Document Detail


Early development in Dravet syndrome; visual function impairment precedes cognitive decline.
MedLine Citation:
PMID:  21109403     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
Aim of the study was to describe prospectively the early neuropsychological evolution including the first pre-cognitive stages of the Severe Myoclonic Epilepsy in Infancy (SMEI) or Dravet syndrome. Five cases, four of whom since before a diagnostic evidence of the Dravet syndrome, were followed up. Full clinical assessment including developmental, visual function and behaviour assessments were serially performed. In four cases, a variable onset age of cognitive decline assessed with developmental scales was preceded some months before by an impairment of visual function; the remaining patient during all the course of follow-up till 51 months of age showed a normal development without visual impairment. A cognitive decline with variable onset was generally confirmed in Dravet syndrome. The previous early impairment of visual function seems to herald the cognitive decline and provides useful prognostic information; furthermore, it possibly suggests some clues for a better understanding of the mechanisms of cognitive deterioration in this syndrome.
Authors:
Daniela Chieffo; Daniela Ricci; Giovanni Baranello; Diego Martinelli; Chiara Veredice; Donatella Lettori; Domenica Battaglia; Charlotte Dravet; Eugenio Mercuri; Francesco Guzzetta
Related Documents :
18500803 - Entrainer effect on photochirogenesis in near- and supercritical carbon dioxide: dramat...
21597923 - Type iii klippel-feil syndrome: case report and review of associated craniofacial anoma...
20716963 - Disrupted microrna expression caused by mecp2 loss in a mouse model of rett syndrome.
Publication Detail:
Type:  Journal Article     Date:  2010-11-24
Journal Detail:
Title:  Epilepsy research     Volume:  93     ISSN:  1872-6844     ISO Abbreviation:  Epilepsy Res.     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2010-12-27     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8703089     Medline TA:  Epilepsy Res     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  73-9     Citation Subset:  IM    
Copyright Information:
2010 Elsevier B.V. All rights reserved.
Affiliation:
Catholic University, Child Neurology and Psychiatry, Rome, Italy.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:

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


Previous Document:  Midface distraction osteogenesis: Internal vs. external devices.
Next Document:  Attentional processes and ADHD-related symptoms in pediatric patients with epilepsy.