Landau-Kleffner Syndrome (LKS)
Rare Disorder Affects Speech and Language Comprehension in Children
Landau-Kleffner syndrome (LKS), also called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder, is a rare, childhood neurological syndrome that occurs exclusively in childhood.
LKS was first described in 1957 by Dr. W.M. Landau and Dr. F.R. Kleffner, however, the cause of LKS is still unknown. More than 200 cases of Landau-Kleffner syndrome have been reported in worldwide medical literature.
While in some cases LKS is believed to result from a lack of full development of the networks within the brain, other cases seem to be precipitated by a viral infection (Centers for Disease Control and Prevention Fact Sheet, 2004).
Males are More Affected with LKS Than Females
The disorder typically affects children, between the ages of 3 and 7 years, who previously had no developmental, language, or interactional difficulties (Centers for Disease Control and Prevention, 2004).
Children usually experience an abrupt loss of language and comprehension and a diminution in their ability to express themselves. Males are more affected with LKS than females by a ratio of 2:1. Approximately 70-80% of children with LKS have seizures, however, the presence of seizures is not a requirement for a diagnosis (Centers for Disease Control and Prevention).
Since children with Landau-Kleffner syndrome have difficulty communicating and behaving, they may be misdiagnosed as having autism, pervasive developmental disorder, or hearing impairment.
Most children outgrow the seizures, and electrical brain activity on the EEG usually returns to normal by age 15. Some children 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 (Centers for Disease Control and Prevention).
The seizures, abnormal EEG, behavioral problems, and other problems usually decrease or resolve completely as the child reaches adolescence. Generally, the later the child develops LKS, the greater the chance of him or her having a complete recovery.
Symptoms of Landau-Kleffner Syndrome
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, it is believed that as many as 80 percent of children with Landau-Kleffner syndrome have behavioural problems, such as hyperactivity or autistic-like behaviors (Centers for Disease Control and Prevention).
Since speech is greatly affected by LKS, speech therapy should be provided to the child as early as possible. Sign language should be used with the child who has little or no language comprehension. In addition to traditional speech therapy, some children have shown improvements in their language abilities when treated with steroids.
Many children with LKS are in special education classrooms that are equipped to provide an appropriate education to those with severe speech and language disorders. In most cases described in detail, a clearly normal period of motor and language development occurs before AEA symptoms appear.
There is no cure for LKS, therefore, the treatment program involves managing the child’s symptoms. Corticosteroid therapy, if given early, can in many cases restore speech and eliminate seizures. For patients who have persistent seizures associated with lasting language difficulties, neurosurgery can be an effective method of treatment.
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