Landau-Kleffner Syndrome is just one type of epilepsy found among children who are also diagnosed with an Autism Spectrum Disorder, though the disorder is considered very rare.
Landau-Kleffner Syndrome was originally discovered by William Landau and Frank Kleffner. In 1957, they recognized 6 children with a convulsive disorder and an acquired aphasia. Aphasia is a disorder that affects language comprehension and articulation. Acquired aphasia is where the loss of understanding and articulation is sudden or occurs over time. Being extremely rare, it is difficult to estimate the number of children currently effected by this disorder. Between 1957 and 1990 there was thought to be little over 160 cases.
Similar to autism, Landau-Kleffner Syndrome (LKS) is often confused with an ASD, while it can also be diagnosed in conjunction with autism. It is reported that LKS, which is more prevalent in boys, can have an onset between 2 and 7 years old, though there have instances where it has been diagnosed at a slightly younger age. As for the onset, the status elipticus that is the basis for LKS organizes and ‘disappears’ around 15 years of age for unknown reasons. However, the damage done with the pervasiveness of the disorder require that all avenues are exhausted to stop the activity, as the damage done can be permanent and even go as far as causing an auditory verbal agnosia (inability to recognize objects, people, shapes or sounds where no memory loss nor sense loss is present).
This disorder can be extremely difficult to treat. Many parents find that the constant chase of LKS is exhausting and frustrating. Medications that work to stop the status elipticus one month, may not another; or when the proper dosage is found, but the medication is pushed to its fullest potential, returning to that previously ‘magic’ dosage is futile - the medication no longer works. More often that not, children begin with various anti-epileptic drugs (AEDs), lightest first, climbing their way in potency and side-effects to quiet the brain. Steroids often find relief in many cases for the longest times, however, the side-effects become more severe. In still others, an Intravenous Immuniglobin or IVIG is found helpful when it appears LKS is the result of an autoimmune disfunction. The last course of treatment may be a surgical removal of the part of the brain causing the status elipticus.
Landau-Kleffner Syndrome appears as:
- sudden or subtle loss of language, onset can be as early as 1 ½ years of age
- gesturing as communication begins or increases
- language difficulties lead to behavioral problems
- behavioral manifestations can include hyperactivity, poor attention, depression and irritability
- intelligence is in tact, though it may be difficult to determine
- an epileptic seizure is likely to occur just before or shortly after the onset of LKS
- epileptic seizures are more likely to occur at night, but may also happen during the day
- it is not necessary to have a seizure to have LKS
- the epileptic activity of LKS occurs at night and can not be observed by others
- left uncontrolled or undiagnosed, can lead to inability to recognize environmental sounds such as the telephone ringing or a dog barking
- may appear to be deaf though an examine would show the hearing is functioning
- extreme cases develop temporary neurological problems (loss of bladder / bowel control, visual disturbances were the child can see but does not understand what he sees. This includes recognizing family, friends or objects such as food and clothes)
- exhibit autistic-like behavior, including: avoidance of contact with family and friends, avoidance of eye contact, extreme pickiness over food, very disturbed sleep, attacks of rage and aggression, insensitivity to pain, bizarre, inappropriate and repetitive play
- many parents report ‘getting a different child’ every day or even every hour with no predictability
The only way to determine if a child has Landau-Kleffner Syndrome is through an electroencephalogram (EEG) which must be conducted primarily during the night when LKS is prevalent. Due to the rarity and complex effects on language, sleep and behavior, it may take time for medical professionals to recognize and diagnoses LKS. Landau-Kleffner Syndrome may also be overlooked where a diagnosis of an Autism Spectrum Disorder is present.
It is imperative that if a child exhibits any of the above, an investigation into Landau-Kleffner Syndrome, or any other abnormal eliptiform activity begin and if diagnosed, immediate efforts to manage the activity.