Landau-Kleffner syndrome: description, causes, prognosis and ways of treatment

Landau-Kleffner syndrome is termed acquired aphasia with signs of epilepsy. Usually they suffer from children between the ages of three and seven, they suddenly lose the ability to communicate, and also do not understand what other people tell them. Symptoms are also epileptic seizures. The syndrome was first described in 1957.

Clinical picture

The clinic is such that it resembles a disorder of receptive speech, that is, the ability to understand others. This syndrome differs in that the patient does not show strong deviations. In such a disease, symptoms are initially invisible. The development of the psyche occurs accordingly to the norm. So often the Landau-Kleffner syndrome occurs. The child is practically nothing to worry about, he is active, agile, like his peers.

Then, as he grows up, the kid may lose the ability to distinguish known sounds, familiar speech, but understands what he has written (if he can read) or gestures. The first signs flow into a complete loss of speech skill after weeks or even months. Sometimes there are periods of remission with speech restoration.

Epilepsy

Seizures, as in epilepsy, occur in a significant number of cases. Either they appear before the absence of speech, or together with it. But a third of patients suffering from a disease such as Landau-Kleffner syndrome can only once experience an attack for the entire course of treatment.

With this disease manifest forms of autism, aggressiveness. This is often associated with such phenomena in patients as stammering, dysarthria. The child does not fix his gaze on the interlocutor, is immersed in his inner world, becomes isolated, becomes immune to pain, the surrounding people notice the monotony of his voice. There are problems with motor skills. Babies can lick and sniff the food before the reception, they are disturbed by sleep, they react strangely to the sounds.

Boys suffer more often than girls. A total of 50 new cases are detected each year in all countries.

Differences in the disease

The difficulty in diagnosing is that many children with different forms of autism are not diagnosed with "Landau-Kleffner syndrome." Because it is difficult to identify and distinguish from the disorder. Therefore, such children do not receive the necessary therapy. In the case of early detection of pathology and timely assistance it is possible to restore lost speech.

The main difference from autism is normal development during the first years of life, in children with autism, violations occur up to three years.

How much speech is lost is also unclear. Sometimes this occurs up to complete dumbness, jargonaphasia, and sometimes - only to the depletion of speech. In mild cases, there is a lack of language, a loss of fluency in speech, a violation in pronunciation.

Landau-Kleffner syndrome: etiology and prognosis

Etiology is unclear to this day. Of the putative causes, the effects of encephalitis are highlighted, to which there is no direct evidence, and the genetic factor (due to 12% of cases of epilepsy among close relatives).

The human brain, more precisely, all the accumulated knowledge about it will help in later to find out the causes and the true etiology of the syndrome. As you know, cerebrum (the main part of the brain) is divided into the left and right hemispheres. Most people manage speech with the help of the left hemisphere, it is responsible for language abilities. Here we store data on the meaning of words, meaning, how to use them and in what situations. If the right hemisphere is damaged, the same symptoms of aphasia are observed: speech loss. So it can be manifested and Landau - Kleffner syndrome.

A person loses only the ability to speak, but as a whole is able to understand and realize what is happening around. Sometimes left-handers for language abilities, on the contrary, are responsible for the right hemisphere. What kind of violations will be caused depends on which part of the brain is damaged.

The Landau-Kleffner syndrome has a good prognosis. But everything will depend on the stage of the disease and on the methods of treatment.

Causes of the disease

The disease is also called organic brain pathology. It is assumed that children have genetic or acquired changes in the brain organization, which leads to epilepsy.

Diagnostics

The diagnostic criteria for the Landau-Kleffner syndrome are not so extensive. To make a diagnosis, you need to collect anamnesis. Attention is paid to such factors:

• About 6 months the child loses the ability to understand and reproduce speech;
• Before that the child could speak and understand words;
• The child has a normal hearing;
• Signs of conscious thinking in a non-verbal manifestation persist;
• Paroxysmal anomaly of electroencephalography in one or both temporal lobes;
• There is no other disease in the line of neurology other than the previous one, and seizures of epilepsy;
• There are no other forms of disorders in general development.
  

If a brain trauma or stroke occurs, the child may also show similar reactions. If after the carried out researches of activity of a brain and infringements the given syndrome is revealed, treatment is appointed corresponding.

Consequences of the syndrome

Sometimes the development of the syndrome is also associated with hearing loss. Difficult swallowing may also occur. Symptoms can be temporary, sometimes they are permanent, depending on how damaged the brain is. Distinguishing Landau - Kleffner syndrome helps that at an average level of development a child loses speech for no apparent reason.

