Landau-Kleffner syndrome: symptoms, causes and treatment

Although we usually don’t realize this, each of us goes through many very complex cognitive processes. And is that the activities and skills that we usually take for granted and even simple require a lot of interactions between different regions of the brain that handle different types of information. An example of this is speaking, development is very useful to us when it comes to communicating and adjusting to life in society.

However, there are different disorders, illnesses and injuries that can lead to major complications in the development and maintenance of this skill. This is the case with Landau-Kleffner syndrome, A rare disease that we will talk about in this article.

  • Related article: “The differences between syndrome, disorder and disease”

Landau-Kleffner syndrome: description and symptoms

Landau-Kleffner syndrome is a branch and a rare neurological disease of onset in childrenCharacterized by the appearance of a progressive aphasia at least at the receptive level which generally appears linked to electroencephalographic alterations, generally associated with the suffering of epileptic seizures. In fact, it is also called epileptic aphasia, acquired epileptic aphasia or convulsive aphasia.

One of the symptoms of this condition is the onset of the aforementioned aphasia, which can be global (that is, there are problems with understanding the language), expressive (in the production of this) or mixed, then a period of time in which the language development was normative for the child’s age. In fact, the child may suddenly or gradually lose previously acquired skills. The most common is that there are understandable problems, the loss of the ability to understand the language and can even lead to silence.

Another of the most common symptoms or which in fact is linked to the onset of aphasia (and which in fact largely explains the alterations that generate it) is the suffering of epileptic seizures, since you are in practically three quarters of those affected. These seizures can be of any type and can occur both unilaterally and bilaterally in one region of the brain and at a generalized level.

The most common is that at the epileptic seizure appears or affects the temporal lobe, they usually activate during slow sleep and tend to spread to the rest of the brain. There are also cases where they do not occur, or at least not at the clinical level.

Behavioral problems: irritability, anger, aggressiveness and motor agitation, as well as autistic traits, can and do appear secondarily, although this is not a determinant of the disorder itself.

Symptoms of this disease can appear at any age between 18 months and 13 years, although it is most common between three and four and seven years.

    Causes of this disorder

    The causes of this unusual disease are still unclear today, although there are several hypotheses.

    One of them is studying the possibility of being confronted with a genetic disorder, in particular product of mutations in the GRIN2A gene.

    Other hypotheses, which should not contradict the above, indicate that the problem may be due to a reaction or an alteration in the immune system of minors, and even be due to infections such as herpes.

    Prices and forecasts

    The course of Landau-Kleffner syndrome is generally gradual and fluctuating, some of the symptoms may go away with age.

    As for the prognosis, it can vary considerably from case to case. a generally disappearing part of the symptomatology (in particular epilepsy usually resolves in adolescence), although aphasic problems may persist throughout the subject’s life.

    Full recovery can occur in about a quarter of cases as long as they are treated. However, it is much more common for small sequelae to remain and speech difficulties. Finally, about a quarter of patients can have severe sequelae.

    As a general rule, the earlier the symptoms appear, the worse the prognosis and the greater the possibility of sequelae, not only due to the problem itself, but due to the lack of development of communication skills during growth. .

    treatment

    Treatment of this disease requires a multidisciplinary approach, After dealing with different disciplines the problems presented.

    Seizure disorders, although they usually go away with age, require medical treatment. Antiepileptic drugs, such as lamotrigine, are usually used for this purpose. Steroids and corticotropin have also been shown to be effective, as have immunoglobulins. Vague nerve stimulation has also been used occasionally. In some cases, surgery may be necessary.

    As for aphasia, in-depth work was needed in terms of logotherapy and language therapy. In some cases, it may be necessary to adapt curricula or even to employ special education schools. Behavioral problems and psychological disorders also need to be treated differently.

    Finally, the psychoeducation of the child and his parents and his environment can promote better development of the child and a better understanding and ability to cope with the disease and the complications it can cause in the lives of all. days.

    Bibliographical references:

    • Aicardo, J. (1999). Landau-Kleffner syndrome. Rev Neurol., 29: 380-5.
    • Landau-Kleffner Syndrome Association (sf). What is SLK? [Online]. Available at: http://www.landau-kleffner.org/sindrome-landau-kleffner/.
    • Landau, WM and Kleffner, FR (1957). Acquired aphasia syndrome with seizure disorder in children. Neurology, 7: 523-30.
    • Nieto, M., López, MI, Candau, R., L. Ruiz, L., Rufus, M. and Correa, A. (1997). Acquired epileptic aphasia (Landau-Kleffner syndrome). Contribution of 10 cases. Spanish Annals of Pediatrics, 47 (6): 611-617.
    • Pou, AJ, Pou, D., Carrillo, B., Simó, N., Llanes, M. and Pou, D. (2005). Landau-Kleffner syndrome. Presentation of two cases. Cuban Journal of Pediatrics, 77 (2).

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