Symptoms and Treatment of Landau-Kleffner Syndrome
Landau-Kleffner syndrome is an acquired epileptic aphasia in children. It is characterized by a violation of speech function. In this case, not the articulatory components of speech themselves( sound modification with the help of teeth, tongue and pharynx) suffer, but the ability to formulate phonemes at the level of the central nervous system. By the way, a certain area in the cerebral cortex is responsible for this.
The disease develops in absolutely healthy children. Initially, they lose the ability to adapt to the external environment, then can not expressively, clearly and logically express their thoughts. The fact that the brain of the baby is pathological changes, 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 the Landau-Kleffner syndrome are not completely determined. Although in some cases, doctors can call the factor that led to the development of the disease. In the first place - these are closed head injuries, brain tumors, parasitic disease caused by larvae of the pork chain, - neurocysticercosis. The cause can also be autoimmune demyelinating ailments, during which the white matter of the nervous system, myelin, is destroyed. Provoke the development of the syndrome is also capable of inflammatory processes on the walls of blood vessels in the brain. Several cases of the appearance of pathology were associated with a deficit in the mitochondrial respiratory complex. Sometimes the cause was too thin cortex of the brain, as a result of which the child developed hemispheric damage.
Syndrome may have a transient form. That is, yesterday a two-year-old boy walked, talked and was absolutely healthy child, and today he wakes up, unable to connect a few words. Sometimes the disease develops slowly: the crumb gradually loses its ability to verbal communication and an adequate perception of reality.
How is it manifested?
The disease was described in detail by a female scientist Alexandrova N. Sh. Landau-Kleffner syndrome, she claims, also arises from the so-called plasticity of the brain. He seems to be "rebuilt" in order to better perform his functions. In the process of such transformation, temporary aphasia develops. As with the acquired form caused by injuries and illnesses, this type of pathology can be diagnosed by EEG.In addition, "the first bells" may be epileptic seizures that have appeared in the baby. They in 70% of cases accompany the onset of the disease.
Approximately in a quarter of all cases, the patient loses speech function for a month. But more often its loss occurs virtually instantaneously. Sometimes a kid can not utter a sound. Half of the patients have behavioral disorders. In this case, operational thinking is preserved. With age, epileptic seizures disappear. Complete or partial restoration of speech functions occurs by the age of 16.
The Landau-Kleffner syndrome in a child begins with a loss of speech function - this is one of the main symptoms of the ailment. It is characterized by verbal or auditory agnosia - impaired perception, in which consciousness and sensitivity remain. In the first case, the patient is not able to speak normally. In the second, he may not recognize familiar noises and sounds. At the same time, diagnostic answers to phonological audiograms are normal.
The main problem lies precisely in the sphere of sound susceptibility. Despite this, the syndrome manifests itself during the critical period of mastering the language apparatus. Violations of speech reproduction can be of varying complexity. Aphasia is very strong in a number of patients. According to the scientists, complicated cases are diagnosed in 6 patients out of 77. If the child has minor violations, he can keep reading and writing skills. Language disorders manifest themselves as follows: the child confuses letters and sounds, improperly constructs sentences, can not logically express his thoughts and so on.
Clinical seizures are observed in 70-85% of cases of acquired Landau-Kleffner syndrome. The ailment in a third of children begins with a single epileptic fit. This is the start of the disease. A number of patients between the seizure and speech disorders can take several years. Seizures are often recorded between the ages of 4 and 10 years. Many doctors claim that in adulthood they should not be observed. That is, after 16 years of seizures do not occur.
58% of patients have partial seizures. They cause damage to brain cells in a specific area of one hemisphere of the brain. Seizures are observed in 39% of children, fits with loss of consciousness - in 16%.Some patients are susceptible to myoclonic disorders affecting the facial muscles and eyeballs. In 12% of patients, epilepsy is transmitted at the genetic level.
They are typical for almost all patients. In 78% of young children, behavioral disorders are observed if they are diagnosed with Landau-Kleffner syndrome. Adults should not be surprised that their once quiet and affectionate kid suddenly became aggressive. Fits of rage are a standard symptom. Anger can be so strong and uncontrollable that the child can not cope with it without having to go to a psychiatric institution. Otherwise, on the contrary, they become sharply closed. They avoid the society of people, suffering from anxiety and obsessive thoughts.
