Although we are not usually aware of it, each and every one of us carries out a large number of highly complex cognitive processes. Activities and skills that we generally take for granted and even for simple ones 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 communicating and adapting to life in society.

However, there are a number of disorders, diseases and injuries that can create significant complications in developing and maintaining this ability. This is the case of Landau-Kleffner syndrome , a rare condition that we will discuss in this article.

Landau-Kleffner syndrome: description and symptoms

Landau-Kleffner syndrome is a branch and rare neurological disease of appearance 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 the suffering of 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 already mentioned aphasia, which can be comprehensive (i.e. there are problems in understanding language), expressive (in the production of language) or mixed, after a period of time in which the development of language was normative for the age of the child. In fact, the child may suddenly or progressively lose previously acquired abilities . Most commonly, comprehension problems occur, losing the ability to understand language and even leading to mutism.

Another of the most common symptoms related to the appearance of aphasia (and that in fact largely explains the alterations that generate it) is the suffering of epileptic seizures, which exist in almost three quarters of those 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, 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 on a clinical level.

Behavioral problems-irritability, anger, aggression, and motor agitation, as well as autistic traits-can also, and often do, appear secondarily, although not as a definition of the disorder itself.

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

Causes of this disorder

The causes of this rare disease remain unclear to this day, although there are several hypotheses in this regard.

One of them considers the possibility of a genetic alteration, specifically resulting from mutations in the GRIN2A gene .

Other hypotheses, which do not necessarily contradict the previous one, indicate that the problem may derive from a reaction or alteration of the children’s immune system, and even due to infections such as herpes

Course and forecast

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

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

Full recovery can occur in about a quarter of the cases as long as they are treated. It is much more frequent however that there are small sequelae and speech difficulties. Finally, about a quarter of the patients may 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 because of the problem itself but also because of the lack of development of communication skills during growth.

Treatment

The treatment of this disease requires a multidisciplinary approach , and the problems presented must be treated from different disciplines.

Epileptic-type disorders, although they usually disappear with age, require treatment at the medical level. Anti-epileptic drugs, such as lamotrigine, are generally used for this purpose. Steroids and adrenocorticotropic hormone have also been found to be effective, as well as immunoglobulins. Stimulation of the vagus nerve has also been used on occasion. In some cases surgery may be necessary .

As far as aphasia is concerned, it is going to be necessary to work deeply on the level of speech and language therapy. In some cases it may be necessary to make curricular adaptations or even employ special education schools. Behavioral problems and psychological disorders will also have to be worked on differently.

Finally, the psychoeducation of both the child and his or her parents and environment can favour a better development of the child and a greater understanding and ability to deal with the illness and the complications that it can generate in daily life.

Bibliographic references:

  • Aicardi, J. (1999). Landau-Kleffner syndrome. Rev Neurol.,29: 380-5.
  • Landau-Kleffner Syndrome Association (n.d.). What is SLK? [Online]. Available at: 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 Pozo, D. (2005). Landau-Kleffner syndrome. Presentation of two cases. Cuban Journal of Pediatrics, 77 (2).