Document Detail

Epileptic encephalopathy of late childhood: Landau-Kleffner syndrome and the syndrome of continuous spikes and waves during slow-wave sleep.
MedLine Citation:
PMID:  14734935     Owner:  NLM     Status:  MEDLINE    
Landau-Kleffner syndrome (LKS) and the syndrome of continuous spikes and waves during slow wave sleep (CSWS) are two points on the spectrum of functional childhood epileptic encephalopathies. They are characterized by a severe paroxysmal EEG disturbance that may permanently alter the critical synaptogenesis by strengthening synaptic contacts that should have been naturally "pruned." The much more common benign epilepsy with centrotemporal spikes is also related to LKS and CSWS by a common pathophysiology. Although prognosis in LKS and CSWS for seizure control is good, cognitive function declines and permanent neuropsychologic dysfunction is seen in many cases. This permanent damage is most evident in those patients who had early-onset EEG abnormality and a prolonged active phase of continuous spike-and-wave discharges during sleep. If the active phase of paroxysmal activity persists for over 2 to 3 years, even successful treatment does not resolve neuropsychologic sequelae. In LKS, the paroxysmal activity permanently affects the posterior temporal area and results in auditory agnosia and language deficits; in CSWS, the frontal lobes are more involved and other cognitive disturbances predominate. Aggressive treatment should include high-dose antiepileptic drugs, corticosteroids, and surgery in specific cases.
Michael C Smith; Thomas J Hoeppner
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Journal of clinical neurophysiology : official publication of the American Electroencephalographic Society     Volume:  20     ISSN:  0736-0258     ISO Abbreviation:  J Clin Neurophysiol     Publication Date:    2003 Nov-Dec
Date Detail:
Created Date:  2004-01-21     Completed Date:  2004-03-15     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  8506708     Medline TA:  J Clin Neurophysiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  462-72     Citation Subset:  IM    
Departmernt of Neurosciences, Rush University Medical Center, Chicago, IL 60612-3833, USA.
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MeSH Terms
Brain Mapping
Child, Preschool
Diagnosis, Differential
Landau-Kleffner Syndrome / epidemiology,  physiopathology*
Magnetic Resonance Imaging
Sleep / physiology*
Treatment Outcome

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

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