Landau-Kleffner syndrome: symptoms, causes and treatment

Although generally we do not realize it, each and every one of us carries out a large number of highly complex cognitive processes. And is that activities and skills that we generally take for granted and even for simple require a lot of interactions between different brain regions that handle different types of information. An example of this is speech, whose development is very useful when communicating and adapting to life in society.

However, there are different disorders, illnesses and injuries that can generate important complications when 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

The Landau-Kleffner syndrome is a branch and rare neurological disease of appearance in children , characterized by the appearance of a progressive aphasia at least receptive level that appears usually linked to electroencephalographic alterations, which are usually associated with the suffering of epileptic seizures. In fact, it is also called epileptic aphasia, acquired epileptic aphasia or aphasia with convulsive 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 the language), expressive (in the production of this) or mixed, after a period of time in which the development of language was normative for the child's age. 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 the language and can even lead to silence.

Another of the most common symptoms u that is in fact related to the appearance of aphasia (and that in fact largely explains the alterations that generate it) is the suffering of epileptic seizures, existing in almost three quarters of those affected . These crises can be of any type, and can appear both unilaterally and bilaterally in one area of ​​the brain as well as at a generalized level.

The most common is that an epileptic seizure appears in or affects the temporal lobe, they usually activate 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 defining the disorder itself, problems at the behavioral level: irritability, anger, aggression and motor agitation, as well as autistic features.

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 continue to this day without being clear, 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 can be derived from a reaction or alteration of the immune system of minors, and even due to infections such as herpes.

Course and forecast

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

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

Complete recovery can occur in about a quarter of cases as long as they are treated. It is much more frequent, however, that there remain small sequelae and speech difficulties. Finally around a quarter of patients can have serious sequelae.

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 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 dealt with from different disciplines.

Epileptic type alterations, although usually disappear with age, require medical treatment. Generally, antiepileptic drugs, such as lamotrigine, are used for this purpose. Also steroids and adrenocorticotropic hormone have been effective, as well as immunoglobulins. Vagus nerve stimulation has also been used occasionally. In some cases surgery may be necessary .

With regard to aphasia, a deep work will be necessary at the level of speech therapy and speech therapy. In some cases it may be necessary to make curricular adaptations or even to use special education schools. Behavioral problems and psychological alterations should also be worked differentially.

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

Bibliographic references:

• Aicardi, J. (1999). The Landau-Kleffner syndrome. Rev Neurol., 29: 380-5.
• Association Landau-Kleffner Syndrome (s.f.). What is the SLK ?. [On-line]. Available at: //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., Simon, N., Llanes, M. and Pozo, D. (2005). The Landau-Kleffner syndrome. Presentation of two cases. Cuban Journal of Pediatrics, 77 (2).