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Landau-Kleffner Syndrome

Disorder Affects the Central Nervous System

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Updated April 27, 2007

Landau-Kleffner syndrome, also called acquired epileptic aphasia, is a disorder of the central nervous system that begins in childhood. It causes children to have seizures, and disrupts their ability to understand what is said to them, and how to reply back. The cause of the disease is unknown.

Landau-Kleffner syndrome is named after the two physicians who first identified the syndrome. The syndrome affects both males and females, but it is not known exactly how often it occurs. More than 200 cases of Landau-Kleffner syndrome have been reported in the worldwide medical literature.

Symptoms

Symptoms of Landau-Kleffner syndrome usually begin between the ages of 3 and 7 years old (although they may begin as early as 18 months and as late as 13 years old). They include:
  • difficulty understanding what is said (auditory agnosia, or “word deafness”)
  • difficulty replying (aphasia)
  • seizures (70-85 percent of individuals).
In addition, as many as 80 percent of children with Landau-Kleffner syndrome have behavioral problems, such as hyperactivity or autistic-like behaviors.

Diagnosis

Because children with Landau-Kleffner syndrome have difficulty communicating and behaving, they may be misdiagnosed as having autism, pervasive developmental disorder, or hearing impairment. However, in Landau-Kleffner syndrome there are changes in brain-wave patterns when a brain wave test (encephalogram, or EEG) is performed. These test results can help confirm the diagnosis.

Magnetic resonance imaging (MRI) is done to check for any brain abnormalities such as tumors, infections, or diseases which may be causing the speech and language symptoms. Special hearing tests such as brainstem auditory evoked response (BAER) or behavior hearing test (BHT) are done to test the child’s ability to hear.

Treatment

Medication is given to control seizures if they are occurring. There is no specific drug for the language problems in Landau-Kleffner syndrome. Children who learned to read and write before the symptoms began may still be able to communicate by writing. Children may be able to learn sign language or lip reading to help with communication. Some children are helped by corticosteroids such as prednisone or by corticotrophin (ACTH). Speech therapy is important for children with Landau-Kleffner syndrome.

Some children affected by Landau-Kleffner syndrome may have a permanent language disorder, while others may regain their ability to communicate over months or years. In general, the earlier the symptoms begin, the poorer the language recovery. Also, affected children tend to outgrow the seizures. More research is needed to determine the long-term effects of Landau-Kleffner syndrome.

Sources:

Sotero de Menezes, Marcio. "Landau-Kleffner Syndrome." eMedicine 20 Mar 2007 26 Apr 2007 <http://www.emedicine.com/neuro/topic182.htm>.

"Landau-Kleffner Syndrome." Health Information: Voice, Speech, and Language. 21 Feb 2006. National Institute on Deafness and Other Communication Disorders. 27 Apr 2007 <http://www.nidcd.nih.gov/health/voice/landklfs.asp>.

"NINDS Landau-Kleffner Syndrome Information Page." Disorder Index. 13 Feb 2007. National Institute of Neurological Disorders and Stroke. 27 Apr 2007 <http://www.ninds.nih.gov/Disorders/landaukleffnersyndrome/landaukleffnersyndrome.htm>.

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