What is Landau Kleffner Syndrome?
Landau Kleffner syndrome is an uncommon and poorly understood form of childhood epilepsy. It usually starts between the ages of 3 and 8. It may develop slowly over many months or very suddenly.
A major feature is the loss of ability to understand and use spoken language. When it develops in young children who have not yet learnt to talk it may be mistaken for a developmental language disorder, deafness or autism.
For no apparent reason these children begin having trouble understanding what is said to them. This is often referred to as auditory agnosia or “word deafness”. The auditory agnosia may occur slowly or quickly. The inability to understand language eventually affects the child’s spoken language, which may progress to a complete loss of the ability to speak.
Children who have learned to read and write before the onset of auditory agnosia can often continue communication through written language.
Language problems may include:
- Lose all intelligible speech
- Regression of receptive and expressive speech abilities
- May echo back words and phrases
- May delete final consonant sounds and interchange letters
- Voice quality may sound similar to a person with a hearing impairment
- Uses generic speech consisting mainly of nouns
- Varying receptive skills from being able to follow simple verbal commands to a total inability to understand any verbal input
- Gradual increase in misarticulations and a decrease in sentence complexity
- Speech decreases to the use of only single words, jargon or total mutism
- Language problems such as dysnomia (saying hammer for shovel), semantic paraphasia (saying cut for scissors) or reverse compounds (saying lightstop for stoplight)
- If they can hear words, they may hear it differently each time and consequently have great difficulty making the necessary associations to place information into memory storage.
Approximately 50% of children with Landau Kleffner syndrome have a behavioural disorder.
The most common behavioural problems are:
- Outbursts of rage with aggressiveness
- Gestural sterotypies
- Avoidance of interpersonal contact
- Sleep disturbances
- Mouthing of objects
How common is it?
The prevalence of Landau Kleffner syndrome is unclear. There have been approximately 200 cases reported since the condition was identified in 1957. Due to the nature of the syndrome, there may be more cases that are undiagnosed. Landau Kleffner syndrome is twice as common in males as in females.
Epilepsy and Landau Kleffner Syndrome
- All children with Landau Kleffner syndrome have abnormal electrical activity in one or both temporal lobes.
- About two thirds of children with Landau Kleffner syndrome have seizures.
- Seizures usually occur at night.
- Most children outgrow the seizures and electrical brain activity usually returns to normal by the age of fifteen.
- Initially seizures present as focal seizures, usually of the face.
- They may also have myoclonic jerks which progress to focal seizures or generalised tonic clonics.
- Noticeable facial indicators include eye blinking, rolling eyes, one eye blinking out of sync with the other, blank stares, raising eyebrows, slurping sounds, teeth chattering, shivering, chewing motions, grimacing smiles, mouth stretching motions.
- Convulsive and non convulsive status epilepticus can occur.
Treatment includes early identification of the disorder and medications to abolish the active epileptiform disturbance. Initial therapy includes medications such as anticonvulsants, and steroid therapy.
Other treatment options may include immune globulin and dietary therapy. In selected cases surgery in the temporal lobe (focal resection or multiple sub-pial resection) has been performed.
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Updated August 2013. To be reviewed August 2015.