children with LKS. The earlier speech therapy begins, the better your child’s chance of regaining their language skills. Sometimes, healthcare providers recommend a ketogenic diet for epilepsy.
Your healthcare provider may prescribe:
Anticonvulsant medications to control seizures.
Corticosteroids to reduce immune system response.
References
TEXTBOOKS
Mantovani JF. Developmental regression, autism and epilepsy. In: Shapiro BK, Accardo PJ, eds. Autism Frontiers: Clinical Issues and Innovations. Baltimore, MD: Paul H. Brookes; 2008:49-56.
Mantovani JF. Landau-Kleffner Syndrome. In: The NORD Guide to Rare Disorders, Philadelphia, PA: Lippincott, Williams and Wilkins; 2003:547-8.