Landau - Kleffner syndrome is called acquired aphasia with signs of epilepsy. Usually, children between the ages of three and seven suffer from it, they suddenly lose their ability to communicate, and they also don’t understand what other people are saying. Symptoms are also epilepsy attacks. 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, the symptoms are invisible at first. The development of the psyche occurs according to the norm. So most often occurs Landau-Kleffner syndrome. The child is practically not worried about anything, he is active, agile, like his peers.
Then, as they grow older, the baby may lose the ability to distinguish between known sounds, familiar speech, but understands what is written (if he can read) or gestures. The first symptoms flow into a complete loss of speech skill after weeks or even months. Sometimes there are periods of remission with restoration of speech.
Epilepsy
Seizures, as in epilepsy, occur in a significant number of cases. Either they appear before the absence of speech, or together with him. But a third of patients suffering from a disease such as Landau-Kleffner syndrome can only survive a seizure once during the entire course of treatment.
With this disease, forms of autism, aggressiveness are manifested. Often this is due to such phenomena in patients as stuttering, dysarthria. The child does not fix his gaze on the interlocutor, is immersed in his inner world, closes, becomes immune to pain, people around him notice the monotony of his voice. There are problems with motor skills. children can lick and smell food before meals, they have a disturbed sleep, they react strangely to sounds.
Boys suffer more often than girls. In all, 50 new cases are detected every year in all countries.
Disease differences
The difficulty in making the diagnosis is such 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 aforementioned disorder. Therefore, such children do not receive the necessary therapy. In the case of early detection of pathology and the provision of timely assistance, it is quite possible to restore lost speech.
The main difference from autism is the normal development during the first years of life, in children with autism, disorders are manifested up to three years.
How much speech is lost is also unclear. Sometimes this happens up to complete dumbness, jargon phase, and sometimes - only to the impoverishment of speech. In mild cases, there is a shortage of language, loss of smoothness of speech, impaired pronunciation.
Landau-Kleffner syndrome: etiology and prognosis
The etiology is unclear today. Of the alleged causes, the effects of encephalitis are highlighted, for which there is no direct evidence, and the genetic factor (caused by 12% of epilepsy patients among close relatives).
The human brain, more precisely, all the accumulated knowledge about it, will help in the future 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 control the speech with the help of the left hemisphere, it is responsible for language abilities. It stores data on the meaning of words, meaning, how to use them and in what situations. With damage to the right hemisphere, the same symptoms of aphasia are observed - speech loss. So Landau - Kleffner's syndrome can be shown also.
A person loses only the ability to speak, but in general is able to understand and realize what is happening around. Sometimes for left-handed people, on the contrary, the right hemisphere is responsible for linguistic abilities. What type of disturbance will be caused depends on which part of the brain is damaged.
Landau-Kleffner syndrome has a good prognosis. But everything will depend on the stage of the disease and on the treatment methods.
Causes of the disease
The disease is also called organic brain pathology. Children are thought to have genetic or acquired changes in brain organization, leading to epilepsy.
Diagnostics
The diagnostic criteria for Landau-Kleffner syndrome are not so extensive. To make a diagnosis, you need to collect an anamnesis. Attention is paid to such factors:
- about 6 months, the child loses the ability to understand and reproduce speech;
- before this, the child could speak and understand the words;
- the child has normal hearing;
- signs of conscious thinking in non-verbal manifestation persist;
- paroxysmal anomaly of electroencephalography in one or both temporal lobes;
- no other neurological disease is observed, except the previous one, and seizures of epilepsy;
- There are no other forms of disorders in the overall development.
With a brain injury or stroke, the child may also exhibit similar reactions. If, after studies of brain activity and disorders, this syndrome is detected, appropriate treatment is prescribed.
