LKS Definition and Meaning


According to abbreviationfinder, LKS stands for Landau-Kleffner Syndrome. Landau-Kleffner syndrome is a form of epilepsy that primarily affects childhood patients. The disease is often referred to in medical jargon with the abbreviation LKS. The Landau-Kleffner syndrome usually occurs with a very low frequency and therefore only affects a relatively small number of people. The disease is characterized primarily by the fact that it progressively leads to the loss of the ability to speak in the affected person.

What is Landau-Kleffner Syndrome?

The disease usually begins in childhood. In many cases, the affected children do not show any abnormalities with regard to the development of their motor and language skills until the onset of the disease.

In Landau-Kleffner syndrome, a certain type of epilepsy occurs together with a language disorder. The condition is sometimes also referred to as acquired aphasia associated with epilepsy. Basically, the Landau-Kleffner syndrome is a very rare disease.

When looking at the gender distribution of the disease, it is noticeable that male patients are more frequently affected by Landau-Kleffner syndrome than female patients. In the majority of cases, the disease begins between the ages of 3 and 7 years. While affected children have acquired normal language skills for their age before the onset of Landau-Kleffner syndrome, language loss occurs within a short period of time.

This can be a few days, weeks or a few months. In addition, the EEG findings of the sick children are conspicuous. In addition, epileptic seizures are seen in many cases. The loss of speech and the epileptic seizures vary in time from patient to patient and do not always follow the same pattern.

Some sufferers experience seizures first, while others are more likely to lose speech. Often, however, the disturbances in the ability to speak are evaluated as a consequence of epilepsy.

Causes

At the current time, the exact causes of the development of Landau-Kleffner syndrome are still largely unclear. There is a lack of sufficient studies and research results that address the reasons for the development of the disease. In principle, however, it is assumed that an encephalitic inflammatory process plays a role in the development of the disease.

When carrying out an EEG examination, potentials for epileptic seizures come to light. These focus primarily on certain temporal areas of the cortex. The center for hearing and speaking is also affected.

Symptoms, Ailments & Signs

The symptoms and signs of Landau-Kleffner syndrome are varied and differ from person to person. The disease usually begins in childhood. In many cases, the affected children do not show any abnormalities with regard to the development of their motor and language skills until the onset of the disease.

In principle, the Landau-Kleffner syndrome is characterized by a so-called progressive course. As a rule, the central auditory perception is lost first, which is also referred to as acoustic agnosia. This disorder progresses over time to secondary global aphasia.

In 80 percent of cases, cerebral epileptic seizures occur in parallel . The frequency of seizures varies. Patients often continue to develop cognitively without any limitations. Dementia disorders only occur in a small proportion of patients. Nevertheless, the typical symptoms of Landau-Kleffner syndrome have an enormous impact on the quality of life of those affected.

Because the loss of the ability to speak has a negative effect on communication and thus on the social life of the patient. The school or vocational training as well as the work can usually not be carried out as usual. In addition, the seizures are associated with various risks of injury, so that, for example, driving a car and thus individual locomotion is made more difficult.

Diagnosis & course of disease

In order to diagnose Landau-Kleffner syndrome, various tests can be carried out. At the beginning, however, there is a thorough anamnesis, in which the treating specialist finds out the exact symptoms, past illnesses and characteristics of the patient’s personal lifestyle.

In this way, a suspected diagnosis can already be made. This is strengthened by the typical clinical picture with the characteristic symptoms of Landau-Kleffner syndrome. Various neuropsychological examination methods are used to reliably diagnose Landau-Kleffner syndrome.

For example, an EEG examination is carried out. The results are often inconclusive when awake, but abnormalities occur during the sleep phases. In the context of the differential diagnosis, the Asperger syndrome and the ESES syndrome are particularly relevant.

Complications

Landau-Kleffner syndrome is associated with many different complaints and symptoms. In most cases, the affected children suffer from severe limitations in their language and motor development and therefore need special support at school. Furthermore, those affected can also be so severely restricted in their everyday life that they are permanently dependent on the help of other people.

It is not uncommon for this to lead to epileptic seizures or other muscle problems. The hearing ability of those affected is also relatively often limited, so that the development of the children is further delayed. In general, the patient’s quality of life is significantly reduced due to Landau-Kleffner syndrome. Furthermore, the syndrome promotes the symptoms of dementia. As a rule, the patients cannot work in the usual way and are therefore extremely restricted in their everyday life.

Some of the symptoms of Landau-Kleffner syndrome can be treated with medication. However, there is no generally positive course of the disease, so that those affected are dependent on therapy and the help of other people throughout their lives. As a rule, however, the life expectancy of the person affected is not changed.

When should you go to the doctor?

