Landau-Kleffner Syndrome
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.
Symptoms
• difficulty understanding what is said (auditory agnosia, or “word deafness”)
• difficulty replying (aphasia)
• seizures (70-85 percent of individuals).
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 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.
Sources:
Sotero de Menezes, Marcio. “Landau-Kleffner Syndrome.” eMedicine 20 Mar 2007 26 Apr 2007 .
“Landau-Kleffner Syndrome.” Health Information: Voice, Speech, and Language. 21 Feb 2006. National Institute on Deafness and Other Communication Disorders. 27 Apr 2007 .
“NINDS Landau-Kleffner Syndrome Information Page.” Disorder Index. 13 Feb 2007. National Institute of Neurological Disorders and Stroke. 27 Apr 2007 .
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