Document Detail


Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome, LKS) and regressive autistic disorders with epileptic EEG abnormalities: the continuing debate.
MedLine Citation:
PMID:  20637551     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome) may present as a developmental language disturbance and the affected child may also exhibit autistic features. Landau-Kleffner is now seen as the rare and severe end of a spectrum of cognitive-behavioural symptoms that can be seen in idiopathic (genetic) focal epilepsies of childhood, the benign end being the more frequent typical rolandic epilepsy. Several recent studies show that many children with rolandic epilepsy have minor developmental cognitive and behavioural problems and that some undergo a deterioration (usually temporary) in these domains, the so-called "atypical" forms of the syndrome. The severity and type of deterioration correlate with the site and spread of the epileptic spikes recorded on the electroencephalogram within the perisylvian region, and continuous spike-waves during sleep (CSWS) frequently occur during this period of the epileptic disorder. Some of these children have more severe preexisting communicative and language developmental disorders. If early stagnation or regression occurs in these domains, it presumably reflects epileptic activity in networks outside the perisylvian area, i.e. those involved in social cognition and emotions. Longitudinal studies will be necessary to find out if and how much the bioelectrical abnormalities play a causal role in these subgroup of children with both various degrees of language and autistic regression and features of idiopathic focal epilepsy. One has to remember that it took nearly 40 years to fully acknowledge the epileptic origin of aphasia in Landau-Kleffner syndrome and the milder acquired cognitive problems in rolandic epilepsies.
Authors:
Thierry Deonna; Eliane Roulet-Perez
Publication Detail:
Type:  Journal Article; Review     Date:  2010-07-15
Journal Detail:
Title:  Brain & development     Volume:  32     ISSN:  1872-7131     ISO Abbreviation:  Brain Dev.     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-09-06     Completed Date:  2010-12-20     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7909235     Medline TA:  Brain Dev     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  746-52     Citation Subset:  IM    
Copyright Information:
Copyright © 2010 Elsevier B.V. All rights reserved.
Affiliation:
Unité de Neurologie et de Neuroréhabilitation Pédiatrique, Département Médico-Chirurgical de Pédiatrie, Switzerland. Thierry.Deonna@chuv.ch
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MeSH Terms
Descriptor/Qualifier:
Autistic Disorder / physiopathology*
Electroencephalography*
Female
Humans
Landau-Kleffner Syndrome / physiopathology*
Language Development Disorders / physiopathology
Male
Regression (Psychology)*
Review Literature as Topic

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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