This information is for people affected by or concerned about Landau Kleffner syndrome.
On this page we talk about how Landau Kleffner syndrome is diagnosed, what causes it, symptoms, treatment options and long-term outlook.
Use this page as a general guide and speak to a health professional for more information and support.
On this page
What is Landau Kleffner syndrome?
Landau Kleffner syndrome is a very rare type of epilepsy, that is thought to affect about one child in every one million children. However, it is hard to know for sure how many people actually have Landau Kleffner syndrome, as the research and records for it are not very good. But we do know that it usually occurs in children between the ages of three and eight years. It also affects more boys than girls.
Landau Kleffner syndrome affects the temporal lobe – the area of the brain that controls speech and understanding. Because of this, children who have this type of epilepsy usually develop problems with their speech and language.
What causes Landau Kleffner syndrome?
We do not know for sure what causes Landau Kleffner syndrome. More research is needed to fully understand the causes. However, it is thought that it may be linked to differences in a few genes, including GRIN2A.
Some research has also shown that autoimmune responses – when the body’s defences mistakenly attack the body’s own healthy cells – might also play a part in causing Landau Kleffner syndrome.
What are the signs and symptoms of Landau Kleffner syndrome?
The main symptoms of Landau Kleffner syndrome are seizures and loss of language skills. We explain both of these in more detail below.
Children with Landau Kleffner syndrome may also experience changes in their behaviour, including their ability to socially interact with others and their general learning and development.
Seizures
Most children with Landau Kleffner syndrome will have seizures, which usually happen at night. Seizures usually start before the child begins to lose language skills and can include:
- Focal onset seizures – these come from one lobe (part) of the brain, and what happens during the seizure depends on which lobe is affected.
- Prolonged absence seizures – these can occur many times a day and are a brief ‘trance-like’ state in which the child stares blankly into space and becomes unresponsive.
- Atonic seizures (also known as drop seizures) – during these seizures, muscle tone is lost, causing the child to flop and fall to the ground.
Find out more about epilepsy seizure types.
Most children with Landau Kleffner syndrome will develop normal language skills at first, but over time they may lose the ability to understand language or use speech. This loss of skills can happen gradually or suddenly.
At first, some children are mistakenly thought to have a hearing problem. This is because it may seem like they cannot recognise different sounds.
Some children with Landau Kleffner syndrome may experience behaviour problems. These types of behavioural problems may include:
- Attention deficit hyperactivity disorder (ADHD)
- Focusing or concentrating for shorter amounts of time
- Being irritable
- Having regular tantrums
- Finding it difficult to socialise with other people.
How is Landau Kleffner syndrome diagnosed?
If you think a child has epilepsy, including Landau Kleffner syndrome, contact a doctor. Children and young people may be referred to a paediatrician (a doctor who specialises in child health). Or they may be referred to a doctor who specialises in diagnosing and treating rare types of epilepsy, such as Landau Kleffner syndrome.
Landau Kleffner syndrome is usually diagnosed using a combination of the following:
- The child’s medical history, including their normal early development followed by any loss of language skills, seizures and changes in behaviour.
- An EEG (electroencephalogram) test – a painless test that uses small sensors attached to the scalp to pick up the electrical signals produced by the brain. The EEG results will usually show abnormalities in the area of the brain known to be important for language. These abnormalities are more obvious during sleep.
Treatment for Landau Kleffner syndrome
It is important for people with epilepsy with Landau Kleffner syndrome to be treated quickly with the most effective medicines.
Remember that everyone is different and what works for one person might not work well for someone else. People with this type of epilepsy may have to try a few different medicines until something works well.
Talk to a doctor about treatment options and what might be suitable. They should explain the risks and benefits of each treatment, including any possible side effects.
Anti-seizure medications
The anti-seizure medications commonly used to treat the seizures are:
- Sodium valproate (also known as Epilim) – a medicine that comes as capsules, tablets or a liquid that you swallow. It also comes as granules that you mix with food or drink. It can also be given by injection, but this is usually only done in hospital or in emergencies. But sodium valproate may not be suitable for everyone. Read more about the process for prescribing sodium valproate below. [Link to box below]
- Ethosuximide (also known as Zarontin) – a medicine that comes as a capsule or solution, that you swallow.
- Benzodiazepines such as clobazam (also known as Frisium) – a medicine that comes as tablets or liquid, that you swallow.
Your child’s doctor may also suggest trying steroids. Their doctor will talk to you about steroids, how to take them, and any possible side effects.
Sodium valproate
In January 2024, new rules were introduced for prescribing sodium valproate.
Two specialists are now needed to approve:
- New or ongoing valproate treatments for girls and women.
- New valproate treatments for boys and men
The specialists must agree that your epilepsy does not respond to other treatments and the benefits of treatment outweigh the risks.
For girls and women, valproate can seriously harm an unborn baby when taken during pregnancy.
For boys and men, valproate may cause infertility. Some people’s fertility may return when their treatment is stopped or reduced. There is also some evidence that valproate can cause harm in the testicles of animals, but it’s unclear what this means for humans.
Never stop taking valproate before talking to your specialist first. Not taking medication as prescribed can increase the risk of a seizure.
Your epilepsy specialist will let you know when your treatment is due to be reviewed.
If you are planning a pregnancy it is important to speak to your healthcare professional as soon as possible about your treatment options.
If you have any questions or concerns about valproate, please speak to your healthcare professional.
Find out more about sodium valproate in the MHRA’s patient guide.
Find out more about Anti-seizure medications, contraception and pregnancy.
Speech and language therapy
Children who have Landau Kleffner syndrome may be referred for speech and language therapy, with regular assessment and monitoring. This is an important part of the management of Landau Kleffner syndrome.
Children with this syndrome are also likely to need additional support in education.
What’s the long-term outlook for people with Landau Kleffner syndrome?
During the active phase of Landau Kleffner syndrome – when children lose language skills – they may have repeated episodes of worsening symptoms (regression) and then recovery. Because of this, children with Landau Kleffner syndrome may need long-term support to help them manage this type of epilepsy.
However, seizures can usually be controlled well with anti-seizure medications and any EEG abnormalities may disappear. Although some children will make a good recovery, many other children with Landau Kleffner syndrome may experience long-term effects on their development, learning and speech.
Living with Landau Kleffner syndrome and getting support
Having a child with any form of epilepsy can be challenging and upsetting. But if the type of epilepsy they have is rare, it can be even harder to manage and come to terms with. Talk to the child’s doctor for help on how to support them well. And talk to your own doctor if you need support for yourself. If you’re caring for someone with Landau Kleffner syndrome, it’s important that you look after yourself and get the support you need.
You may find it helpful joining an in-person or online support group, where you can listen to or share experiences with other people who understand what you’re going through.
Other useful organisations
UK Rare Epilepsies Together
Patient advocacy groups representing rare and complex epilepsies
