Landau-Kleffner syndrome is an acquired epileptic aphasia in children. It is characterized by a violation of speech function. At the same time, it is not the articulatory components of speech (sound modification with the help of teeth, tongue and pharynx) that suffer, but the ability to formulate phonemes at the central nervous system level. By the way, a certain area in the cerebral cortex is responsible for this.
The disease develops in completely healthy children. At first, they lose their ability to adapt to the external environment, then they cannot expressively, clearly and logically express their thoughts. The fact that pathological changes undergo in the baby’s brain shows EEG. The syndrome was described in 1957 by two scientists - Landau and Kleffner. Therefore, their names and called this ailment.
Causes of the disease
The causes of Landau-Kleffner syndrome are not fully defined. Although in some cases, doctors can name the factor that led to the development of the disease. First of all, these are closed head injuries, brain tumors, a parasitic disease caused by larvae of the pork tapeworm, neurocysticercosis. Autoimmune demyelinating illnesses, during which the destruction of the white matter of the nervous system, myelin, can also become a cause. Inflammatory processes on the walls of blood vessels in the brain are also capable of provoking the development of the syndrome. Several cases of the appearance of pathology were associated with a deficiency of the mitochondrial respiratory complex. Sometimes the cause was too thin a cerebral cortex, as a result of which the child developed a hemisphere lesion.
The syndrome may have a transient form. That is, yesterday, a two-year-old baby walked, spoke and was an absolutely healthy child, but today he wakes up, unable to bind a few words. Sometimes a disease develops slowly: the baby gradually loses its ability to verbal communication and an adequate perception of reality.
How is it manifested?
The disease was described in detail by the female scientist Alexandrov N. Sh. Landau-Kleffner syndrome, she argues, also arises from the so-called plasticity of the brain. It is as if “being rebuilt” in order to better fulfill its functions. In the process of such a transformation, temporary aphasia develops. As in the case of the acquired form caused by damage and disease, this type of pathology can be diagnosed using EEG. In addition, the “first bells” can be the epileptic seizures that appear in the baby. They in 70% of cases accompany the onset of the disease.
In about a quarter of all cases, the patient loses speech function during the month. But more often, her loss occurs almost instantly. Sometimes a baby cannot utter a sound. Half of the patients have behavioral disorders. Operational thinking is maintained. With age, epileptic seizures disappear. Full or partial restoration of speech functions occurs by 16 years.
Speech impairment
Landau-Kleffner syndrome in a child begins with loss of speech function - this is one of the main symptoms of the disease. It is characterized by verbal or auditory agnosia - impaired perception, in which consciousness and sensitivity are preserved. In the first case, the patient is not able to speak normally. In the second - he may not recognize familiar noises and sounds. In this case, diagnostic responses to phonological audiograms are normal.
The main problem lies precisely in the field of sound susceptibility. Despite this, the syndrome manifests itself in a critical period of mastery of the linguistic apparatus. Speech impairment can be of various difficulty levels. Aphasias are very strong in a number of patients. According to scientists, 6 out of 77 patients are diagnosed with complex cases. If a child has minor disturbances, he can maintain reading and writing skills. Language disorders are manifested as follows: the child confuses letters and sounds, incorrectly constructs sentences, cannot logically express his thoughts, and so on.
Bouts of epilepsy
Clinical seizures are observed in 70-85% of cases of acquired Landau-Kleffner syndrome. An illness in a third of children begins with a single epileptic seizure. This is the start of the disease. In some patients, several years may elapse between an attack and speech impairment. Seizures are often reported between the ages of 4 and 10 years. Many doctors say that they should not be observed in adulthood. That is, after 16 years, seizures do not occur.
In 58% of patients, partial seizures are recorded . They cause damage to brain cells in a specific area of one hemisphere of the brain. Convulsions are observed in 39% of children, seizures with loss of consciousness - in 16%. Some patients are prone to myoclonic disorders affecting the muscles of the face and eyeballs. In 12% of patients, epilepsy is transmitted at the genetic level.
Behavioral Disorders
They are characteristic of almost all patients. 78% of young children have behavioral disorders if they are diagnosed with Landau-Kleffner syndrome. Adults should not be surprised by the fact that their once calm and affectionate baby suddenly became aggressive. Attacks of rage are a standard symptom. Anger can be so strong and uncontrollable that a child cannot cope with it without being hospitalized in a psychiatric institution. Others, on the contrary, abruptly become closed. They shun society of people, suffering from anxiety and obsessive thoughts.
