Encyclopedia of Clinical Neuropsychology

2018 Edition
| Editors: Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan

Landau-Kleffner Syndrome

  • Jeffrey B. TitusEmail author
  • Dave Clarke
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_1559

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

  1. 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.CrossRefGoogle Scholar
  2. 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.PubMedCrossRefGoogle Scholar
  3. Mantovani, J. F., & Landau, W. M. (1980). Acquired aphasia with convulsive disorder: Course and prognosis. Neurology, 30(5), 524–529.PubMedCrossRefGoogle Scholar
  4. 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.Google Scholar
  5. Titus, J. B. (2017). Neuropsychological assessment of children with Landau-Kleffner syndrome. Journal of Pediatric Epilepsy, 6(1), 62–68.Google Scholar
  6. Van Slyke, P. A. (2002). Classroom instruction for children with Landau-Kleffner Syndrome. Child Language Teaching and Therapy, 18, 23–42.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Comprehensive Epilepsy ProgramDell Children’s Medical Center of Central TexasAustinUSA
  2. 2.Department of PsychologyThe University of Texas at AustinAustinUSA
  3. 3.Department of Pediatrics, Dell Medical SchoolThe University of Texas at AustinAustinUSA