Children often have seizures while sleeping? Watch for Symptoms of Landau-Kleffner Syndrome
Landau-Kleffner syndrome (Landau-Kleffner syndrome) is a rare disorder in children that causes behavioral disorders, seizures, and the inability to understand or express language. Usually, the onset of events begins in children 2 to 8 years of age.
This syndrome is known to affect twice as many boys as girls. What to look out for from Landau-Kleffner syndrome? The following reviews include the symptoms, causes, diagnosis and treatment.
1. Children with Landau-Kleffner syndrome can experience communication problems
A common symptom of Landau-Kleffner syndrome (LKS) is an inability to understand language (agnosia verbal audition) and express language (aphasia). In addition, there were also findings from abnormal electroencephalogram (EEG) tests, namely tests of the electrical activity of the brain.
Children who initially have normal language skills, both speaking and understanding language, such as reading and writing, may experience deterioration in language skills. They cannot communicate with other people.
Not only that, in 70 percent of cases, children with LKS also had seizures, especially while sleeping. These spasms can cause shaking and jerking on one or all sides of the body.
Usually the seizures last for a few minutes, but in some cases, the child has episodes of status epilepticus (seizures that do not stop on their own or require certain treatment).
In a minority of cases, the person may also develop serious behavioral dysfunction, including hyperactivity, attention deficits, impulsivity, emotional outbursts, and / or withdrawal behavior. Some children may also have intellectual disabilities.
2. Landau-Kleffner syndrome is caused by a genetic mutation
It is not clear what causes LKS to occur. Launching Verywell Health, in 20 percent of cases, it is known that LKS is caused by a mutation of the GRIN2A gene, which is located on chromosome 16. This gene plays a role in the production of the protein GluN2A, which is a glutamate receptor which usually helps prevent overactivity of nerves in the brain.
Launching MedlinePlus, the GluN2A protein is found in brain nerve cells, including the part of the brain involved in speech and language. This protein sends signals to nerve cells in the brain. When there is interference, the cell signaling is not transmitted properly, which in turn affects the ability to understand and express language.
Meanwhile, in 80 percent of cases, GRIN2A gene mutations were not found in LKS patients. This possibility occurs due to mutations of other genes or interactions between genes and environmental factors. Other causes such as autoimmune response have also been reported for this case, as explained by the National Institutes of Health (NIH).
3. This neurological disorder can be inherited from parents or the result of new mutations
LKS caused by mutations in the GRIN2A gene are usually inherited from parents in an autosomal dominant manner. This means, one mutated copy of the gene can cause the symptoms of this syndrome.
In some cases, LKS also occurs due to new or de novo spontaneous mutations, in which the child develops symptoms on their own without inheriting from their parents.
4. The results of the electroencephalogram (EEG) test are usually the basis for the diagnosis of Landau-Kleffner syndrome
The diagnosis of LKS is based on the results of a clinical examination and the results of the brain electrical activity test (EEG). Children with LKS have abnormal brain electrical activity in the left brain and right brain, namely the presence of slow wave surges from the temporal lobe. It has evidence of persistent seizures during sleep.
Several other tests may also be needed to confirm a diagnosis and rule out other causes of your symptoms, including:
Neuropsychological tests to evaluate learning abilities, attention, and understanding.
Audiometric tests to confirm that communication problems or language loss are not due to hearing problems.
Brain imaging such as MRI to rule out other causes.
Lumbar puncture to look for any infection or inflammation that can exclude encephalitis.
5. Speech therapy and the use of anti-seizure drugs are usually used to treat this rare disorder
LKS treatment is tailored to the specific symptoms experienced by the patient. Children with LKS who have seizures may be prescribed anticonvulsants and corticosteroids. In addition, speech therapy is usually recommended to be carried out as soon as possible so that the child regains his speech ability.
Those are some medical facts about Landau-Kleffner syndrome. This disease is not life threatening, but it can interfere with the patient’s quality of life. Therefore, consult your doctor if you find the symptoms mentioned above so that they can be examined further.