Some children seem completely deaf. Another is mistakenly diagnosed with autism. Indeed, it is sometimes difficult for doctors to determine that the real cause of this strange behavior is the Landau-Kleffner syndrome. After all, he and autistic behavior are characterized by such phenomena as an inability to build a logically complete sentence, an obsessive repetition of the same phrases, words. A weak concentration of attention and increased activity are observed in 80% of patients. Although behavioral disorders are secondary symptoms in relation to speech problems, some children may be surprised by unusual, illogical, and bizarre reactions. For example, they are able to repeat the same gesture for hours.
Modern therapy
At the first signs of the disease, it is necessary to begin treatment of the Landau-Kleffner syndrome. The clinic you are contacting will be able to offer several solutions to the problem:
- medication course;
- surgical intervention.
In the latter case, the skull is opened to the patient and the epileptogenic focus is removed. For this, some bones and part of the dura mater are cut off. After that, the surgeon makes incisions on the gray matter. They will further prevent the onset and spread of epileptogenic impulses. Then the hard shell and bones are put in place. The wound is sutured. 6-7 weeks after surgery, the child from the hospital can return home. After surgery, children with Landau-Kleffner syndrome notice an improvement in intellectual and psychological functions.
Remedies for seizures
The treatment of Landau-Kleffner syndrome does not always provide only an operational option. Sometimes drug therapy helps get rid of the disease. It provides for the administration of anticonvulsants. Unfortunately, they do not always give a positive result. For example, Carbamazepine, Phenobarbital, Phenytoin are often ineffective - aphasia can only worsen. Seizures in patients will be repeated with a vengeance. This is especially true for patients with frequent seizures or atypical absences.
In addition, doctors often recommend Nicardipine. It helps block calcium channels. The intravenous administration of gamma globulin is also beneficial. Such medicines proved to be quite effective: Ethosuximide, Valproic acid. Benzodiazepines also help, especially Klobazam and Midazolam. But they should be administered intravenously.
Other drugs
Landau-Kleffner syndrome, the symptoms and treatment of which are described in this article, also involves taking Diazepam before bedtime. This drug gives good results. Therapy is prescribed for a period of 6 weeks to avoid tachyphylaxis - a rapid decrease in the effect with repeated use of the medication. Studies have shown that Levetiracetam may be useful. It also helps very well in the treatment of childhood idiopathic focal seizures, electrical sleep status epilepticus, suppression of a continuous wave surge in its slow phase. In addition, the drug "Felbamat" is successful in treating aphasia and seizures. But with its use, the development of many side effects is possible, among which the most common are aplastic anemia and liver dysfunction.
Corticosteroids
Corticosteroids also help in the treatment of Landau-Kleffner syndrome. Especially well-known "Adrenocorticotropin". It reduces the manifestation of symptoms of acquired epileptic aphasia, normalizes the state of EEG. Long-term therapy with these drugs is recommended for most patients. However, it should be remembered that the hormonal treatment option is not suitable for everyone.
Doctors often prescribe steroids for patients. For example, Prednisone is administered intravenously for three months. Therapy is used to induce remission. It is important to consider that lowering the dosage of steroids is often associated with persistent relapses of signs of the disease. The total duration of the course can last from six months to several years. By the way, treatment with corticosteroids has a lot of side effects, such as: weight gain, hypertension, diabetes, cataracts, renal failure, depression, manic syndrome and so on. In addition, they are not recommended for patients with immunity problems, as they inhibit it even more. They are also contraindicated in people with aseptic necrosis, tuberculosis or ailments, the appearance of which is provoked by the activity of roundworms.
Opinions of parents
Particular care and attention requires a child who is diagnosed with Landau-Kleffner syndrome. Parents' reviews indicate that with the baby it is necessary to go to classes to a speech therapist-defectologist. The doctor is not able to restore speech functions. But such sessions accustom the crumbs to perseverance, bring up responsibility and organization in it. Parents claim that abroad, such children are prescribed a good anticonvulsant and antiepileptic drug called Keppra. We recommend “Depakine”, but it often negatively affects the pancreas, so some go to homeopathic treatment. Many mothers and fathers also note that the best remedy for the syndrome is growing up the baby. He is getting older - his speech is gradually improving. In addition, seizures and paroxysms pass with age.
Forecast
As for doctors, they also continue to study what Landau-Kleffner syndrome is. Photos of patients, a video with their seizures, anamnesis, the conclusion of specialists of a narrow profile - all this is part of the research. Doctors noticed that therapy works better if the child attends sessions of psychotherapy, speech classes. Full recovery of verbal functions is quite rare. Typically, 2/3 of children retain a more or less serious speech impairment.