FACTS FOR FAMILIES: LANDAU-KLEFFNER SYNDROME

The ability to produce and understand language is the most complex task the human brain performs and it is remarkable that in most children this ability evolves without difficulty. In some children, like those with Landau-Kleffner syndrome, something affects the way the brain functions or develops, and this causes language development to be disturbed (This problem is commonly referred to as an "aphasia", pronounced a-faze-ee-a, a term taken from the Greek which literally means absence or loss of speech). While severe language problems occur in many childhood conditions, Landau-Kleffner syndrome is associated with a specific cluster of symptoms that distinguish it from other entities.

One of the special features to the language disturbance in Landau-Kleffner syndrome, is that it occurs for no obvious reason usually afterthe child has developed language normally for a period of time. The language disturbance may appear rather suddenly in the course of a few days, or may progress slowly over a number of months. Children may lose the ability to understand, or use words or phrases they had previously known, or may respond inconsistently. The severity can range from significant problems in articulating words clearly to almost complete inability to understand or produce speech. Sometimes the language disturbance can set in early in development at a time when it is difficult to tell whether there has been a true loss of language milestones, or a fading of progress in language acquisition. When the child loses language, there may be some reason to question his\her hearing but examination of their hearing usually proves normal. It can often be demonstrated that nonverbal intelligence is reasonably normal. Rather, the syndrome is associated with problems in areas of the brain responsible for language processing and this impedes their ability to understand and produce speech.

The second major feature of Landau-Kleffner syndrome is that the aphasia is often associated with signs of a seizure disorder. The child may have convulsions, or have periods of "blanking out". However, about 30 percent of LKS children may not exhibit signs of a seizure related disturbance and identification of the brain electrical problem may require specialized medical testing such as an electroencephalogram (EEG) to be observed. A sleep EEG is important to obtain in all cases of children suspected of having Landau-Kleffner Syndrome since an EEG abnormality is one of the more important features of the diagnosis. However, a normal EEG need not necessarily rule out the diagnosis, since these abnormalities can be variable. Often other tests are required to make the diagnosis. Other studies that can be considered are neuropsychological testing, auditory evoked potentials, MRI (Magnetic Resonance Imaging) scan, and a SPECT (Single Photon Emission Computed Tomography) or PET (Position Emission Tomography) scan. These tests look at brain function in different ways, and may be needed to reveal the abnormalities associated with LKS.

Behavioral disturbances are apparent in about 2/3 of LKS cases, and range from aggressivity and attention deficit hyperactivity disorder symptomology, to behaviors commonly associated with autism such as avoidant and withdrawn behavior, resistance to change in daily activity, gestural stereotypies, echolalia and echopraxia, hyperlexia and even psychotic-like disturbances. The presence of these behavioral problems can lead to confusion in arriving at a final diagnosis, especially in young children. They can, for example, prompt a diagnosis of Autism or Pervasive Developmental Disorder, even though children may not fit typical descriptions of these conditions.

The long term outcome of children with Landau-Kleffner syndrome varies a great deal. Some children recover spontaneously, while others will improve slowly over a long period of time. Some have lifelong and severe language problems. Treatment involves special education and speech and language therapy as well as medicine. It is not uncommon for children to be prescribed anticonvulsant medication and sometimes corticosteroids are used.

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