Landau-Kleffner Syndrome

Landau-Kleffner Syndrome 0

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What is Landau-Kleffner Syndrome?

Landau-Kleffner Syndrome (LKS) is a neurological disease that impacts the part of the brain that processes language comprehension and speech. It typically affects children between the ages of 3 and 7. Patients develop normally but then lose their ability to understand and use language.

Often, LKS is related to or accompanied by seizures and partial seizures, many of which occur during sleep.

Initially, LKS is often diagnosed as autism, hearing loss or verbal processing disorder. The disease is usually properly diagnosed via an electroencephalogram (EEG) that shows abnormal brain wave patterns.

What are the Symptoms of Landau-Kleffner Syndrome?

Children with Landau-Kleffner Syndrome forget the meanings of words that they once knew. Some sufferers lose the ability to speak normally, and some have seizures.

Landau-Kleffner Syndrome Causes

Landau-Kleffner syndrome is likely caused by changes or mutations within the GRIN2A gene. This gene is responsible for sending directions to the body and telling it to produce a protein that signals the neurons (nerve cells) within the brain. Ability to express or understand language can be affected when the signals are not working properly.

The brain may also turn on certain receptors, which can conclude with seizures. It is suspected that seizures associated with LKS could damage the portions of the brain that control speech. Other gene mutations that could be linked to LKS are BSN, EPHB2, and RELN genes. Environmental impacts on these genes may also contribute to the cause. Rare cases could be the body attacking itself due to an autoimmune illness.

How is Landau-Kleffner Syndrome Treated?

If a patient has seizures, those must be managed through medication. Other drugs, including diazepam, can help with the language issues. Some medical professionals also prescribe steroids to help reduce any inflammation in the brain.

Some experimental surgeries have resulted in improvements for LKS patients, but no surgery for the condition is considered mainstream.

Many children recover from LKS and recover or re-learn their language skills, especially when speech therapy and other cognitive therapies are started right away. Some have permanent issues with understanding and using language.

Landau-Kleffner Syndrome Prevention

As Landau-Kleffner Syndrome is suspected to be caused by a mutation within a gene, the diagnosis is likely hereditary. Genetic counseling should take place when a couple is looking to become pregnant and has Landau-Kleffner Syndrome. However, as LKS is not yet determined to have a single hereditary cause, not all genetic testing centers will test for LKS.

Major symptoms and side effects of LKS (including seizures) can be prevented with the help of medication. Symptoms usually do not last beyond adulthood. Seizures and speech can be improved with immunotherapy. Speech therapy aids in improving linguistic abilities, however, potential loss of complete linguistic ability cannot be prevented. Therapy can help children improve their language and prevent permanent impacts of the diagnosis after months or years in extreme cases.

Permanent impacts have a better chance of being prevented if symptoms are addressed as soon as possible. Speech therapy needs to be started right away for kids to have a better chance of relearning their speech. Learning sign language or special education are alternatives for those who do not want to resort to surgery. Complete seizure prevention can occur with a multiple subpial transection surgery. This option is only for rare cases that were not aided with healing alternatives.

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Last Reviewed:
October 09, 2016
Last Updated:
March 12, 2018