Landau-Kleffner syndrome (LKS), also known as acquired epileptiform aphasia, is a rare childhood disorder characterized by a deterioration in language skills in association with abnormal electrical brain waves, which can be documented by an EEG. The disorder typically affects children, between the ages of 3 and 7 years, who previously had no developmental, language, or interactional difficulties. The children usually experience an abrupt loss of language and comprehension and a diminution in their ability to express themselves. Approximately 70-80% of children with LKS have seizures, however, the presence of seizures is not a requirement for a diagnosis.
LKS was first described in 1957 by Dr. W.M. Landau and Dr. F.R. Kleffner, however, the cause of LKS is still unknown. While in some cases LKS is believed to result from a lack of full development of the networks within the brain, other cases seem to be precipitated by a viral infection. As there have been no reports of children who have a family history of LKS, it is not likely to be an inherited disorder. Males are more affected with LKS than females by a ratio of 2:1.
Features and Characteristics
- Abnormal EEG
- Aphasia (diminished understanding of language); many children are unable to understand their own name and have difficulty understanding environmental sounds, such as a telephone ringing or dog barking
- Expressive language difficulties (the ability to speak is often seriously affected; some children lose their speech completely)
- Seizures (typically tonic clonic or complex partial seizures and occurring at night)
- Behavioral problems (hyperactivity, aggressiveness, decreased attention, easily distracted, temper tantrums, and social withdrawal)
- Autistic-like behaviors (avoidance of human contact, extreme pickiness over food, sleep disturbances, insensitivity to pain, and bizarre, inappropriate repetitive play)
- Loss of bladder and bowel control
- Visual disturbances (difficulty recognizing family and friends or common objects)
LKS is often difficult to diagnose and some children may be misdiagnosed as having autism, pervasive developmental disorder, a hearing impairment, learning disabilities, attention deficit disorder, a mental illness, emotional/behavioral problems, or mental retardation. A diagnosis of LKS should be considered when a child suddenly begins to develop a difficulty in communicating (prior to this occurrence, they have experienced normal development), they show a difficulty understanding what is said to them, and have difficulty putting their thoughts into words. The diagnosis can be confirmed by an EEG, most likely when the child is asleep. The EEG will show signs of brain malfunction involving both cerebral hemispheres, but usually the spike and wave activity will be more prominent in the dominant cerebral hemisphere, which deals with language function. For most, this is on the left side.
There is no cure for LKS, therefore, the treatment program involves managing the childs symptoms. Many children with LKS will be treated with anticonvulsants to control their seizures. Typically these medications will be effective in controlling the seizures and improving the EEG. For those who have persistent seizures associated with persistent language difficulties, neurosurgery can be an effective method of treatment.
Since speech is greatly affected by LKS, speech therapy should be provided to the child as early as possible. Sign language should be used with the child who has little or no language comprehension. In addition to traditional speech therapy, some children have shown improvements in their language abilities when treated with steroids. Many children with LKS are in special education classrooms that are equipped to provide an appropriate education to those with severe speech and language disorders.
What to Expect
The long-term prognosis for children with LKS is good. The seizures, abnormal EEG, behavioral problems, and other problems usually decrease or resolve completely as the child reaches adolescence. Some individuals with LKS will recover completely, while others will continue to have language difficulties. Generally, the later the child develops LKS, the greater the chance of him or her having a complete recovery.
In January 1996, Tyler was born a strapping 9 lb boy with all fingers and toes in the right places. From the outset, he was different; he screamed continually and never slept. After a variety of comical diagnoses from well-meaning specialists, we settled down to a routine of how our life was going to be: sleepless.
His development was well advanced for his age; he walked at 8 months and was talking by 12 months. Our local mothers group constantly remarked on how they could not possibly have him as their child. They were exhausted just watching him as he raced around the living room whilst their kids were just working out how to crawl.
His advanced language skills were a godsend. We were able to tell him about hot, cold, no and danger so that he was relatively safe whilst roaming around the house. At 12 months he was given an MMR shot and immediately suffered 17 seizures in 48 hours. He became virtually deaf and mute overnight and spent most of his time rocking and collecting all the blue things in the house. He found walking a difficulty.
The immunization issue is a very contentious issue here in Australia, which is why we had deliberately not entered into this debate in regards to the LKS. A significant number of LKS children have had a reaction after an MMR shot, so research is currently under way in this area.
After many confused diagnoses, we stumbled upon several disorders that we felt might be appropriate labels for Tyler. Each one was methodically tested through the process of elimination and by examination of the new symptoms that Tyler presented. We were able to confirm it was most probably LKS.