Consequences of the syndrome
Sometimes the development of the syndrome is associated with hearing loss. Difficulty swallowing may also occur. Symptoms may be temporary, sometimes permanent, depending on how damaged the brain is. Landau-Kleffner syndrome can be distinguished by the fact that with an average level of development, the child loses speech for no apparent reason.
The disease can be confused with a developmental disability, attention deficit disorder, childhood schizophrenia, auditory diseases, mental retardation and problems in behavior. All of these diseases have similar symptoms.
Epilepsy in the syndrome does not affect the cerebral substrate and the created language system, but creates problems for its normal functioning. Because the disease is recognized as a functional disorder. But not as it happens, for example, with brain pathologies.
The periods between the appearance 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 is still unknown whether the syndrome occurs in an adult. It is generally 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 has gone beyond the norms of age. And also if there are concomitant disorders 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 what was said, is limited in interests and is not socially adapted. At the same time, unlike autistic children, such children can interact, play roles, turn to parents in search of protection and comfort, and use gestures.
Landau-Kleffner syndrome and methods 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 incorrect treatment are extremely undesirable. The child must be observed in the neurological and neuropsychiatric departments.
The necessary steps in therapy
- Examinations by a neurologist, psychiatrist, audiologist, speech therapist, psychologist.
- Conducting magnetic resonance imaging of the brain.
- Electroencephalogram and optionally night EEG.
All this will slow down the development of such a nedun as Landau-Kleffner syndrome. Treatment will depend on how the body responds to the prescribed drugs, as well as on the stage of the disorder. It is necessary to be observed in a hospital, and after the onset of improvements, it can be treated on an outpatient basis.
As for the treatment of speech disorders, speech therapy methods help. There are no drugs for treatment yet. Specialists can help preserve the remaining speech skills, compensate for their further loss. Training is conducted with the repetition of words, training in gesturing, the development of facial muscles, learning to read and write, pictures and cards are introduced for communicating with others, there are computer programs for developing the skills of memorization, speech perception, repetition of what you hear.
How else can this syndrome be treated?
Surgically, the pressure of the tumor on the brain is reduced if it has caused speech impairment as a result of a blow or head injury. If there are no such injuries, then medication is used. The controversial point is the impact on the zones with the help of electric current: it is believed that in this way the wrong directions of brain activity are eliminated.
Modern treatment, provided timely detection and correct diagnosis helps 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 methods and programs there are sections devoted to the correction of the autonomic nervous system and neurotransmitter metabolism. These treatments include:
- neurobiomodulating control
- transcranial synchronization modulation,
- sensory vegetation,
- coherence
- flowering method
- empirically-centered therapy.
Is there any hope?
Landau-Kleffner syndrome is not a sentence. A third of these patients return to normal life. But a lot depends on when they asked for help and how correctly the treatment was prescribed. With the help of anticonvulsants, epilepsy attacks can be eliminated. Seizures can go to puberty, by 12-14 years.
The earlier hormonal drugs begin to be taken, the higher the likelihood of speech return. In severe forms, half of patients remain with problems understanding and reproducing speech for life.
Many spend years restoring children's speech functions. Sometimes, if the disease appeared earlier than 6 years, it has more positive prognoses.
According to studies, if a patient maintains the status of epilepsy during a night's sleep for 36 months, then, most likely, speech function will not fully recover. Landau - Kleffner's syndrome will be cured faster if the child's ability to communicate is preserved, thereby preserving the learning ability. Therefore, visual forms of the tongue are so popular in the treatment. At the level of neuropsychology, the skills of studying a sign or sign language can partially restore oral speech. So, children with the syndrome, by studying these techniques, can further improve their data in speech activity with a decline in epilepsy.
The disease is treated with surgery. You can use the Multiple Subpial Transaction method - dissection under the pia mater. It is carried out if speech is not restored within 3 years. Some children may spontaneously experience improvements, because before the three years of the course of the disease, this operation is not performed.
We examined what Landau-Kleffner syndrome is: symptoms, treatment, and prognosis. We hope you find the information useful.