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Landau-Kleffner Syndrome (LKS) - Facts and Information
Information provided by Thomas C. Weiss - Published: 2010-04-14
Children with LKS develop regularly, but then lose the language skills they have gained, apparently for no reason.
Defining Landau-Kleffner Syndrome
Landau-Kleffner Syndrome, also known as, ‘Acquired Epileptiform Aphasia,’ is a form of rare, childhood neurological disorder that is characterized by the sudden or gradual development of aphasia. Aphasia is the inability to understand or express language. Landau-Kleffner syndrome (LKS) also finds people affected by it presenting with an abnormal electroencephlogram (EEG) results. LKS affects the parts of the person’s brain that control their speech and comprehension. The disorder commonly occurs in children who are between the ages of five and seven years old.
Children with LKS develop regularly, but then lose the language skills they have gained, apparently for no reason. Although a number of the people affected by the disorder experience seizures, some of them do not. The disorder is hard to diagnose and might be misdiagnosed as autism, hearing impairment, pervasive developmental disorder, auditory/verbal processing disorder, learning disability, childhood schizophrenia, mental retardation, or emotional or behavioral problems.
The year 1957 found Landau and Kleffner describing six children with a, “syndrome of acquired aphasia with convulsive disorder.” The language disorder was later something that was recognized as being different from common childhood aphasia, which is usually expressive. The aphasia associated with LKS is an, ‘auditory-verbal agnosia.’ The language disorder that is a part of the syndrome was at first considered to be a seizure manifestation because epileptiform activity from one or both of the person’s temporal lobes is often present on their EEG recordings. Seizure activity is not a consistent feature, and when it is present, it may either precede or follow the onset of the language disorder.
Mantovani and Landau continued to follow the six children, as well as four others, and discovered that the outcome was variable. Five of the children recovered good language abilities as they became adults. Their hypothesis that outcome correlated with the frequency of seizure activity was not substantiated by subsequent reports.
The 1989 International League Against Epilepsy placed LKS in the classification of, ‘Epilepsies and syndromes undetermined as to whether they are focal or generalized.” The same category includes the continuous spikes and waves during slow sleep. Recent years have found a number of common features between the two syndromes being recognized, finding questions being raised as to whether they are two distinct entities, or subclasses of a single syndrome.
Causes of LKS
Medical science has not discovered the cause of LKS. Some experts think there may be more than one cause of the disorder. Children with the syndrome seem to be fine until they experience their first seizure, or begin experiencing language difficulties. There are no reports of children with a family history of the syndrome. Researchers do not believe that LKS is likely to be a form of inherited disorder. There have been greater than one-hundred and sixty reported cases of LKS between the years of 1957 and 1990.
There have been isolated cases of LKS that were associated with overt cerebral pathology such as ateritis, acute inflammatory disease, tumors, cysticercosis, and arachnoid cyst. A study in a surgical series of fourteen people with LKS found a number of abnormalities. Despite these findings, the language symptomatology is interpreted as a dysfunctional disorder associated with the bilateral EEG discharges, and deafferentation of temporal cortex. The people who have experience benign childhood epilepsy with centrotemporal spikes that has evolved into LKS, as well as the prompt response to anti-epileptic medications in some people, also points to a secondary bilateral synchrony type of phenomenon.
Symptoms of LKS
LKS is a syndrome that most frequently occurs in children who are developing regularly and are between five and seven years of age. Apparently for no reason, these children start experiencing difficulties with understanding what is said to them. Many doctors refer to this problem as, ‘auditory agnosia,’ or, ‘word deafness.’ The auditory agnosia can happen slowly, or very rapidly. Parents of children with LKS many time believe their child is developing a hearing problem, or has suddenly become deaf. After pursuing hearing testing; however, their children present with average hearing. Children with LKS can also appear to be developmentally delayed or autistic.
The inability to understand language on the part of children with LKS eventually affects their spoken language, which may then progress to a complete loss of their ability to speak. Children with the syndrome who had learned to read and write prior to the onset of auditory agnosia may often continue communicating through written language. Some children with the disorder develop a form of gestural communication or sign-like language. The communication issues can lead to behavioral or psychological issues. The child’s intelligence is something that usually remains unaffected.
The loss of language experienced by children with LKS might be preceded by a seizure that commonly occurs during the night. At some point, eighty-percent of children with the syndrome experience one or more seizures. The seizure activity usually ceases by the time the child reaches adolescence. Every child with LKS presents with abnormal electrical brain activity on both the right and left sides of their brains.
Diagnosing LKS
LKS is something that can be hard to diagnose, it can also be misdiagnosed as autism, hearing impairment, pervasive developmental disorder, learning disability, learning impairment, attention deficit disorder, auditory/verbal processing disorder, childhood schizophrenia, mental retardation, behavioral issues, or emotional problems.
Treatment of LKS
Treatment of LKS commonly consists of the administration of medications that include corticosteroids and anticonvulsants. A doctor may also order speech therapy, something that should be started early. There is a controversial form of treatment involving a surgical technique called, ‘multiple subpial transection,’ in which the pathways of abnormal electrical brain activity are severed.
The prognosis of children with this syndrome varies. While some children affected by LKS might experience a permanent and severe language disorder, other can regain a great deal of their language abilities, even though it might take them months or even years to do so. Some children with LKS experience remission, and relapse can occur. Children with LKS who experience an onset of the syndrome after they have reached the age of six have an improved prognosis if speech therapy is started early. Seizures commonly disappear by the time a child with LKS has reached adulthood.
Unfortunately, there have not been many long-term follow up studies of children with this syndrome. The lack of evidence, combined with the range of differences between the children affected with the syndrome, makes it impossible to predict the outcomes of the disorder. There have been reports of complete language recovery, although language issues commonly continue into adulthood. The language problems experienced by children with LKS can range from the following of simple commands to a complete lack of verbal communication. Should a recovery occur, it may happen within days or even years. To date, there has been no relationship found between the extent of the language impairment, the presence or absence of seizure activity, and the extent of the child’s language recovery. In general, the earlier the disorder starts, the poorer the child’s recovery will be. The majority of children with LKS outgrow the seizures they experience. Their electrical brain activity and EEG results commonly return to average by the time they reach adolescence.
- This web page is from the Disabled World Pediatric Health Concerns section which provides: There are a number of health concerns children face today these pediatric conditions are the most common chronic childhood diseases.
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