Landau-Kleffner syndrome: symptoms, causes and treatment

Although we generally don't realize it, each and every one of us carries out a large number of highly complex cognitive processes. And it is that activities and skills that we generally take for granted and even for 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 for us when it comes to communicating and adapting to life in society.

However, there are different disorders, diseases and injuries that can cause significant complications when it comes to developing and maintaining this ability. This is the case of Landau-Kleffner syndrome., a rare disease that we are going to talk about in this article.

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Landau-Kleffner syndrome: description and symptoms

Landau-Kleffner syndrome is a branch and rare neurological disease of onset in children, characterized by the appearance of a progressive aphasia at least at the receptive level that appears generally linked to electroencephalographic alterations, which are generally associated with suffering from seizures epileptic. 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 sympathetic (that is, there are problems in understanding speech). language), expressive (in the production of this) or mixed, after a period of time in which language development was normative for the age of the child. minor. In fact, the child may suddenly or progressively lose previously acquired abilities. The most common is that there are problems of a comprehensive nature, losing the ability to understand language and can even lead to mutism.

Another of the most common symptoms that in fact is 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, existing in almost three quarters of the affected. These seizures can be of any type, and can appear both unilaterally and bilaterally, both in one area of ​​the brain and at a general level.

Most commonly, epileptic seizures appear in or affect the temporal lobe, they are usually 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.

They can also and usually appear secondarily, although it is not something that defines the disorder itself, behavioral problems: irritability, anger, aggressiveness 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.

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Causes of this disorder

The causes of this unusual disease remain unclear to this day, although there are various hypotheses about it.

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 derive from a reaction or alteration of the immune system of minors, and even be due to infections such as herpes

course and prognosis

The course of Landau-Kleffner syndrome is usually progressive and fluctuating, part of the symptoms may disappear with age.

As far as the prognosis is concerned, it can vary greatly depending on the case. generally disappearing part of the symptoms (specifically epilepsy usually disappears during adolescence), although aphasic problems can remain throughout the life of the child. subject.

Full 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 sequelae.

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

Treatment

Treatment of this disease requires a multidisciplinary approach, and the problems presented must be dealt with from different disciplines.

Epileptic-type disorders, 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 found to be effective, as well as immunoglobulins. The stimulation of the vagus nerve it has also been used on occasion. In some cases surgery may be necessary.

With regard to aphasia, it will be necessary a deep work at the level of logotherapy, and language therapy. In some cases it may be necessary to make curricular adaptations or even use special education schools. Behavioral problems and psychological disorders must also be dealt with differentially.

Finally, the psychoeducation of both the minor and the parents and her environment can favor a better development of the child. and a greater understanding and ability to deal with the disease and the complications that it can generate on a day-to-day basis. day.

Bibliographic references:

• Aicardi, J. (1999). Landau-Kleffner syndrome. Rev Neurol.,29: 380-5.
• Landau-Kleffner Syndrome Association (s.f.). What is the SLK? [On-line]. Available in: http://www.landau-kleffner.org/sindrome-landau-kleffner/.
• Landau, W.M. & Kleffner, F.R. (1957). Syndrome of acquired aphasia with convulsive disorder in children. Neurology, 7: 523-30.
• Nieto, M., López, M.I., Candau, R., L. Ruiz, L., Rufo, M. and Correa, A. (1997). Acquired epileptic aphasia (Landau-Kleffner syndrome). Contribution of 10 cases. Spanish Annals of Pediatrics, 47 (6): 611-617.
• Pozo, A.J., Pozo, D., Carrillo, B., Simón, N., Llanes, M. and Well, D. (2005). Landau-Kleffner syndrome. Presentation of two cases. Cuban Journal of Pediatrics, 77 (2).