What is Landau-Kleffner Syndrome?

Landau-Kleffner syndrome is a very rare childhood neurological disorder which was first identified and described in 1957. Children with this condition experience abrupt aphasia, a loss of language skills, and they also develop seizures. Patients with this condition commonly experience a misdiagnosis with another condition, such as autism, because Landau-Kleffner is so rare. Proper diagnosis and rapid intervention can greatly improve the prognosis for the patient, especially if the patient is seen by a physician who has experience with this condition.

In patients with Landau-Kleffner syndrome, early development is normal, with the child reaching expected development targets and developing early language skills. Between the ages of five and seven, the child develops aphasia, and clinical seizures may appear. Even if clinical seizures do not appear, the child will have an abnormal electroencephalogram (EEG) which indicates seizure activity in the brain.

Parents of children with Landau-Kleffner syndrome may originally think that their children are experiencing hearing problems, or that the child is developing a psychological issue. Pediatricians can have difficulty diagnosing this condition unless they have experience with it, and they may be reluctant to provide a referral to a neurologist if the patient does not experience clinical seizures and no EEG is performed to reveal abnormalities in the brain. Patients can become aggressive, unfocused, and confused. Acquired epileptic aphasia, as it is also known, can be confused with autism, attention deficit disorder, hearing impairment, auditory processing disorder, and a variety of developmental disorders.

With a diagnosis of Landau-Kleffner syndrome, a patient can be given medications which are designed to control the seizures and start attending speech therapy to address the aphasia. Landau-Kleffner syndrome treatment is most effective when it is provided early, and when the onset of the condition occurs after the age of six. During treatment, the patient can experience radical fluctuations, which is something for parents to be aware of: progress can appear to reverse, or a child can experience a radical improvement followed by a decline.

The prognosis for patients with LKS is quite variable. Some patients experience continued development problems and abnormal brain activity which can require more aggressive or controversial treatment approaches, including brain surgery. Others may overcome their aphasia with periodic relapses. Patients and parents should be aware that it is difficult for a doctor to make a prediction about the prognosis for an individual patient, because many factors are involved and the brain is an extremely mysterious organ which sometimes defies expectations.