Document Detail

Cryptogenic late-onset epileptic spasms: an overlooked syndrome of early childhood?
MedLine Citation:
PMID:  16822250     Owner:  NLM     Status:  MEDLINE    
PURPOSE: Few reports detailing late-onset epileptic spasms have been published. To determine whether this condition merely represents a late variant of classic West syndrome or exhibits specific features distinct from the latter and related to a later stage of brain maturation, we analyzed the whole population with this specific seizure type, excluding symptomatic cases to avoid the effect of brain lesion. METHODS: We reviewed the files of the 56 children evaluated for epileptic spasms in clusters having begun at age 12 months or later and analyzed clinical and video-EEG data of the 22 patients (4-17 years; mean, 8.5 years) without obvious cause. RESULTS: Interictal EEG did not show classic hypsarrhythmia. A temporal or temporofrontal slow wave and/or spike focus could be identified in all cases. Twelve children showed spasms with a tonic component. Ictal EEG revealed generalized high-voltage slow wave followed by diffuse voltage attenuation with superimposed fast activity. All children also exhibited other types of recorded seizures consisting of bursts of spike-waves with temporofrontal predominance, reminiscent of "atypical absences." In contrast with the occurrence of tonic components within a cluster of spasms, no tonic seizure stricto sensu was recorded or reported by the caregivers. In 10 children, treatment (two vigabatrin, seven hydrocortisone, one adrenocorticotropic hormone) achieved complete cessation of seizures and disappearance of focal EEG anomalies, but spasms persisted in 12 children. CONCLUSIONS: The cryptogenic group in our series without recognized cause and temporal or temporofrontal EEG anomalies seems to represent a type of epileptic encephalopathy intermediary between West and Lennox-Gastaut syndromes, in terms of seizure types and interictal EEG, and could correspond to dysfunction of the maturation process of the temporal lobe, possibly due to an undisclosed lesion.
Monika M Eisermann; Dorothée Ville; Christine Soufflet; Perrine Plouin; Catherine Chiron; Olivier Dulac; Anna Kaminska
Publication Detail:
Type:  Comparative Study; Journal Article    
Journal Detail:
Title:  Epilepsia     Volume:  47     ISSN:  0013-9580     ISO Abbreviation:  Epilepsia     Publication Date:  2006 Jun 
Date Detail:
Created Date:  2006-07-06     Completed Date:  2006-07-28     Revised Date:  2007-03-27    
Medline Journal Info:
Nlm Unique ID:  2983306R     Medline TA:  Epilepsia     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1035-42     Citation Subset:  IM    
Department of Clinical Neurophysiology, Saint Vincent de Paul Hospital, Paris, France.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Age Distribution
Age Factors
Age of Onset
Cerebral Cortex / physiopathology*
Child, Preschool
Electroencephalography / statistics & numerical data*
Epilepsy, Absence / diagnosis,  physiopathology
Follow-Up Studies
Frontal Lobe / physiopathology
Infant, Newborn
Middle Aged
Retrospective Studies
Spasms, Infantile / diagnosis*,  physiopathology
Temporal Lobe / physiopathology
Videotape Recording
Comment In:
Epilepsia. 2007 Jan;48(1):206-8   [PMID:  17241232 ]

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

Previous Document:  Linkage analysis and disease models in benign familial infantile seizures: a study of 16 families.
Next Document:  Benign partial epilepsy in infancy: myth or reality?