Literature DB >> 3177444

Cognitive profiles of boys with the fragile X syndrome.

M B Kemper1, R J Hagerman, D Altshul-Stark.   

Abstract

Testing 20 boys with the fragile X (or Martin-Bell) syndrome with the Kaufman Assessment Battery for Children (K-ABC) showed a consistent pattern of strengths and weaknesses that may be useful in predicting a fragile X positive result from cytogenetic testing. This K-ABC pattern included 1) Sequential Scale score less than the Simultaneous Scale score; 2) Mental Processing Composite less than the Achievement Scale score; 3) Spatial Memory subtest score less than the Matrix Analogies subtest score; and 4) Arithmetic subtest score less than the mean of the Achievement subtest scores. A comparison group of 20 boys did not demonstrate such a pattern. Testing with the K-ABC should be considered for boys who present as learning disabled, hyperactive with attentional problems, or mildly retarded. Boys with three or four of the four features of the K-ABC fragile X pattern should be considered for medical evaluation and cytogenetic testing.

Entities:  

Mesh:

Year:  1988        PMID: 3177444     DOI: 10.1002/ajmg.1320300118

Source DB:  PubMed          Journal:  Am J Med Genet        ISSN: 0148-7299


  19 in total

1.  Temporal ordering deficits in female CGG KI mice heterozygous for the fragile X premutation.

Authors:  Michael R Hunsaker; Naomi J Goodrich-Hunsaker; Rob Willemsen; Robert F Berman
Journal:  Behav Brain Res       Date:  2010-05-15       Impact factor: 3.332

Review 2.  Comprehensive neurocognitive endophenotyping strategies for mouse models of genetic disorders.

Authors:  Michael R Hunsaker
Journal:  Prog Neurobiol       Date:  2012-01-13       Impact factor: 11.685

3.  Use of emotional cues for lexical learning: a comparison of autism spectrum disorder and fragile X syndrome.

Authors:  Angela John Thurman; Andrea McDuffie; Sara T Kover; Randi Hagerman; Marie Moore Channell; Ann Mastergeorge; Leonard Abbeduto
Journal:  J Autism Dev Disord       Date:  2015-04

4.  Executive Dysfunction in Female FMR1 Premutation Carriers.

Authors:  Annie L Shelton; Kim M Cornish; Claudine M Kraan; Reymundo Lozano; Minh Bui; Joanne Fielding
Journal:  Cerebellum       Date:  2016-10       Impact factor: 3.847

5.  Effect of speaker gaze on word learning in fragile X syndrome: a comparison with nonsyndromic autism spectrum disorder.

Authors:  David P Benjamin; Andrea S McDuffie; Angela J Thurman; Sara T Kover; Ann M Mastergeorge; Randi J Hagerman; Leonard Abbeduto
Journal:  J Speech Lang Hear Res       Date:  2015-04       Impact factor: 2.297

6.  Motor deficits on a ladder rung task in male and female adolescent and adult CGG knock-in mice.

Authors:  Michael R Hunsaker; Ramona E von Leden; Binh T Ta; Naomi J Goodrich-Hunsaker; Gloria Arque; Kyoungmi Kim; Rob Willemsen; Robert F Berman
Journal:  Behav Brain Res       Date:  2011-03-31       Impact factor: 3.332

7.  Working memory subsystems and task complexity in young boys with Fragile X syndrome.

Authors:  S Baker; S Hooper; M Skinner; D Hatton; J Schaaf; P Ornstein; D Bailey
Journal:  J Intellect Disabil Res       Date:  2010-12-01

8.  Glycogen synthase kinase-3 inhibitors reverse deficits in long-term potentiation and cognition in fragile X mice.

Authors:  Aimee V Franklin; Margaret K King; Valle Palomo; Ana Martinez; Lori L McMahon; Richard S Jope
Journal:  Biol Psychiatry       Date:  2013-09-13       Impact factor: 13.382

9.  Neurobehavioral effects of the fragile X premutation in adult women: a controlled study.

Authors:  A L Reiss; L Freund; M T Abrams; C Boehm; H Kazazian
Journal:  Am J Hum Genet       Date:  1993-05       Impact factor: 11.025

10.  Evidence that methylation of the FMR-I locus is responsible for variable phenotypic expression of the fragile X syndrome.

Authors:  A McConkie-Rosell; A M Lachiewicz; G A Spiridigliozzi; J Tarleton; S Schoenwald; M C Phelan; P Goonewardena; X Ding; W T Brown
Journal:  Am J Hum Genet       Date:  1993-10       Impact factor: 11.025

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