What Is Landau Kleffner Syndrome (LKS)?
Landau Kleffner syndrome is also known as infantile acquired aphasia, acquired epileptic aphasia, or aphasia with the convulsive disorder. Landau Kleffner syndrome is a condition characterized by abnormal electrical activity in the brain that affects the children's speech and language understanding ability. It is a condition that affects the brain, and children are reported to suffer from seizures.
What Causes Landau Kleffner Syndrome?
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Landau Kleffner syndrome's exact cause is unknown, even though researchers have found mutations in about 20 % of the children tested. Upon studying a particular group of children with Landau Kleffner syndrome, they have found that there is a mutation in the GRIN2A gene.
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Children might have inherited this gene from their parents, but all the children with it did not develop this syndrome. Also, in some other groups of children, the mutation is seen in other genes, and they might be the first ones to have this mutation in their family, known as De Novo Mutation.
Scientists think that this mutated gene sends an abnormal electrical signal from the brain, which results in LKS symptoms.
There is also a study happening to find any link between the immune system and LKS.
What Are the Symptoms of Landau Kleffner Syndrome?
Landau Kleffner syndrome symptoms vary for different children.
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Children with LKS are likely to have acquired deafness as they fail to respond to verbal commands, and in some cases, to nonverbal communication too.
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These children are unable to speak.
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LKS children have seizures (sudden uncontrolled electrical disturbance in the brain which results in changes in behavior, movements or feelings, and level of consciousness).
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Also, these children tend to have behavioral disorders such as hyperactivity, temper outbursts, attention deficit hyperactivity syndrome, or withdrawn behaviors.
Who Does Landau Kleffner Syndrome Affect?
Landau Kleffner syndrome is seen in children aged between three years and eight years. But in some cases, children of age two years and adolescents can also have this condition.
LKS is seen to be more prevalent in the male population than the females. LKS is reported twice as many as in females.
How Is Landau Kleffner Syndrome Diagnosed?
The developing stage in children needs to be observed very keenly, and parents should consult a doctor when they observe any unusual changes in their child’s development. The doctor will take a detailed history of the child’s observed symptoms.
Apart from the history, the doctor will advise some tests to monitor the electrical activity of the brain, like an electroencephalogram (EEG). EEG shows unusual electrical activity patterns and can be used to diagnose seizures with these patterns. This test is done when the child is sleeping and also when awake. The waves slow down when the child enters the early stages of sleep, and the waves become more child active when the child enters the rapid eye movement (REM) sleep phase. Whereas in children with Landau Kleffner syndrome, their brain activity remains at continuous levels of abnormal brain wave activity during all phases of sleep.
Other tests which the doctor might advise are:
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MRI and CT scans can be used to study where the seizure is happening in the brain and also, it can eliminate other possible causes of the seizure.
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Audiometry (hearing test).
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An evaluation by a psychologist.
What Is the Treatment for Landau Kleffner Syndrome?
The treatment of LKS includes a panel of doctors and therapists. Health providers will advise mediations, speech therapy, and behavioral therapy as the treatment for Landau Kleffner syndrome.
Medications:
The medications used are:
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Anticonvulsant medications to control seizures.
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Corticosteroids or Adrenocorticotropin (ACTH) in order to reduce immune system response.
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In case when the antiepileptic drug is found to be ineffective, then the ketogenic diet and intravenous immunoglobulins are given.
Therapy:
The combined effort of the speech and language therapist is effective in the treatment of the Landau Kleffner syndrome. Sign language training could be given to children with no understanding of language. Alternative communication devices can also be used in children, which will be of use to them. Special education classes for these children will also be beneficial for them.
Surgery:
Rarely in certain cases do surgeons perform multiple subpial transections, but the effectiveness of this surgery in all cases is still not sure, and hence the benefits and the risks should be carefully studied before opting for the surgery.
Is Landau Kleffner Syndrome Curable?
The prognosis of children with Landau Kleffner syndrome varies in different children. Some affected children will have permanent speech and language problems, while some may regain language abilities. In some other cases, relapse is also seen in cured cases. The prognosis is better when the age of onset of the disease is after six years and when the speech therapy is started as early as possible. Seizures usually disappear by adulthood, and the epilepsy activity on the EEG also tends to improve by that time.
What Other Disorders Are Related to LKS?
The following syndromes are associated with Landau Kleffner syndrome as a secondary characteristic.
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Autism Spectrum Disorders- This is a group of neurodevelopmental conditions that appears before the age of 30 months. Children with this disorder are characterized by problems in social communication and other behavioral problems such as restricted interests and repetitive behavior.
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Rett Syndrome- Rett syndrome is a rare neurodevelopmental condition caused by a gene mutation. It almost occurs exclusively in females. This is characterized by acquired microcephaly (slow growth of head), loss of control of voluntary movements required for walking, and repetitive hand movements such as clapping or rubbing. These children also develop difficulties in swallowing, feeding and breathing, growth retardation, and seizures.
Conclusion
Landau Kleffner syndrome is a neuropsychological condition that affects the activity of the brain, and the children can have episodes of seizures also; these children will have difficulty in speech and understanding language. This condition is seen in children of age three years to eight years old. Males are generally affected more than females. The treatment involves a team effort from the specialists as well as from the therapists. The prognosis is better when the treatment and the therapies are started at an early stage. The parents should carefully observe the developing stage for an abnormality as many conditions are curable when diagnosed early and appropriate treatment is taken.