If adolescents develop motor or language abnormalities, a doctor should be consulted to clarify the cause. If developmental discrepancies and delays in maturation can be noticed in a direct comparison to peers of the same age, the observations should be discussed with a doctor. If there is a reduction or loss of hearing, a check-up is necessary. Hearing loss or one-sided hearing are warning signs of the organism.

They should be examined by a specialist as soon as they are detected. If the symptoms increase in intensity or extent, it is advisable to see a doctor as soon as possible. If the person concerned suffers from epileptic seizures or other convulsive attacks, a doctor should be consulted. Memory disorders are a concern in children, adolescents and young adults. A doctor should be consulted to determine the cause and begin treatment.

Decreased performance in school or work activities is unusual and must be clarified by a doctor. If the symptoms interfere with the performance of everyday tasks, a doctor should be asked for help and support. If the risk of injury increases in everyday life, there is a need for action to prevent further health problems. The reason for the symptoms is found out in a check-up so that the quality of life can then be improved.

Treatment & Therapy

Landau-Kleffner syndrome is usually treated with medication. Drugs such as carbamazepine, valproate and sulthiame are used in the majority of cases. These active ingredients are so-called anticonvulsants. The use of glucocorticoids is also possible.

In order to improve their language skills, the affected patients often receive logotherapy. A prognosis for Landau-Kleffner syndrome is difficult because the course of the disease varies greatly from person to person. Sometimes the symptoms improve, but complete remission is relatively rare.

So far, there are no possibilities for a causal therapy of the disease. Some of the sick patients are affected by the speech disorders in the long term. Other people regain their ability to speak after a while.

Outlook & Forecast

Landau-Kleffner syndrome has an unfavorable prognosis. The course of the disease is considered to be progressive, so that a deterioration in general health can be expected with increasing age. The more often seizures occur, the higher the risk of subsequent disorders or disturbances in general life processes.

The patient and his or her environment must plan their everyday life with consideration for the symptoms of the disease. The affected person loses their ability to speak temporarily or permanently and thus experiences a significant impairment in their quality of life. In a large number of cases, this leads to severe emotional stress and the development of subsequent psychological disorders. In addition, problems in motor development are to be expected. The movement sequences are more difficult and thus also lead to a restriction in everyday life and an increased risk of accidents.

The use of long-term therapies is necessary to alleviate existing symptoms. However, complete freedom from complaints is not achieved. So far, there is no treatment option that could eliminate the cause of Landau-Kleffner syndrome.

The application of self-help measures has a positive influence on the prognosis. Dealing emotionally with the disease is also important in coping with it. Speech can be trained on one’s own initiative to support speech therapy. The exercise units lead to improved coping with everyday life in the long term.

Prevention

To date, no efficient measures for the prevention of Landau-Kleffner syndrome are known. Because the causes have not yet been sufficiently researched. Competent treatment is therefore particularly important.

Aftercare

In the case of Landau-Kleffner syndrome, the options for aftercare in most cases prove to be relatively difficult or sometimes not available at all. Since this disease is a hereditary condition, the person affected should ideally have a genetic test and counseling carried out if they wish to have children, so that the syndrome can no longer appear in their own offspring.

The Landau-Kleffner syndrome cannot heal itself, so a doctor should be contacted at the first signs and symptoms. Treatment is usually through the use of various medications. It is always important to ensure that the dosage is correct and that the medication is taken regularly. Parents should also pay attention to the medication their children are taking.

Since the Landau-Kleffner syndrome often leads to speech problems, many children are dependent on the intensive support and encouragement of their parents. Loving conversations with the patient are often necessary here, so that no depression or other mental upsets can occur. As a rule, Landau-Kleffner syndrome does not negatively affect or reduce the life expectancy of the person affected.

You can do that yourself

The Landau-Kleffner syndrome usually shows up in children, so that those affected are dependent on the help of their guardians. Independent self-help measures are out of the question for the child patients; instead, the parents accompany the medical examinations and therapies. The correct intake of prescribed medication is also primarily monitored by the guardians and not by the underage patients themselves.

The disease results in a variety of restrictions in the everyday life of those affected. The symptoms typical of the disease usually begin with characteristic hearing problems, so that the patients have difficulties, especially in school lessons. Therefore, those affected are often forced to switch to a special school in order to continue their school career. At the same time, the risk of accidents increases in everyday life, for example if the patient no longer properly perceives traffic noise.

Another symptom that develops is severe speech disorders. Visiting a logopedic therapy is helpful, but cannot stop the progressive course. In addition, some patients suffer from epileptic seizures, which further increase the risk of accidents and reduce independence in everyday life. Because of the seizures, many patients are no longer able to drive themselves, for example for safety reasons. Emerging depressions must always be looked after by a psychologist.

Landau-Kleffner Syndrome

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