Landau-Kleffner Syndrome: Symptoms, Causes And Treatment
Although we generally do not realize it, each and every one of us carries out a large number of highly complex cognitive processes. And activities and skills that we generally take for granted and even simple require a large number of interactions between different brain regions that handle different types of information. An example of this is speech, the development of which is very useful to us when it comes to communicating and adapting to life in society.
However, there are different disorders, diseases and injuries that can generate significant complications when developing and maintaining this skill. This is the case of Landau-Kleffner syndrome a rare disease that we are going to talk about in this article.
Landau-Kleffner syndrome: description and symptoms
Landau-Kleffner syndrome is a branch and rare neurological disease that occurs in children , characterized by the appearance of a progressive aphasia at least at the receptive level that generally appears linked to electroencephalographic alterations, which are generally associated with suffering from epileptic seizures. In fact, it is also called epileptic aphasia, acquired epileptic aphasia or aphasia with seizure disorder.
One of the symptoms of this condition is the appearance of the aforementioned aphasia, which can be comprehensive (that is, there are problems in understanding language), expressive (in its production) or mixed, after a period of time in which Language development was normative for the age of the minor. In fact, the child may suddenly or progressively lose previously acquired abilities The most common thing is that comprehension problems occur, losing the ability to understand language and even leading to mutism.
Another of the most common symptoms that is in fact related to the appearance of aphasia (and that in fact explains to a large extent the alterations that generate it) is the suffering of epileptic seizures, which exist in almost three quarters of those affected. . These crises can be of any type, and can appear both unilaterally and bilaterally, both in one area of ​​the brain and at a general level.
The most common thing is that an epileptic seizure appears in or affects the temporal lobe, They tend to be activated during slow sleep and tend to generalize to the rest of the brain There are also cases in which they do not occur, or at least not at a clinical level.
Behavioral problems can also and usually appear secondarily, although it is not something that defines the disorder itself: irritability, anger, aggression and motor agitation, as well as autistic traits.
The symptoms of this disease can appear at any age between 18 months and 13 years, although it is more common between three-four and seven years of age.
Causes of this disorder
The causes of this unusual disease remain unclear to this day, although there are various hypotheses in this regard.
One of them considers the possibility of being faced with a genetic alteration, specifically product of GRIN2A gene mutations
Other hypotheses, which do not have to contravene the previous one, indicate that the problem may arise from a reaction or alteration of the immune system of minors, and even be due to infections such as herpes.
The course of Landau-Kleffner syndrome is usually progressive and fluctuating, Part of the symptoms may disappear with age
Regarding the prognosis, it can vary greatly depending on the case. Generally, part of the symptoms disappear (specifically, epilepsy usually disappears during adolescence), although aphasic problems can remain throughout the subject’s entire life.
Complete recovery can occur in about a quarter of cases as long as they are treated. It is much more frequent, however, that small sequelae remain and speech difficulties. Finally, around a quarter of patients may have serious consequences.
As a general rule, the earlier the onset of symptoms, the worse the prognosis and the greater the possibility of sequelae, not only due to the problem itself but also due to the lack of development of communication skills during growth.
Treatment
The treatment of this disease requires a multidisciplinary approach the problems presented must be treated from different disciplines.
Epileptic-type alterations, although they usually disappear with age, require medical treatment. Antiepileptic drugs, such as lamotrigine, are generally used for this. Steroids and adrenocorticotropic hormone have also been effective, as have immunoglobulins. Vagus nerve stimulation has also been used on occasion. In some cases surgery may be necessary
Regarding aphasia, deep work at the level of logotherapy and language therapy will be necessary. In some cases it may be necessary to make curricular adaptations or even use special education schools. Behavioral problems and psychological alterations must also be worked on differentially.
Finally, psychoeducation of both the minor and their parents and environment can promote better development of the minor and greater understanding and ability to cope with the disease and the complications it can generate on a daily basis.
