Landau-Kleffner syndrome: symptoms, causes and treatment.

This neurological disease of childhood is associated with aphasia and behavioral disturbances.

Although we generally do not realize 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 take for granted as 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 when it comes to communicating and adapting to life in society.

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

Landau-Kleffner syndrome: description and symptoms

Landau-Kleffner syndrome is a rare, rare a rare, rare neurological disease of onset in childrenIt is characterized by the appearance of a progressive aphasia at least at the receptive level, usually associated with electroencephalographic disturbances, which are generally associated with the occurrence 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 aforementioned aphasia, which can be comprehensive (i.e. there are problems in language comprehension), expressive (in language production) or mixed, after a period of time in which language development was normative for the child's age. In fact, the child may suddenly or progressively lose the child may suddenly or progressively lose previously acquired language skills.. Most commonly, comprehension problems occur, losing the ability to understand language and may even lead to mutism.

Another of the most common symptoms or that in fact is related to the appearance of aphasia (and that in fact explains to a great extent the alterations that generate it) is the suffering of epileptic seizures, existing in practically three quarters of those affected. These seizures can be of any type, and can appear unilaterally or bilaterally, either in one area of the brain or at a generalized level.

Most commonly, the seizure appears in or affects the temporal lobe, They are usually activated during slow sleep and tend to spread to the rest of the brain.. There are also cases in which they do not occur, or at least not at the clinical level.

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

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 several hypotheses about it.

One of them suggests the possibility of a genetic alteration, specifically the product of mutations in the GRIN2A gene. product of 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 immune system of the children, or even be due to infections such as herpes.

Course and prognosis

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

As for the prognosis, it can vary greatly depending on the case. Usually, part of the symptoms disappear (in particular epilepsy usually disappears during adolescence), although aphasic problems may remain throughout the subject's life.

Complete recovery may occur in about a quarter of the cases as long as they are treated. It is much more common, however, to have minor sequelae and speech difficulties. and speech difficulties. Finally, about a quarter of patients may have severe 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 approachThe treatment of this disease requires a multidisciplinary approach, being necessary to treat the problems presented from different disciplines.

Epileptic disorders, although they usually disappear with age, require medical treatment. Antiepileptic 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. Vagus nerve stimulation has also been used on occasion. Surgery may be necessary in some cases.

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

Finally, psychoeducation of the child as well as the parents and environment can favor a better development of the child and a better understanding and ability to cope with the disease and the complications it can generate in daily life.

Bibliographical references:

• Aicardi, J. (1999). Landau-Kleffner syndrome. Rev Neurol.,29: 380-5.
• Landau-Kleffner Syndrome Association (n.d.): What is LKS? [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. & Correa, A. (1997). Acquired epileptic aphasia (Landau-Kleffner syndrome). Contribution of 10 cases. Anales Españoles de Pediatría, 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. Revista Cubana de Pediatría, 77 (2).