Landau-Kleffner syndrome is a form of epilepsy that primarily affects childhood patients. The condition is often referred to in medical jargon by the abbreviation LKS. Landau-Kleffner syndrome usually occurs with a very low frequency and for this reason affects relatively few people. The disease is primarily characterized by the fact that it leads to progressive loss of speech in affected individuals.
What is Landau-Kleffner syndrome?
In Landau-Kleffner syndrome, a specific type of epilepsy occurs together with a disorder of speech. The condition is also called acquired aphasia associated with epilepsy in some cases. Basically, Landau-Kleffner syndrome is a very rare disease. In the gender distribution of the disease, it is noticeable that male patients are more frequently affected by Landau-Kleffner syndrome than females. In the majority of cases, the disease begins between the ages of 3 and 7. While the affected children have acquired normal language skills for their age before the onset of Landau-Kleffner syndrome, a loss of language occurs within a short period of time. This may be a matter of a few days, weeks or a few months. In addition, the EEG findings of the affected children are striking. In addition, epileptic seizures show up 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. In some affected individuals, seizures occur first; in others, the loss of speech presents sooner. Often, however, the speech disturbances are judged to be a consequence of the epilepsy.
Causes
At the current time, the exact causes of Landau-Kleffner syndrome are still largely unclear. There is a lack of sufficient studies and research findings that address the reasons for the development of the disease. However, it is generally suspected that an encephalitic inflammatory process plays a role in the development of the disease. When an EEG examination is performed, potentials for epileptic seizures are revealed. These are primarily concentrated in certain temporal areas of the cortex. The center for hearing and speech are also affected.
Symptoms, complaints, and signs
The symptoms and signs of Landau-Kleffner syndrome are varied and differ from individual to individual. Usually, the disorder begins in childhood. In many cases, affected children do not show any abnormalities in the development of their motor and language skills until the onset of the disease. In principle, Landau-Kleffner syndrome is characterized by a so-called progressive course. As a rule, central auditory perception is lost first, which is also referred to as acoustic agnosia. Over time, this disorder develops into secondary global aphasia. In 80 percent of cases, cerebral epileptic seizures occur in parallel. The frequency of seizures varies. Often, the affected individuals continue to develop cognitively without impairment. Only a small proportion of patients develop dementia. Nevertheless, the typical symptoms of Landau-Kleffner syndrome have an enormous impact on the quality of life of the affected persons. This is because the loss of the ability to speak has a negative effect on communication and thus on the social life of the patients. Schooling or vocational training as well as work are usually not feasible as usual. In addition, seizures are associated with various risks of injury, so that, for example, driving a car and thus individual locomotion is also made more difficult.
Diagnosis and course of the disease
To diagnose Landau-Kleffner syndrome, the performance of various examinations comes into question. At the beginning, however, a thorough anamnesis is performed, during which the attending specialist learns about the patient’s exact complaints, past illnesses, and characteristics of the patient’s personal lifestyle. In this way, a tentative 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 for a reliable diagnosis of Landau-Kleffner syndrome.For example, an EEG examination is performed. The results are often without findings in the awake state, but abnormalities occur during the sleep phases. In the context of differential diagnosis, Asperger syndrome and ESES syndrome are particularly relevant.
Complications
Landau-Kleffner syndrome is associated with many different complaints and symptoms. In most cases, affected children suffer from severe limitations in their language and motor development in the process and therefore require 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 epileptic seizures or other muscular complaints to occur. The hearing ability of those affected is also relatively often restricted, so that the development of the children continues to be delayed. In general, the patient’s quality of life is significantly reduced due to Landau-Kleffner syndrome. Furthermore, the syndrome favors the symptoms of dementia. Patients are usually unable to function in their usual manner and are extremely limited in their daily lives as a result. Some of the complaints of Landau-Kleffner syndrome can be treated with the help of medication. However, a general positive course of the disease does not occur, so that those affected are dependent on therapy and the help of other people for the rest of their lives. As a rule, however, the life expectancy of the affected person is not changed.
When should you see a doctor?
