What is Landau-Kleffner Syndrome?
Landau-Kleffner Syndrome is an extremely rare form of seizures or epilepsy occurring in children normally between the ages of 3 and 9 years of age. This disorder impairs the ability of the child to understand instructions and be able to communicate to others. Landau-Kleffner Syndrome affects the functioning of the brain such that there are abnormal electrical brain waves especially when the child is asleep resulting in the child having seizures.
Apart from seizures, children with Landau-Kleffner Syndrome also have impaired behavior, motor skills, and have problems learning. Another name given to Landau-Kleffner Syndrome is epileptic aphasia where aphasia clearly signifies the inability of the child to use language and speak or communicate.
What are the Causes of Landau-Kleffner Syndrome?
There is not much known about the causes of Landau-Kleffner Syndrome. This condition is seen predominantly in boys than girls. In some cases, there has been a family history of Landau-Kleffner Syndrome. Researchers believe that a child with Landau-Kleffner Syndrome has an inbuilt abnormality that leads to abnormal electrical activity in that area of brain that is responsible for language processing. This abnormal electrical activity prevents the brain cells from working normally resulting in the child losing his or her language skills. In some cases, this abnormal electrical activity causes seizures, especially when the child is asleep.
It cannot be said that the brain of the child with Landau-Kleffner Syndrome is damaged to any extent but that the brain is unable to perform certain functions due to some abnormal electrical activity which causes the child to lose skills that he or she may have had previously like the ability to speak, read, wrist, understand instructions. These impairments may get better or take a turn towards worsening with time as the child grows. This makes Landau-Kleffner Syndrome a bit unpredictable.
What are the Symptoms of Landau-Kleffner Syndrome?
There are three major symptoms of Landau-Kleffner Syndrome. These symptoms are:
Loss of language skills: In the initial stages of life, the child with Landau-Kleffner Syndrome will have normal language skills and will be able to understand and comprehend normally but after reaching the age of 3 or 4 the child starts to gradually lose all the skills that he or she had gained previously. His loss of skills may be of sudden onset of may be gradual due to the unpredictability of Landau-Kleffner Syndrome. Some children may even try to hide this and may use visual cues to compensate for their lack of hearing or inability to understand but gradually there is a trend towards regression where the child starts to lose his or her ability to speak or understand language.
Behavioral Changes: This is the second symptom of Landau-Kleffner Syndrome. The behavioral changes associated with Landau-Kleffner Syndrome may include hyperactivity, reduced concentration, irritability, temper tantrums, and problems with interaction with peers. The child may also have issues with fine motor coordination and movement. The child may also have problems with eating and may not have clarity in speech. The child may also demonstrate clumsiness and shakiness.
Seizures: This is the third and most clinching evidence that the child is suffering from Landau-Kleffner Syndrome. A child with Landau-Kleffner Syndrome will have seizures first before the real regression of their ability starts. The seizures associated with Landau-Kleffner Syndrome are rather short and have a distinct relationship with the child’s ability to understand and comprehend language skills and ability to do other motor coordination activities.
How is Landau-Kleffner Syndrome Diagnosed?
The diagnosis of Landau-Kleffner Syndrome can be made just by taking a clinical history of the child and examining the child’s ability to communicate and understand language skills. During clinical interview, it will become more or less apparent that the child apparently regressed when he or she reached age 3 or 4 along with a history of short spanned seizures will decisively point towards Landau-Kleffner Syndrome. A physical examination may show difficulty with movement in some cases. The doctor may order an MRI of the brain for completeness sake and rule out other disorders but in majority of the cases this comes back as normal.
EEG of the child will detect abnormal electrical activity over the areas of the brain which are responsible for language perception. This abnormal electrical activity will increase when the test is conducted with the child being asleep. Since Landau-Kleffner Syndrome is so rare, it becomes quite common for doctors to look for other causes for the condition and when all the conditions are ruled out the diagnosis of Landau-Kleffner Syndrome is confirmed.
How is Landau-Kleffner Syndrome Treated?
When the disease condition is in its active phase then medications will be given for seizures to control the abnormal activity of the brain. Generally in Landau-Kleffner Syndrome, there are two types of seizures which occur. One is visible and affects the language skills of the child while the other one is invisible and occurs when the child is asleep and is actually the cause for regression of the skills that the child has. For these seizures, the child is given anticonvulsants. These medications are quite effective for the visible form of the seizures but are not as effective for the invisible form of the seizures, although some children may respond to them favorable if given in high doses.
Steroid medications have shown to be quite effective in stopping both type of seizures in Landau-Kleffner Syndrome and also allows the child to regain a lot of the lost skills. The steroids are given in short bursts and the child is closely monitored for side effects. Some children regain their lost skills and some lose them when the steroids are stopped. Such children become steroid dependent and may have to be on steroids on a weekly basis for a number of years. In some cases, the child may have no response to the steroid doses.
Surgery is yet another form of treatment for Landau-Kleffner Syndrome. The main goal of the surgery is to prevent the spread of the abnormal electrical activity in the brain. The surgery done for Landau-Kleffner Syndrome is called as multiple subpial transection. This involves making a series of fine cuts through the surface of the brain under continuous EEG supervision and only the area of the abnormal electrical activity is involved and any damage there is corrected. Surgery is recommended only for those children who are unresponsive to other conservative medications like anticonvulsants and steroids and are severely impaired by Landau-Kleffner Syndrome.
Prognosis & Recovery of Landau-Kleffner Syndrome
The active phase of Landau-Kleffner Syndrome normally ends by the time the child becomes an adolescent and the child is able to regain all of the skills that he or she lost due to Landau-Kleffner Syndrome. About 50% of children with Landau-Kleffner Syndrome make reasonable recovery while about 25% make partial recovery. The remaining 25% of children with Landau-Kleffner Syndrome have poor recovery and are not able to regain their lost abilities in language, comprehension and learning skills. Children with a good outcome from treatment may recover most of their lost skills but may still be troubled with things like short term memory and may have poor hearing skills. Children with poor outcome with all forms of treatment tend to never regain their speech and language skills which they had lost due to Landau-Kleffner Syndrome.