- Landau-Kleffner Syndrome (LKS or Infantile Acquired Aphasia) Center
- Patient Comments: Landau-Kleffner Syndrome - Signs
- Patient Comments: Landau-Kleffner Syndrome - Outcome
What is Landau-Kleffner syndrome?
Landau-Kleffner syndrome (LKS) is a childhood disorder. A major feature of LKS is the gradual or sudden loss of the ability to understand and use spoken language. All children with LKS have abnormal electrical brain waves that can be documented by an electroencephalogram (EEG), a recording of the electric activity of the brain. Approximately 80 percent of the children with LKS have one or more epileptic seizures that usually occur at night. Behavioral disorders such as hyperactivity, aggressiveness and depression can also accompany this disorder. LKS may also be called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder. This syndrome was first described in 1957 by Dr. William M. Landau and Dr. Frank R. Kleffner, who identified six children with the disorder.
What are the signs of Landau-Kleffner syndrome?
LKS occurs most frequently in normally developing children who are between 3 and 7 years of age. For no apparent reason, these children begin having trouble understanding what is said to them. Doctors often refer to this problem as auditory agnosiaor "word deafness." The auditory agnosia may occur slowly or very quickly. Parents often think that the child is developing a hearing problem or has become suddenly deaf. Hearing tests, however, show normal hearing. Children may also appear to be autistic or developmentally delayed.
The inability to understand language eventually affects the child's spoken language which may progress to a complete loss of the ability to speak (mutism). Children who have learned to read and write before the onset of auditory agnosia can often continue communicating through written language. Some children develop a type of gestural communication or sign-like language. The communication problems may lead to behavioral or psychological problems. Intelligence usually appears to be unaffected.
The loss of language may be preceded by an epileptic seizure that usually occurs at night. At some time, 80 percent of children with LKS have one or more seizures. The seizures usually stop by the time the child becomes a teenager. All LKS children have abnormal electrical brain activity on both the right and left sides of their brains.
How common is Landau-Kleffner syndrome?
More than 160 cases have been reported from 1957 through 1990.
What causes Landau-Kleffner syndrome?
The cause of LKS is unknown. Some experts think there is more than one cause for this disorder. All of the children with LKS appear to be perfectly normal until their first seizure or the start of language problems. There have been no reports of children who have a family history of LKS. Therefore, LKS is not likely to be an inherited disorder.
What is the outcome of Landau-Kleffner syndrome?
There have not been many long-term follow-up studies of children with LKS. This lack of evidence, along with the wide range of differences among affected children, makes it impossible to predict the outcome of this disorder. Complete language recovery has been reported; however, language problems usually continue into adulthood. The continued language problems can range from difficulty following simple commands to no verbal communication. If recovery takes place, it can occur within days or years. So far, no relationship has been found between the extent of the language impairment, the presence or absence of seizures and the amount of language recovery. Generally, the earlier the disorder begins, the poorer the language recovery.
Most children outgrow the seizures, and electrical brain activity on the EEG usually returns to normal by age 15.
What treatments are available?
Medication to control the seizures and abnormal brain wave activity (anticonvulsants) usually has very little effect on language ability. Corticosteroid therapy has improved the language ability of some children. Sign language instruction has benefited others.
Where can I get more information?
NIDCD maintains a directory of organizations that can answer questions and provide printed or electronic information on ear infections. Please see the list of organizations at www.nidcd.nih.gov/directory.
Use the following keywords to help you search for organizations that are relevant to Landau-Kleffner Syndrome:
- Landau-Kleffner syndrome
- Rare disorders
For more information, additional addresses and phone numbers, or a printed list of organizations, contact:
NIDCD Information Clearinghouse
1 Communication Avenue
Bethesda, MD 20892-3456
Toll-free Voice: (800) 241-1044
Toll-free TTY: (800) 241-1055
Fax: (301) 770-8977
E-mail: [email protected]
Daily Health News
Subscribe to MedicineNet's Children's Health & Parenting Newsletter
Last update: 9/21/2007
SOURCE: U.S. National Institutes of Health, National Institute on Deafness and Other Communication Disorders
Landau-Kleffner Syndrome - Signs
What were the signs and symptoms associated with your child's Landau-Kleffner syndrome?Post View 1 Comment
Landau-Kleffner Syndrome - Treatment
What kinds of treatment did you or your child receive for Landau-Kleffner syndrome?Post
Landau-Kleffner Syndrome - Outcome
Please describe your experience with Landau-Kleffner syndrome, including the outcome.Post View 2 Comments
Top Landau-Kleffner Syndrome Related Articles
AphasiaAphasia is a condition that is the result of damage to portions of the brain. It can be caused by stroke, head injury, brain tumor, or infection. There are two types of aphasia, fluent and non-fluent. Some patients may fully recover from aphasia over time, while others may not.
Autism and CommunicationAutism in children and adults is a developmental disorder, characterized by impaired development in communication, social interaction, and behavior. Autism is classified as a pervasive developmental disorder (PDD), which is part of a broad spectrum of developmental disorders affecting young children and adults. There are numerous theories and studies about the cause of autism. The treatment model for autism is an educational program that is suitable to an individual's developmental level of performance. There is no "cure" for autism.
Children's HealthChildren's health is focused on the well-being of children from conception through adolescence. There are many aspects of children's health, including growth and development, illnesses, injuries, behavior, mental illness, family health, and community health.
DeafnessHearing loss (deafness) may be present at birth or it may manifest later in life. Deafness may be genetic or due to damage from noise. Treatment of deafness depends upon its cause. Sensorineural hearing loss can be caused by conditions affecting the:
- eighth cranial nerve,
- spinal cord, or
- Meniere's disease,
- noise-induced hearing loss
- hearing loss of aging (presbycusis),
- nerve injury from syphilis,
- hearing loss of unknown cause (idiopathic hearing loss),
- nerve tumors, and
- drug toxicity (such as aspirin and aminoglycosides).
Depression in ChildrenChildhood depression can interfere with social activities, interests, schoolwork and family life. Symptoms and signs include anger, social withdrawal, vocal outbursts, fatigue, physical complaints, and thoughts of suicide. Treatment may involve psychotherapy and medication.
Epilepsy TreatmentEpileptic seizures mostly controlled through drug therapy, particularly anticonvulsant drugs. Types of treatment prescribed depends on several factors including the frequency and severity of the seizures as well as the person's age, overall health and medical history. Other forms of treatment options also exist including ketogenic diet and electrical stimulation.
Pediatric Epilepsy SurgeryThere are many different brain disorders associated with epilepsy in children including congenital or acquired. There are three types of pediatric epilepsy surgery:
- resective epilepsy surgery,
- corpus callosotomy, and
- vagus nerve stimulation.
SeizureEpilepsy is a brain disorder in which the person has seizures. There are two kinds of seizures, focal and generalized. There are many causes of epilepsy. Treatment of epilepsy (seizures) depends upon the cause and type of seizures experienced.
Sign LanguageAmerican Sign Language (ASL) is a complete, complex language that employs signs made with the hands and other movements, including facial expressions and postures of the body. No one form of sign language is universal. For example, British Sign Language (BSL) differs notably from ASL. Different sign languages are used in different countries or regions.
Surgical Options for EpilepsyEpilepsy is mostly treatable with medication, but this method is not effective for about 30% of patients. In some cases, brain surgery may be an option. Epilepsy surgery is an operation on the brain to control seizures and improve the person's quality of life.