Document Detail


Is fragile X syndrome a pervasive developmental disability? Cognitive ability and adaptive behavior in males with the full mutation.
MedLine Citation:
PMID:  7942997     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
In addition to mental retardation (MR), fragile X [fra(X)] syndrome has been associated with various psychopathologies, although it appears that the link is secondary to MR. It has been proposed that individuals with the full mutation be classified as a subcategory of pervasive developmental disorders (PDD). If fra(X) males are to be categorized as PDD, how do they compare with other types of developmental disabilities? We examined 27 fra(X) males aged 3-14 years, from 4 sites in North America. Measures of cognitive abilities were obtained from the Stanford-Binet Fourth Edition (SBFE), while levels of adaptive behavior were evaluated using the Vineland Adaptive Behavior Scales (VABS). Control subjects were sex-, age-, and IQ matched children and adolescents ascertained from the Developmental Evaluation Clinic (DEC) at Kings County Hospital. At the DEC, control subjects were diagnosed as either MR (n = 43) or autistic disorder (AD; n = 22). To compare subjects' adaptive behavior (SQ) with their cognitive abilities (IQ), a ratio of [(SQ/IQ) x 100] was computed. Results graphed as cumulative distribution functions (cdf) revealed that the cdf for AD males, who by definition are socially impaired, was positioned to the left of the cdf for MR controls, as expected. Mean ratio for AD males (70) was lower than for MR males (84). On the other hand, the cdf for fra(X) males was positioned far to the right of either AD or MR controls (mean ratio = 125). Statistical tests showed that SQ of fra(X) males was significantly higher than controls.(ABSTRACT TRUNCATED AT 250 WORDS)
Authors:
G S Fisch; J J Holden; R Simensen; N Carpenter; P N Howard-Peebles; A Maddalena; A Sandgrund; J R Jacques; B McGann
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Publication Detail:
Type:  Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  American journal of medical genetics     Volume:  51     ISSN:  0148-7299     ISO Abbreviation:  Am. J. Med. Genet.     Publication Date:  1994 Jul 
Date Detail:
Created Date:  1994-11-16     Completed Date:  1994-11-16     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7708900     Medline TA:  Am J Med Genet     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  346-52     Citation Subset:  IM    
Affiliation:
Dept. of Psychiatry, Kings County Hospital Center, Brooklyn, NY 11203.
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MeSH Terms
Descriptor/Qualifier:
Adaptation, Psychological
Adolescent
Age Factors
Analysis of Variance
Autistic Disorder / classification
Case-Control Studies
Child
Child Development Disorders, Pervasive / classification,  genetics*
Child, Preschool
Fragile X Syndrome / classification*,  genetics,  psychology*
Gene Dosage
Humans
Intelligence
Linear Models
Male
Mental Retardation / classification
Mutation*
Oklahoma
Ontario
Repetitive Sequences, Nucleic Acid
South Carolina
Stanford-Binet Test
Virginia

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


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