A rare brain disorder in which children lose their ability to speak and understand language is called Landau-Kleffner syndrome (LKS). Sometimes, it is called acquired aphasia with epilepsy by doctors. Those who develop this condition often have abnormal electrical activity in the brain and seizures. The most common treatments used in children with LKS are medicines, speech therapy, and behavioral therapy.
Most of the time, the symptoms appear between the ages of 3 and 8, but they can appear at any age (including into the teen years). Recently, healthcare providers have updated the way they group and name certain epilepsy-related disorders. Thus, LKS is considered a specific type of a broader category called epileptic encephalopathy with spike-wave activation in sleep (EE-SWAS), which means it belongs to a group of disorders that involve abnormal brain wave activity during sleep and speech problems.
What Are The Symptoms of LKS?
Most children with LKS develop aphasia (loss of the ability to speak) and auditory agnosia (inability to understand language). These symptoms may happen suddenly or slowly over time. Most of them also experience seizures, often when they are sleeping. While children with this condition may not be able to tell you about their symptoms due to aphasia, you may notice the following symptoms if your child develops LKS. Examples include:
- Frustration
- Inability to pay attention
- Angry or unusually upset
- Agitated
- Restless
If any of the previous symptoms appear, immediately contact your healthcare professional. Otherwise, it may lead to serious complications.
Causes
Healthcare providers do not fully understand what exactly causes this disorder. However, they think it could be a genetic or autoimmune disorder. According to some research, there is a genetic variant (GRIN2A) found in children with LKS that could negatively affect the way brain cells communicate. It also links to other health conditions in the same category as LKS. However, not every child with a GRIN2A gene mutation develops LKS, but more research is needed to understand the connection. Check below for other gene mutations that could also contribute to LKS:
- CACNA1A
- FOXP1
- ATP1A2
- GRIN1
- KCNMA1
- PPFIA3
- KCNQ3
- PUF60
- SETD1B
- ZBTB18
- 2 duplication
- 5q22 deletion
Any changes in the previous genes negatively affect how the brain functions during development, sleep, and speech. Furthermore, researchers continue to study the connection between LKS and the immune system. For instance, if an autoimmune disorder negatively affects the body instead of protecting it, it may lead to damage to the brain and nerves, and eventually LKS. However, there is not enough evidence to prove this yet.
What Are The Potential Complications of LKS?
Children with LKS may also experience some complications, especially if the condition is left untreated. Check some examples below:
- Communication and language difficulties – In more severe cases, children lose their ability to speak and understand language. Without treatment, it may lead to a complete loss of speech.
- Epilepsy (seizures) – Approximately 80% of children who develop LKS will also experience seizures that happen during sleep. They can be partial motor, generalized clonic, or atypical absence seizures.
- Behavioral and psychiatric problems – These include attention deficit hyperactivity disorder (ADHD), hyperactivity and impulsivity, anxiety and depression, irritability, frequent tantrums, and others.
- Cognitive and learning issues – For example, memory impairment and intellectual disability.
- Motor function deficits – This complication occurs less commonly but may cause dyspraxia (lack of coordination), tremors, unsteadiness, or motor weakness (very rarely).
- Feeding and oral motor issues – In some cases, LKS affects the muscles around the mouth and throat, which often causes problems with chewing and swallowing.
- Sleep disorders – Those who suffer from LKS may also experience sleep disruptions.
The previous list does not contain a full list of complications, but doctors may recommend some ways to reduce the risk or even prevent them. Unfortunately, there is no way to prevent or reduce the risk of LKS because doctors do not fully understand what exactly causes it.
Diagnosis
Most of the time, it is difficult to diagnose LKS because it causes symptoms that are hard to notice or that resemble those of other health conditions. However, doctors usually start the diagnosis with a physical examination in which they check the child’s growth against typical childhood development milestones. Furthermore, they may also perform some tests to get more information about your child’s disorder. Check some of them below:
- EEG (electroencephalogram) – This is a painless test that is used to measure the electrical activity of the brain. It often helps identify abnormal brain activity.
- MRI (magnetic resonance imaging) scan – This test is used to get detailed images of different tissues and organs (including the brain) in the body.
- Audiometry to check for hearing loss
- Neurological examination
- Psychological examination
Treatment
Physicians usually prescribe different options to treat this condition because they depend on a few factors. These include the severity of the condition, overall health, gender, age, and preferences. Check some treatments often prescribed by doctors for people with LKS below:
- Medicines – The following medicines are often used to relieve the symptoms. For example, anti-seizure medicines, steroids, benzodiazepines, and others.
- Therapy – Physicians often recommend speech therapy, occupational therapy, and physical therapy because they help manage some symptoms of LKS.
- Surgery – This treatment is rarely recommended by doctors, but in some cases, it may be needed to treat a problem on one side of the brain (such as a unilateral lesion).
In general, there are no specific treatment options for LKS. Doctors often recommend the previous ones to manage the symptoms and improve your child’s quality of life.
Frequently Asked Questions
Can Landau-Kleffner syndrome be cured?
This condition cannot be cured because doctors do not know what exactly causes it. That’s why the treatment goal is to manage the symptoms, reduce the risk of serious complications, and improve your child’s quality of life.
Is Landau-Kleffner syndrome the same as autism?
No, they are different disorders. LKS is not even a type of autism. In people with autism, there are some differences in how the brain works, which often affects their behavior. However, these conditions can share some symptoms. Sometimes, the only way to distinguish these disorders is an EEG.
At what age does LKS typically appear?
While most children with LKS begin to experience symptoms between the ages of 5 and 7, they may occur later in their teen years.
Can children with Landau-Kleffner syndrome attend regular school?
Many children with LKS can attend regular school, especially if they receive appropriate support services such as speech therapy, special education assistance, or individualized learning plans. However, some children may require specialized educational settings depending on the severity of their language and learning difficulties.
Does every child with LKS have seizures?
No. Although seizures are very common in children with LKS, not every child experiences noticeable seizures. Some children may only show abnormal electrical brain activity on an EEG without having obvious seizure episodes.
Can speech and language abilities improve over time?
Yes. With early diagnosis and treatment, some children experience significant improvements in speech and language skills. The degree of recovery varies from person to person, and some individuals may continue to have communication difficulties into adulthood.
Is Landau-Kleffner syndrome inherited?
Most cases of LKS occur sporadically, meaning there is no known family history of the condition. However, certain genetic mutations have been linked to LKS, suggesting that genetics may play a role.
How common is Landau-Kleffner syndrome?
LKS is considered a very rare neurological disorder. It affects only a small number of children worldwide, which can sometimes make diagnosis challenging because many healthcare providers encounter very few cases during their careers.
Can sleep affect the symptoms of LKS?
Yes. Abnormal brain activity in children with LKS often becomes more pronounced during sleep. Because of this, sleep-related EEG testing is frequently important for diagnosis and monitoring the condition.
What specialists are involved in treating a child with LKS?
Treatment often requires a multidisciplinary team that may include pediatric neurologists, speech-language pathologists, psychologists, occupational therapists, educational specialists, and other healthcare professionals. Working together, these specialists help manage symptoms and support the child’s development and quality of life. Ask your healthcare provider if you have additional questions.
