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Landau-Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children.
Ment Retard Dev Disabil Res Rev. 2004; 10(2):144-9.MR

Abstract

The Landau-Kleffner syndrome (LKS) and electrical status epilepticus in slow wave sleep (ESES) are rare childhood-onset epileptic encephalopathies in which loss of language skills occurs in the context of an epileptiform EEG activated in sleep. Although in LKS the loss of function is limited to language, in ESES there is a wider spectrum of cognitive impairment. The two syndromes are distinct but have some overlap. The relationship between the epileptiform EEG abnormalities and the loss of cognitive function remains controversial, even in LKS which is the most widely accepted as an acquired epileptic aphasia. Language regression also occurs in younger children, frequently in the context of a more global autistic regression. Many of these children have epileptiform EEGs. The term autistic regression with epileptiform EEG has been proposed for these children. Whether these children are part of an extended LKS spectrum is very controversial, because there are differences in age of onset, clinical phenotype, and EEG findings. An understanding of the available data on clinical characteristics, EEG findings, pathology, prognosis, and treatment of these syndromes is essential for further progress in this area.

Authors+Show Affiliations

Departments of Neurology, Pediatrics, the Comprehensive Epilepsy Management Center, Montefiore Medical Center, The Albert Einstein College of Medicine, Bronx, New York 10467, USA.No affiliation info available

Pub Type(s)

Journal Article
Research Support, U.S. Gov't, P.H.S.
Review

Language

eng

PubMed ID

15362173

Citation

McVicar, Kathryn A., and Shlomo Shinnar. "Landau-Kleffner Syndrome, Electrical Status Epilepticus in Slow Wave Sleep, and Language Regression in Children." Mental Retardation and Developmental Disabilities Research Reviews, vol. 10, no. 2, 2004, pp. 144-9.
McVicar KA, Shinnar S. Landau-Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children. Ment Retard Dev Disabil Res Rev. 2004;10(2):144-9.
McVicar, K. A., & Shinnar, S. (2004). Landau-Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children. Mental Retardation and Developmental Disabilities Research Reviews, 10(2), 144-9.
McVicar KA, Shinnar S. Landau-Kleffner Syndrome, Electrical Status Epilepticus in Slow Wave Sleep, and Language Regression in Children. Ment Retard Dev Disabil Res Rev. 2004;10(2):144-9. PubMed PMID: 15362173.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Landau-Kleffner syndrome, electrical status epilepticus in slow wave sleep, and language regression in children. AU - McVicar,Kathryn A, AU - Shinnar,Shlomo, PY - 2004/9/14/pubmed PY - 2005/2/9/medline PY - 2004/9/14/entrez SP - 144 EP - 9 JF - Mental retardation and developmental disabilities research reviews JO - Ment Retard Dev Disabil Res Rev VL - 10 IS - 2 N2 - The Landau-Kleffner syndrome (LKS) and electrical status epilepticus in slow wave sleep (ESES) are rare childhood-onset epileptic encephalopathies in which loss of language skills occurs in the context of an epileptiform EEG activated in sleep. Although in LKS the loss of function is limited to language, in ESES there is a wider spectrum of cognitive impairment. The two syndromes are distinct but have some overlap. The relationship between the epileptiform EEG abnormalities and the loss of cognitive function remains controversial, even in LKS which is the most widely accepted as an acquired epileptic aphasia. Language regression also occurs in younger children, frequently in the context of a more global autistic regression. Many of these children have epileptiform EEGs. The term autistic regression with epileptiform EEG has been proposed for these children. Whether these children are part of an extended LKS spectrum is very controversial, because there are differences in age of onset, clinical phenotype, and EEG findings. An understanding of the available data on clinical characteristics, EEG findings, pathology, prognosis, and treatment of these syndromes is essential for further progress in this area. SN - 1080-4013 UR - https://www.unboundmedicine.com/medline/citation/15362173/Landau_Kleffner_syndrome_electrical_status_epilepticus_in_slow_wave_sleep_and_language_regression_in_children_ L2 - https://doi.org/10.1002/mrdd.20028 DB - PRIME DP - Unbound Medicine ER -