Document Detail


Late-onset Lennox-Gastaut syndrome in a patient with 15q11.2-q13.1 duplication.
MedLine Citation:
PMID:  19396834     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The 4 Mb 15q11-q13 region is prone to structural rearrangements. Deletions have been identified among the leading causes for genetic diseases such as the Prader-Willi and Angelman syndromes, while duplications, occurring preferentially on the maternal chromosome, produce a typical phenotype that includes mental retardation, language delay, seizures and autism. Although a number of such patients have been reported, however, there is a paucity of information about their clinical outcomes in adult age. We report on a 33-year-old female with a microduplication of 15q11-q13 detected by array-CGH analysis, with particular reference to the epilepsy phenotype, characterized as a late-onset Lennox-Gastaut syndrome.
Authors:
Alfredo Orrico; Marcella Zollino; Lucia Galli; Sabrina Buoni; Giuseppe Marangi; Vincenzo Sorrentino
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of medical genetics. Part A     Volume:  149A     ISSN:  1552-4833     ISO Abbreviation:  Am. J. Med. Genet. A     Publication Date:  2009 May 
Date Detail:
Created Date:  2009-05-04     Completed Date:  2009-06-18     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1033-5     Citation Subset:  IM    
Affiliation:
Molecular Medicine, Azienda Ospedaliera Universitaria Senese, Siena, Italy. a.orrico@ao-siena.toscana.it
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MeSH Terms
Descriptor/Qualifier:
Adult
Age of Onset
Autistic Disorder / genetics*
Chromosomes, Human, Pair 15 / genetics*
Comparative Genomic Hybridization
Female
Gene Duplication
Humans
Language Development Disorders / genetics*
Mental Retardation / genetics*
Oligonucleotide Array Sequence Analysis
Seizures / genetics*
Syndrome

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


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