Synonyms
Acquired epileptic aphasia
Definition
Landau-Kleffner Syndrome (LKS) is an epileptic encephalopathy that is marked by sudden and relatively rapid onset of aphasia in a child with normal or near normal language development. The acquired aphasia classically begins with a verbal auditory agnosia (VAA), or word deafness, and often progresses to expressive language impairment. Sleep-activated epileptiform abnormalities maximally over the temporal regions are a defining feature. Clinical seizures are evident in most cases, but they are not necessary for the diagnosis.
Epidemiology
LKS is considered to be an especially rare epilepsy syndrome, constituting about 0.2% of childhood epilepsy syndromes. Its prevalence, however, has increased in recent years likely due to better awareness. LKS is believed to be almost twice as common in males.
A genetic predisposition for LKS has not been identified. Rather, some have suggested that an underlying autoimmune condition may be responsible for...
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Further Reading
Cockerell, I., Bølling, G., & Nakken, K. O. (2011). Landau-Kleffner syndrome in Norway: Long-term prognosis and experiences with the health services and educational systems. Epilepsy & Behavior, 21(2), 153–159.
Korkman, M., Granström, M., Appelqvist, K., & Liukkonen, E. (1998). Neuropsychological characteristics of five children with the Landau-Kleffner Syndrome: Dissociation of auditory and phonological discrimination. Journal of the International Neuropsychological Society, 4, 566–575.
Mantovani, J. F., & Landau, W. M. (1980). Acquired aphasia with convulsive disorder: Course and prognosis. Neurology, 30(5), 524–529.
Riviello, J. J. (2017). The Landau-Kleffner syndrome and epilepsy with continuous spike-waves during sleep. In J. M. Pellock, D. R. Nordli Jr., R. Sankar, & J. W. Wheless (Eds.), Pellock’s pediatric epilepsy: Diagnosis and therapy (4th ed., pp. 393–406). New York: Demos Medical.
Titus, J. B. (2017). Neuropsychological assessment of children with Landau-Kleffner syndrome. Journal of Pediatric Epilepsy, 6(1), 62–68.
Van Slyke, P. A. (2002). Classroom instruction for children with Landau-Kleffner Syndrome. Child Language Teaching and Therapy, 18, 23–42.
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Titus, J.B., Clarke, D. (2018). Landau-Kleffner Syndrome. In: Kreutzer, J.S., DeLuca, J., Caplan, B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, Cham. https://doi.org/10.1007/978-3-319-57111-9_1559
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DOI: https://doi.org/10.1007/978-3-319-57111-9_1559
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