Tyler then spent the first year of his LKS diagnosis in a six weekly cycle of gaining speech and some understanding, and then losing it almost immediately when a run of seizures began. This was infuriating, as he would struggle for weeks to gain a sound and just as he got it, it was gone. Thankfully his second year was more stable; a two steps forward, one step back approach, but there is no guarantee that this will remain so, as things can change very quickly with LKS.
In the meantime, Tyler was not waiting around for the doctors to work out what to do next. He decided to sign to us what his needs were. We obtained a sign language book and off we went. Not ones to mess around, Tyler had 67 signs within 3 months of us starting to work with him.
We then embarked on the hardest period of our entire lives. Had we known, we probably would have moved countries instead. It made sense to us that if Tyler could not hear or speak for long periods of time then to communicate in sign language seemed the most appropriate alternative. However, we do not live anywhere near a government signing school and Tylers diagnosis restricted his access to the private school near by. After much lobbying, he gained access to that school where he is today. The cost was high and is still ongoing.
During this period, we made ourselves available to other parents for contact if they needed any help gaining access to signed services. We had been told that Tyler was probably the only one in the country with this disorder; we were isolated and alone in our struggles. The doctors could not have been more wrong. Very slowly, one, two, three, four families contacted us. Many traveled the length and breath of the country to ask questions. Does your child do this? Does your child do that? More importantly, there was so little information on this disorder that many parents did not even know the basics, let alone the academic debate that rages around these kids. As these parents arrived at our home, we were able to trade information until we had a large amount of literature on the subject, which we were able to pass around to other parents.
As these families came to visit, we realized that we were all in the same boat rowing a hundred miles a minute alone. We decided to start a newsletter, whereby parents could exchange details of their struggles and ask advice from other parents about how to handle this or that. Medical and educational aspects were naturally a high priority.
The newsletter was a phenomenal success. Our 12 parents became 50 in one month, 100 by the second month and 400 by the third month. What started out as parents helping each other, became a medical and educational newsletter, helping early intervention services and the medical professionals assist both with diagnosing these children and also providing hope via a managed educational and rehabilitation program. Unlike the rest of the world, Australia has very few services to offer disabled children, and those who obtain private intervention pay quite heavily for it (in the overall cost and in the inexperience of their newly trained operators; fresh out of a training program in another country, keen with new ideas, but restricted in their knowledge of how the Australian system works).
As the months went on, many new members joined, but the same questions still came up with new parents: how, what and where can we get services for our LKS children? It was during this time that we found FOLKS in the UK and joined lks@onelist, an LKS Internet mailing list where over 100 LKS parents were waiting to help us get the services we needed by sharing and exchanging ideas on a "been there done that" format. These parents were so pro-active that no stone has yet to be left unturned in their endeavor to gain access to what they need for their child. Regardless of where we were in the world, literature was exchanged at an alarming rate. This of course made us realize how isolated Australian families really were and how little most parents knew about their rights to access services for their children.
As a result of the success of the newsletter and the phenomenal amount of literature we have, we are creating a book. The aim of this book is to help parents help others to understand LKS. We hope that this book also provides the medical profession and educational experts with the information they need in order to provide what is best for these children. It is vital that experts take the lead from parents; they know their child best and have seen the very best and very worst of their children. Parents know intuitively what is best for their child. - Richard and Katherine Price
If you are interested in meeting other parents and individuals who are involved in raising a child with Landau-Kleffner syndrome, the following listserv is available:
LKS E-mail List - To subscribe, click here: http://www.bobjanet.demon.co.uk/lks/folks.html#response
For more information on Landau-Kleffner syndrome, please see the following references:
- University of North Carolina http://www.unc.edu/depts/teacch/lk.htm
- Albert Einstein College of Medicine http://bulky.aecom.yu.edu/epilepsy/lanklef.html
- Friends of Landau-Kleffner Syndrome (FOLKS) http://www.bobjanet.demon.co.uk/lks/folks.html
- Online Mendelian Inheritance in Man http://www3.ncbi.nlm.nih.gov/htbin-post/Omim/dispmim?245570
- British Epilepsy Association http://www.epilepsy.org.uk/info/landafrm.html
- National Institutes of Neurological Disorders and Stroke http://www.ninds.nih.gov/patients/disorder/landau/landau.htm
- National Institute on Deafness and Other Communication Disorders http://www.nih.gov/nidcd/health/pubs_vsl/landklfs.htm
- The Hyman-Newman Institute for Neurology and Neurosurgery http://www.nyneurosurgery.org/cfr/epilepsy/types/landau-kleffner.htm
The Disorder Zone has been created for educational purposes only and is not intended to serve as medical advice. The information provided in The Zone should not be used for diagnosing or treating a health problem or disease. It is not a substitute for professional care. If your child has any health concerns, please consult your health care provider.
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