If adolescents develop motor or speech abnormalities, a doctor should be consulted to determine the cause. If developmental inconsistencies and delays in maturation can be perceived in direct comparison to peers, observations should be discussed with a physician. If there is a reduction or loss of hearing, a check-up is needed. Hearing loss or unilateral hearing are warning signs of the organism. They should be examined by a specialist immediately after they are detected. If the symptoms increase in intensity or extent, a visit to the doctor is advisable as soon as possible. If the affected person suffers from epileptic seizures or other convulsive attacks, a doctor should be consulted. Impaired memory is a cause for concern in children, adolescents and young adults. A physician should be consulted so that the cause can be determined and treatment initiated. Decreased performance in school or work activities is uncommon and must be evaluated by a physician. If the symptoms cause impairment in performing everyday tasks, a physician should be consulted for help and support. If the risk of injury increases in everyday life, action is required to prevent further health disorders. A follow-up examination will find out the reason for the symptoms so that an improvement in quality of life can subsequently be made.
Treatment and therapy
Landau-Kleffner syndrome is usually treated with medication. Drugs such as carbamazepine, valproate, and sultiam are used in the majority of cases. These drugs are so-called anticonvulsants. The use of glucocorticoids is also possible. To improve language skills, affected patients often receive logotherapy. A prognosis for Landau-Kleffner syndrome is difficult, as the course of the disease varies greatly from person to person. Sometimes the symptoms improve, but complete remission is relatively rare. Also, there are no possibilities for a causal therapy of the disease. Some of the patients are affected by the speech disorders in the long term. Other individuals regain their speech after some time.
Outlook and prognosis
Landau-Kleffner syndrome has an unfavorable prognosis. The course of the disease is considered progressive, so general health can be expected to deteriorate with age. The more frequent seizures occur, the higher the risks for secondary disorders or disturbances of general life processes. The patient as well as his environment must plan the organization of everyday life with consideration for the symptoms of the disease.The affected person temporarily or permanently loses his ability to speak and thus experiences a significant impairment of his quality of life. In a large number of cases, this leads to severe emotional stress and the development of psychological sequelae. In addition, problems with motor development are to be expected. The movement sequences are more difficult and thus also lead to a restriction in everyday life as well as an increased risk of accidents. The application of long-term therapies is necessary in order to alleviate existing complaints. However, complete freedom from symptoms is not achieved. So far, there is no therapeutic option that could eliminate the cause of Landau-Kleffner syndrome. The application of self-help measures has a positive influence on the prognosis. In addition, the emotional handling of the disease is important in coping. Speech can be trained on one’s own initiative in support of speech therapy. The exercise sessions lead to improved everyday coping in the long term.
Prevention
Nowadays, no efficient measures for the prevention of Landau-Kleffner syndrome are known yet. This is because the causes have also not yet been adequately researched. Therefore, competent treatment is particularly significant.
Follow-up
In the case of Landau-Kleffner syndrome, the possibilities for follow-up care prove to be relatively difficult in most cases or are sometimes not available at all. Since this disease is hereditary, the affected person should ideally have genetic testing and counseling if he or she wishes to have children to prevent the syndrome from occurring in his or her own offspring. Landau-Kleffner syndrome cannot cure itself, so a doctor should be contacted at the first signs and symptoms. Treatment is usually carried out by taking various medications. It is always important to pay attention to the correct dosage and to take the medication regularly. Parents should also pay attention to their children’s intake of medication. Since Landau-Kleffner syndrome often leads to speech difficulties, many children are dependent on intensive support and encouragement from their parents. Loving conversations with the patients are often necessary to prevent depression or other psychological upsets. As a rule, Landau-Kleffner syndrome does not negatively affect or reduce the life expectancy of the affected person.
What you can do yourself
Landau-Kleffner syndrome usually manifests in children, so affected individuals rely on their guardians for help. Independent self-help measures are out of the question for child patients; instead, parents accompany medical examinations and therapies. The correct intake of prescribed medication is also primarily monitored by the parents and not by the underage patients themselves. The disease results in a variety of restrictions in the everyday life of those affected. In most cases, the symptoms typical of the disease begin with characteristic hearing problems, so that the patients have difficulties especially in school lessons. As a result, those affected are often forced to transfer to a special school in order to continue their school career. At the same time, the risk of accidents increases in everyday life when, for example, the patients no longer perceive sounds in traffic correctly. Another symptom is the development of severe speech disorders. Attending speech therapy is helpful in this case, but cannot stop the progressive course. In addition, some patients suffer from epileptic seizures, which further increase the risk of accidents and reduce their independence in everyday life. Because of the seizures, many patients are no longer able to drive themselves, for example, for safety reasons. Emerging depressions should always be managed by a psychologist.
