Document Detail


Predictors of seizure onset in Rett syndrome.
MedLine Citation:
PMID:  17011329     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVES: To investigate risk factors for seizure onset in Rett syndrome. STUDY DESIGN: Information on presence and age at onset of seizures, perinatal and developmental history, and genetic status was abstracted on 275 cases in the Australian Rett Syndrome Database. Cox and Weibull regression were used to investigate and provide a model for predicting the effects of genetic and developmental factors on age at seizure onset. RESULTS: Seizures were reported in 81% of 275 cases; the median age of onset was 48 months. Not having gained the ability to walk (P = .003) and developmental problems in the first 10 months of age (P = .04) were associated with an almost 2-fold increased risk of seizures. Cases without a detectable MECP2 mutation had a higher risk of seizure onset up to 4 years of age (P < .001) but a lower risk after 4 years (P = .08). CONCLUSIONS: Seizure onset in Rett syndrome is associated with early developmental factors and with genotype. Information on these factors can be used to predict age at seizure onset after diagnosis.
Authors:
Le Jian; Lakshmi Nagarajan; Nicholas de Klerk; David Ravine; Carol Bower; Alison Anderson; Sarah Williamson; John Christodoulou; Helen Leonard
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Journal of pediatrics     Volume:  149     ISSN:  0022-3476     ISO Abbreviation:  J. Pediatr.     Publication Date:  2006 Oct 
Date Detail:
Created Date:  2006-10-02     Completed Date:  2006-11-28     Revised Date:  2007-12-03    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  542-7     Citation Subset:  AIM; IM    
Affiliation:
Centre for Child Health Research, The University of Western Australia, Telethon Institute for Child Health Research, Perth, Western Australia, Australia.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Australia
Child
Child, Preschool
Humans
Infant
Mutation
Rett Syndrome / complications*
Risk Factors
Seizures / etiology*,  genetics*
Grant Support
ID/Acronym/Agency:
1-R01-HD43100-01A1/HD/NICHD NIH HHS

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