Some children seem completely deaf. Others make the wrong diagnosis - autism. Indeed, it is sometimes difficult for doctors to determine that the real cause of strange behavior is Landau-Kleffner syndrome. After all, he and autistic behavior are characterized by such phenomena as the inability to build a logically complete sentence, the obsessive repetition of the same phrases, words. Weak concentration of attention and increased activity are observed in 80% of patients. And 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.
At the first signs of the disease, it is necessary to begin treatment of the Landau-Kleffner syndrome. The clinic that you contact will be able to offer several solutions to the problem:
- course of medications;
- surgical intervention.
In the latter case, the patient is opened a skull and the epileptogenic focus is removed. For this, some bones and part of the hard shell of the brain are cut off. After that, the gray matter of the surgeon makes the incisions. They will further prevent the occurrence and spread of epileptogenic impulses. Then the hard shell and bones are put in place. The wound is sutured. Six to seven weeks after surgery, a child from the hospital may return home. After the operation, children with Landau-Kleffner syndrome have improved mental and psychological functions.
Means against seizures
Treatment of the Landau-Kleffner syndrome does not always provide only an operational option. Sometimes medication can help get rid of the disease. It provides for the reception 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 redoubled force. This is especially true for patients who have frequent seizures or atypical absences.
In addition, doctors often recommend "Nicardipine".It helps block calcium channels. Intravenous management of gamma globulin is also beneficial. Suffice to prove themselves and such drugs: "Ethosuximide", "Valproic acid".Help and benzodiazepines, especially "Clobazam" and "Midazolam."But they should be administered intravenously.
The Landau-Kleffner syndrome, the symptoms and treatment of which are described in this article, also provides for the reception of "Diazepam" before bedtime. This drug gives good results. Therapy is prescribed for a period of 6 weeks to avoid tachyphylaxis - a rapid reduction in the effect of repeated use of the medication. Studies have shown that "Levetiracetam" may be useful. It also very well helps in the treatment of pediatric idiopathic focal seizures, the electrical epileptic sleep status, the suppression of a continuous surge in the wave in its slow phase. In addition, the preparation "Felbamat" is successful in the treatment of aphasia and seizures. But with its use, many side effects can develop, among which the most common are aplastic anemia and liver dysfunction.
In the treatment of the Landau-Kleffner syndrome, corticosteroids also help."Adrenocorticotropin" was especially successful. It reduces the manifestation of symptoms of acquired epileptic aphasia, normalizes the EEG state. Long-term therapy with these drugs is recommended for most patients. However, it should be remembered that not all fit the hormonal treatment option.
Often physicians prescribe steroids to patients. For example, "Prednisolone" is administered intravenously for three months. Therapy is used to induce remission. It is important to take into account that the reduction in the dosage of steroids is often associated with persistent relapses of the 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, cataract, kidney failure, depression, manic syndrome and so on. In addition, they are not recommended to appoint patients who have problems with immunity, since they are even more depressing it. They are also contraindicated to people who have aseptic necrosis, tuberculosis or ailments, whose appearance is provoked by the vital activity of roundworms.
A child with a diagnosis of Landau-Kleffner syndrome needs special care and attention. Parents' testimonies testify that with the baby it is necessary to go to classes to the speech therapist-defectologist. The doctor is unable to restore speech functions. But such sessions accustom the crumb to assiduity, educate in it responsibility, organization. Parents say that abroad, such children are prescribed a good anticonvulsant and antiepileptic drug called "Keppra."At us recommend "Depakin", but he often negatively influences a pancreas, therefore some pass to a homeopathic treatment. Many mothers and dads also note that the best remedy for the syndrome is the growing up of the baby. He becomes older - his speech is gradually improving. In addition, with age pass seizures and paroxysms.
As for the doctors, they also continue to study what Landau-Kleffner syndrome is. Photos of patients, videos with their bouts, 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 psychotherapy sessions, speech exercises. Complete recovery of verbal functions is rare. Usually, 2/3 children retain a more or less serious speech defect.