Landau-Kleffner Syndrome

 Landau-Kleffner Syndrome

Overview

Landau-Kleffner syndrome is a condition that affects your brain by causing seizures and a loss or decline in speech and language skills. Children with LKS have abnormal electrical signals in their brains that affect their ability to understand language and speak.

LKS typically affects children, starting when they’re between 3 and 8 years old. But children as young as 2, as well as adolescents, can have the condition. Boys are more likely to have LKS than girls.

If your child has LKS, they may lose their ability to speak and understand language (aphasia). This can happen rapidly all at once, or slowly. Many children with LKS — about 70% — also have seizures, often during sleep.

Symptoms

Not every child with LKS has the same symptoms. If your child has LKS, it may seem like they can’t hear you. They may have a hard time understanding what you and others say to them. They may also have new developmental delays, intellectual differences or learning disabilities. Sometimes, these language difficulties can make your child to feel frustrated and cause behavioral symptoms such as agitation, not paying attention, anger outbursts or acting out.

Children with LKS may be:

Unable to speak.

Likely to have seizures.

More likely to have behavioral disorders (such as attention-deficit hyperactivity disorder).

Causes

No cause can be found in the majority of children with LKS. About 20% of children with LKS have changes (mutations) in their GRIN2A gene, though. Children inherit this mutation from one of their parents. But not everyone with this genetic change develops LKS. Some children with LKS have mutations in other genes. They might be the first person in their family to have the mutation, known as a de novo mutation.

Researchers think these gene mutations cause electrical signals in their brains to misfire. These abnormal signals can cause LKS symptoms.

Researchers are also studying whether there’s a link between LKS and the immune system. An autoimmune response in which the immune system attacks the body’s cells may contribute to LKS.

Diagnosis

Children with LKS usually meet typical child development milestones before LKS begins. You may notice your child has trouble speaking. Or your child’s healthcare provider may notice symptoms during a well-child visit.

LKS is challenging to diagnose. Tell your healthcare provider about any behavioral changes you’ve noticed in your child.

Your child’s healthcare provider may recommend an electroencephalogram (EEG) to measure electrical impulses in your child’s brain. This painless test lets their healthcare provider check your child’s brain activity.

Healthcare providers may do this test while your child is sleeping and again when they’re awake. Typically, your child’s brain waves slow down as they enter the early stages of sleep. When they enter the rapid eye movement (REM) sleep phase, their brain waves become more active. But EEGs in children with LKS often show continuous levels of abnormal brain wave activity during all sleep phases.

Your child’s healthcare provider may also recommend:

Audiometry (hearing test).

MRI of their brain (to rule out a brain tumor).

Evaluation by a psychologist.

Treatment

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.

Rarely, surgeons perform multiple subpial transections. It isn’t conclusive whether this brain surgery has proven benefits. Benefits and risks should be carefully considered after a thorough pre-surgical evaluation.

There’s no one right way to treat LKS. Your child may improve with speech therapy and other treatments. But in some cases, they may not respond to treatment. Your healthcare provider will check your child’s progress and adjust their treatment as necessary.

Type of Doctor Department :Epileptologists