Landau-Kleffner syndrome (patient information)

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 * Associate Editor(s)-In-Chief: Varun Kumar, M.B.B.S.

Overview
Landau-Kleffner syndrome (LKS) is a rare, childhood neurological disorder characterized by the sudden or gradual development of aphasia (the inability to understand or express language) and an abnormal electro-encephalogram (EEG)

What are the symptoms 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.

What causes Landau-Kleffner syndrome?
LKS affects the parts of the brain that control comprehension and speech. The disorder usually occurs in children between the ages of 5 and 7 years. Typically, children with LKS develop normally but then lose their language skills for no apparent reason. While many of the affected individuals have seizures, some do not.

When to seek urgent medical care?
Contact your doctor when you observe the above mentioned symptoms in your child.

Diagnosis
Your doctor may do a physical examination and may suggest following tests to aid in diagnosis:
 * Electroencephalography (EEG): It is a test to assess the electrical activities in brain and may be done when awake and during sleep.
 * Magnetic resonance imaging (MRI): Can be helpful in excluding other causes such as tumors, degenerative diseases of nerves, and brain infections.
 * Brainstem auditory evoked potentials (BAERs)
 * Behavioral hearing tests (BHTs): To analyse the reaction of a child to a sound, generally by using positive reinforcement.

Treatment options
Treatment for LKS usually consists of medications, such as anticonvulsants and corticosteroids, and speech therapy, which should be started early. A controversial treatment option involves a surgical technique called multiple subpial transection in which the pathways of abnormal electrical brain activity are severed

Diseases with similar symptoms

 * Autism
 * Pervasive developmental disorder
 * Hearing impairment
 * Learning disability
 * Auditory/verbal processing disorder
 * Attention deficit disorder
 * Mental retardation
 * Childhood schizophrenia
 * Emotional/behavioral problems.

Where to find medical care for Landau-Kleffner syndrome?
Directions to Hospitals Treating Landau-Kleffner syndrome

What to expect (Outlook/Prognosis)?
The prognosis for children with LKS varies. Some affected children may have a permanent severe language disorder, while others may regain much of their language abilities (although it may take months or years). In some cases, remission and relapse may occur. The prognosis is improved when the onset of the disorder is after age 6 and when speech therapy is started early. Seizures generally disappear by adulthood.