Landau-Kleffner syndrome: Difference between revisions
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'''Landau-Kleffner syndrome''' (LKS), also called '''infantile acquired aphasia''', '''acquired epileptic aphasia''' or '''aphasia with convulsive disorder''', is a rare, childhood [[neurologic]]al [[syndrome]] characterized by the sudden or gradual development of [[aphasia]] (the inability to understand or express language) and an abnormal [[Electroencephalography|electroencephalogram]] (EEG). LKS affects the parts of the brain that control comprehension and speech. The disorder usually occurs in children between the ages of 5 and 7 years. Typically, children with LKS develop normally but then lose their [[language]] skills. While many of the affected individuals have clinical seizures, some only have electrographic seizures, including electrographic status epilepticus of sleep (ESES). | '''Landau-Kleffner syndrome''' (LKS), also called '''infantile acquired aphasia''', '''acquired epileptic aphasia''' or '''aphasia with convulsive disorder''', is a rare, childhood [[neurologic]]al [[syndrome]] characterized by the sudden or gradual development of [[aphasia]] (the inability to understand or express language) and an abnormal [[Electroencephalography|electroencephalogram]] (EEG). LKS affects the parts of the brain that control comprehension and speech. The disorder usually occurs in children between the ages of 5 and 7 years. Typically, children with LKS develop normally but then lose their [[language]] skills. While many of the affected individuals have clinical seizures, some only have electrographic seizures, including electrographic status epilepticus of sleep (ESES). | ||
==Signs and Symptoms== | ==Signs and Symptoms== | ||
LKS occurs most frequently in normally developing children who are between 3 and 7 years of age. For no apparent reason, these children begin having trouble understanding what is said to them. Doctors often refer to this problem as auditory agnosiaor "word deafness." The auditory agnosia may occur slowly or very quickly. Parents often think that the child is developing a hearing problem or has become suddenly deaf. Hearing tests, however, show normal hearing. Children may also appear to be autistic or developmentally delayed. | |||
The inability to understand language eventually affects the child's spoken language which may progress to a complete loss of the ability to speak (mutism). Children who have learned to read and write before the onset of auditory agnosia can often continue communicating through written language. Some children develop a type of gestural communication or sign-like language. The communication problems may lead to behavioral or psychological problems. Intelligence usually appears to be unaffected. | |||
The loss of language may be preceded by an epileptic seizure that usually occurs at night. At some time, 80 percent of children with LKS have one or more seizures. The seizures usually stop by the time the child becomes a teenager. All LKS children have abnormal electrical brain activity on both the right and left sides of their brains. | |||
==Differential diagnosis== | ==Differential diagnosis== | ||
Revision as of 18:24, 11 January 2009
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Overview
Landau-Kleffner syndrome (LKS), also called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder, is a rare, childhood neurological syndrome 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 5 and 7 years. Typically, children with LKS develop normally but then lose their language skills. While many of the affected individuals have clinical seizures, some only have electrographic seizures, including electrographic status epilepticus of sleep (ESES).
Signs and Symptoms
LKS occurs most frequently in normally developing children who are between 3 and 7 years of age. For no apparent reason, these children begin having trouble understanding what is said to them. Doctors often refer to this problem as auditory agnosiaor "word deafness." The auditory agnosia may occur slowly or very quickly. Parents often think that the child is developing a hearing problem or has become suddenly deaf. Hearing tests, however, show normal hearing. Children may also appear to be autistic or developmentally delayed.
The inability to understand language eventually affects the child's spoken language which may progress to a complete loss of the ability to speak (mutism). Children who have learned to read and write before the onset of auditory agnosia can often continue communicating through written language. Some children develop a type of gestural communication or sign-like language. The communication problems may lead to behavioral or psychological problems. Intelligence usually appears to be unaffected.
The loss of language may be preceded by an epileptic seizure that usually occurs at night. At some time, 80 percent of children with LKS have one or more seizures. The seizures usually stop by the time the child becomes a teenager. All LKS children have abnormal electrical brain activity on both the right and left sides of their brains.
Differential diagnosis
The syndrome can be difficult to diagnose and may be misdiagnosed as autism, pervasive developmental disorder, hearing impairment, learning disability, auditory/verbal processing disorder, attention deficit disorder, mental retardation, childhood schizophrenia, or emotional/behavioral problems.
Treatment
Treatment for LKS usually consists of medications, such as anticonvulsants and corticosteroids, and speech therapy, which should be started early. A controversial treatment option involves a surgical technique called multiple subpial transection in which multiple incisions are made through the cortex of the affected part of the brain, severing the axonal tracts in the subjacent white matter.
Prognosis
The prognosis for children with LKS varies. Some affected children may have a permanent severe language disorder, while others may regain much of their language abilities (although it may take months or years). In some cases, remission and relapse may occur. The prognosis is improved when the onset of the disorder is after age 6 and when speech therapy is started early. Seizures generally disappear by adulthood.
References
- "Landau-Kleffner syndrome information page". National Institute of Neurological Disorders and Stroke. 2007-02-13. Retrieved 2007-08-23.
- "Landau-Kleffner syndrome". National Institute on Deafness and other Communication Disorders. 2002. Retrieved 2007-08-23.
- Pearl PL, Carrazana EJ, Holmes GL (2001). "The Landau-Kleffner syndrome". Epilepsy Curr. 1 (2): 39–45. PMID 15309183.
- Rotenberg J, Pearl PL (2003). "Landau-Kleffner syndrome". Arch Neurol. 60 (7): 1019–21. PMID 12873863.
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