Document Detail


Epileptic spasms in clusters without hypsarrhythmia in infancy.
MedLine Citation:
PMID:  12875954     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Spasms are defined as epileptic seizures characterized by brief axial contraction, in flexion, extension or mixed, symmetric or asymmetric, lasting from a fraction of a second to 1-2s, and are associated with a slow-wave transient or sharp and slow-wave complex, followed or not by voltage attenuation. Epileptic spasms usually appear in clusters and are age-dependent. This type of epileptic spasms associated with the particular EEG pattern, hypsar rhythmia, constitutes the basis for the diagnosis of West syndrome. The question is, how to nosologically define those patients who clearly present epileptic spasms in clusters without modified or typical hypsarrhythmia and with or without focal paroxysmal discharges on the interictal EEG. In the present series, the four patients show that epileptic spasms in clusters may occur in infancy, without hypsarrhythmia. They all presented the following features: normal neuropsychological development before onset of epileptic spasms, clusters of epileptic spasms, focal clinical and/or EEG abnormalities, normal neuroradiological imaging, neurometabolic investigations and karyotypes. In three of the patients, seizures were refractory to AEDs. Epileptic spasms in clusters without hypsarrhythmia that start in the first year of life represent a subtype of infantile spasms that generally are refractory to AEDs. It is not yet clear whether it should be considered as a variant of West syndrome or not [Published with Video sequence].
Authors:
Roberto Horacio Caraballo; Natalio Fejerman; Bernardo Dalla Bernardina; Victor Ruggieri; Ricardo Cersósimo; Carlos Medina; Juan Pociecha
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Epileptic disorders : international epilepsy journal with videotape     Volume:  5     ISSN:  1294-9361     ISO Abbreviation:  Epileptic Disord     Publication Date:  2003 Jun 
Date Detail:
Created Date:  2003-07-23     Completed Date:  2003-12-09     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  100891853     Medline TA:  Epileptic Disord     Country:  France    
Other Details:
Languages:  eng     Pagination:  109-13     Citation Subset:  IM    
Affiliation:
Servicio de Neurología, Hospital de Ninos "Prof. Dr. Juan P. Garrahan", Buenos Aires, 2, Argentina. rcaraballo@janssen.com.ar
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MeSH Terms
Descriptor/Qualifier:
Anticonvulsants / therapeutic use
Brain Damage, Chronic / diagnosis,  physiopathology
Child
Child, Preschool
Dominance, Cerebral / physiology
Drug Therapy, Combination
Electroencephalography*
Female
Follow-Up Studies
Frontal Lobe / physiopathology
Humans
Infant
Infant, Newborn
Mental Retardation / diagnosis,  physiopathology
Recurrence
Spasms, Infantile / classification,  diagnosis*,  drug therapy,  physiopathology
Temporal Lobe / physiopathology
Chemical
Reg. No./Substance:
0/Anticonvulsants

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