Literature DB >> 3177438

Fragile X syndrome: growth, development, and intellectual function.

L A Prouty1, R C Rogers, R E Stevenson, J H Dean, K K Palmer, R J Simensen, G N Coston, C E Schwartz.   

Abstract

We collected data on growth, psychomotor development, speech and language development, and intellectual function on a cohort of 100 males with the fragile X chromosome and 95 carrier females. The data include information on prenatal growth (33 males), growth during the preadult years (32 males), psychomotor development during the first 2 years (25 males), speech and language development (15 males and 5 females), and intellectual function (93 males, 33 females, and 10 obligate carriers who were cytogenetically normal). Birth measurements appeared normal when plotted on the Usher/McLean curves of newborn infants (mean head circumference - OFC - at 40th centile, length at 60th centile and weight at 55th centile). Following birth, OFC rose above the 50th percentile and continued above average throughout the preadult years, whereas average length was above average for the first 5 years only and weight did not deviate from the normal mean. Psychomotor development lagged behind the norm from birth with affected males requiring nearly twice as long as expected to sit alone, walk unassisted, and say first words clearly. All males and females studied had significant language delay; all except one male had abnormalities of articulation. All on whom a clear voice sample was obtained had low voice pitch, and 80% had a hoarse or harsh quality of voice. Five males had word repetitions or perseverative speech during the preadult years. The mean IQ of the 93 males studied was 33 and regression analysis demonstrated a decrease in intellectual performance with age. Four fifths of the female carriers who expressed the fra(X) had intellectual performance in the mentally retarded range and showed similar decrease in performance with age. Obligate female carriers who did not express the fra(X) site had normal IQs (IQ 102 +/- 13.3).

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Year:  1988        PMID: 3177438     DOI: 10.1002/ajmg.1320300111

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


  10 in total

1.  Perseveration in the connected speech of boys with Fragile X syndrome with and without autism spectrum disorder.

Authors:  Gary E Martin; Joanne E Roberts; Nancy Helm-Estabrooks; John Sideris; Jacqueline Vanderbilt; Lauren Moskowitz
Journal:  Am J Intellect Dev Disabil       Date:  2012-09

2.  Macrostructural narrative language of adolescents and young adults with Down syndrome or fragile X syndrome.

Authors:  Lizbeth H Finestack; Meghan Palmer; Leonard Abbeduto
Journal:  Am J Speech Lang Pathol       Date:  2011-11-02       Impact factor: 2.408

3.  Linkage analysis of families with fragile-X mental retardation, using a novel RFLP marker (DXS 304).

Authors:  N Dahl; P Goonewardena; H Malmgren; K H Gustavson; G Holmgren; E Seemanova; G Annerén; A Flood; U Pettersson
Journal:  Am J Hum Genet       Date:  1989-08       Impact factor: 11.025

4.  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

Review 5.  Fragile X syndrome: A review of clinical management.

Authors:  Reymundo Lozano; Atoosa Azarang; Tanaporn Wilaisakditipakorn; Randi J Hagerman
Journal:  Intractable Rare Dis Res       Date:  2016-08

6.  Phonological accuracy and intelligibility in connected speech of boys with fragile X syndrome or Down syndrome.

Authors:  Elizabeth Barnes; Joanne Roberts; Steven H Long; Gary E Martin; Mary C Berni; Kerry C Mandulak; John Sideris
Journal:  J Speech Lang Hear Res       Date:  2009-08       Impact factor: 2.297

7.  Neuroanatomy in fragile X females: the posterior fossa.

Authors:  A L Reiss; L Freund; J E Tseng; P K Joshi
Journal:  Am J Hum Genet       Date:  1991-08       Impact factor: 11.025

8.  Development of socio-communicative skills in 9- to 12-month-old individuals with fragile X syndrome.

Authors:  Peter B Marschik; Katrin D Bartl-Pokorny; Jeff Sigafoos; Leo Urlesberger; Florian Pokorny; Robert Didden; Christa Einspieler; Walter E Kaufmann
Journal:  Res Dev Disabil       Date:  2014-01-27

9.  Parents' initial concerns about the development of their children later diagnosed with fragile X syndrome.

Authors:  Dajie Zhang; Walter E Kaufmann; Jeff Sigafoos; Katrin D Bartl-Pokorny; Magdalena Krieber; Peter B Marschik; Christa Einspieler
Journal:  J Intellect Dev Disabil       Date:  2016-09-18

10.  Characterizing the Richness of Maternal Input for Word Learning in Neurogenetic Disorders.

Authors:  Laura J Mattie; Pamela A Hadley
Journal:  Semin Speech Lang       Date:  2021-07-26       Impact factor: 1.734

  10 in total

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