The ailment can be confused with a deviation in development, attention deficit disorder, childhood schizophrenia, auditory ailments, mental retardation and behavioral problems. All these diseases have similar symptoms.

Epilepsy in the syndrome does not affect the cerebral substrate and the created system of language, but creates problems for its normal functioning. Therefore, ailment is recognized as a functional disorder. But not such, which happens, for example, with pathologies of the brain.

The periods between the occurrence of seizures and loss of speech can be different. Sometimes the interval between these symptoms is one or several months, and sometimes a couple of years. It remains to be seen whether the syndrome occurs in an adult. It is usually believed that children with Landau-Kleffner syndrome are already predisposed to epilepsy.

The diagnosis is made at a time when the delay in speech development is beyond the norms of age. And also if there are accompanying violations of the emotional sphere: hyperactivity, anxiety, isolation from others. In severe forms, the child in his speech imitates the ordinary language and does not understand the meaning of the above, is limited in the interests and socially not adapted. In this case, unlike autistics, such children can interact, play by roles, turn to their parents in search of protection and comfort, use gestures.

Landau-Kleffner syndrome and ways of treatment

First of all, anticonvulsants and corticosteroids, in particular "Prednisolone", are prescribed. The course of the disease is unpredictable, the consequences are severe, therefore untimely assistance and wrong treatment are highly undesirable. The child should be observed in the neurological and neuropsychiatric departments.

Necessary steps in therapy

• Inspections of a neurologist, psychiatrist, surdologist, speech therapist, psychologist.
• Conduction of magnetic resonance imaging of the brain.
• Electroencephalogram and additionally nocturnal EEG.

All this will allow to slow down the development of such neduna as the Landau-Kleffner syndrome. The treatment will depend on how the body reacts to the prescribed drugs, as well as from the stage of disturbances. It is necessary to be observed in a hospital, and after the onset of improvements, it is possible to be treated also out-patiently.

As for the treatment of speech disorders, the methods of speech therapy help. There are no drugs for treatment yet. Specialists can help preserve the remaining speech skills, compensate for their further loss. Training with repetition of words, learning to communicate with gestures, development of facial muscles, learning to read and write, drawing pictures and cards for communicating with others, computer programs for developing memorization skills, perception of speech, repetition of heard are conducted.

How else can you treat this syndrome?

The tumor pressure on the brain is reduced surgically, if it causes speech disorders as a result of a stroke or a head injury. If there are no such injuries, then medicamental treatment is used. The controversial issue is the impact on the zones by means of an electric current: it is believed that in this way the wrong directions of brain activity are eliminated.

Modern treatment under the condition of timely detection and diagnosis is helping to cope with many manifestations of the syndrome. If the child does not receive treatment, the uncontrolled development of pathology will lead to the death of brain cells. Therefore, in the methods and programs there are sections on the adjustment of the autonomic nervous system, neurotransmitter metabolism. These types of treatment include:

• Neurobiomodulating control,
• Transcranial synchronization modulation,
• Vegetosensory stimulation,
• Coherence,
• Method of flowering,
• Empanic-centered therapy.

Is there any hope?

The Landau-Kleffner syndrome is not a verdict. A third of these patients return to normal life. But much depends on when asked for help and how correctly the treatment is prescribed. With the help of anticonvulsants, it is possible to eliminate epileptic seizures. Seizures can pass to the period of puberty, to 12-14 years.

The earlier hormonal drugs are taken, the higher the probability of a return of speech. In severe cases, half of the patients have problems with understanding and reproduction of speech for life.

Many spend years on restoring children's speech function. Sometimes, if the disease has appeared before 6 years, it has more positive predictions.

According to the research, if the patient has a status of epilepsy during the night sleep for 36 months, then, most likely, the speech function will not fully recover. Landau-Kleffner syndrome is more quickly cured if the child's ability to communicate is preserved, thereby retaining the ability to learn. Therefore, visual forms of language are so popular in the treatment. At the level of neuropsychology, the skills of learning sign or sign language can partially restore and oral speech. So, children with the syndrome, studying these techniques, can later improve their data in speech activity with the decline of epilepsy.

The disease is treated with a surgical procedure. It is possible to use the Multiple Subpial Transaction method - dissection under the soft dura mater. It is held in case if speech is not restored within 3 years. Some children may experience spontaneous improvements, therefore, prior to three years of the disease, this operation is not performed.

We examined what Landau-Kleffner syndrome is: symptoms, treatment and prognosis. We hope that the information will be useful to you.