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Landau-Kleffner Syndrome

  • Overview

    Landau-Kleffner Syndrome (LKS) is a rare neurological disorder. It is characterized by the sudden or gradual development of aphasia (the inability to understand or express language) and an abnormal electroencephalogram (EEG). LKS affects the parts of the brain that control comprehension and speech. The disorder usually occurs in children between the ages of 3 and 6 years old. Typically, children with LKS develop normally for the first few years but then lose their language skills.

    How Boston Children's Hospital approaches LKS

    The Augmentative Communication Program at Children's treats LKS. Our program is known internationally for its work with children and adults who are non-speaking or whose speech is severely impaired. The program provides comprehensive, state-of-the-art evaluation and treatment for children and adults with congenital or acquired disorders affecting expressive communication, comprehension, and computer access.

    Boston Children's Hospital at Waltham
    9 Hope Avenue
    Waltham MA 02453

     781-216-2209
  • In-Depth

    What is Landau-Kleffner Syndrome?

    Doctors might suspect LKS if your child begins to lose language skills, initially not recognizing or understanding words that had been familiar. This is called "word deafness" or "verbal auditory agnosia" and it typically occurs in combination with an abnormal electroencephalogram that shows sleep-activated spike or spike and wave discharges.

    Sometimes, children suspected of having LKS have pervasive developmental disorder (PDD), autism with regression, a congenital disorder such as developmental speech delay, or another epileptic syndrome called electrical status epilepticus of sleep (ESES).

  • Tests

    How is LKS diagnosed?

    If you or your doctor suspects LKS, contact a neurologist. A neurologist can make an initial diagnosis to determine appropriate therapies for your child, including medication, speech therapy and behavioral therapy.

    Why is LKS difficult to diagnose?

    LKS can be difficult to diagnose because it can resemble other conditions. Sometimes doctors suspect Asperger's Syndrome (high functioning) or Pervasive Developmental Disorder (severe impairment). What distinguishes LKS is the sudden, late loss of language and the appearance of autistic characteristics between the ages of 3 to 6.

    Sometimes, a seizure can precipitate these symptoms; other times, simple illness (like chicken pox) or trauma (a fall from the changing table) might spark a change.

    How do I know this isn't autism?

    Children who have autism typically regress somewhere between 18 and 24 months of age--earlier than children with pure LKS, who tend to develop symptoms at 3 to 6 years of age. Typically, a child with LKS should have good eye contact. "You can tell by looking at them that they're trying to understand what you say or they're trying to be engaged or involved in their surroundings," says James Riviello, MD. On the other hand, children with autism who have regression do not seem involved in an examination, or when interacting with other people, exhibit poor eye contact and no language.

    What are the primary symptoms?

    About two-thirds of children with LKS will have seizures and two-thirds will have behavioral problems, but the primary cognitive dysfunction remains loss of language.

    Also, patients with LKS may show spikes on their EEGs, especially when asleep.

  • Your child's care team will most likely include a neurologist, a neuropsychologist, and a speech pathologist or audiologist. Some children with behavioral problems may also need to see a child psychologist and a psychopharmacologist. Your child will also likely undergo treatment with steroids, anti-epileptic, or anti-convulsant medications.

    Your child should also begin speech therapy at the time of diagnosis. An intensive language-based speech program is best when continued throughout the educational years.

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