A Comparison of Phonological Skills of Boys With Fragile X Syndrome and Down Syndrome

2005 ◽  
Vol 48 (5) ◽  
pp. 980-995 ◽  
Author(s):  
Joanne Roberts ◽  
Steven H. Long ◽  
Cheryl Malkin ◽  
Elizabeth Barnes ◽  
Martie Skinner ◽  
...  

In this study, the authors compared the phonological accuracy and patterns of sound change of boys with fragile X syndrome, boys with Down syndrome, and typically developing mental-age-matched boys. Participants were 50 boys with fragile X syndrome, ages 3 to 14 years; 32 boys with Down syndrome, ages 4 to 13 years; and 33 typically developing boys, ages 2 to 6 years, who were matched for nonverbal mental age to both the boys with fragile X syndrome and the boys with Down syndrome. All participants were administered a standardized articulation test, and their sound accuracy, phonological process, and proportion of whole-word proximity scores were analyzed. Although boys with fragile X syndrome were delayed in their speech development, they did not differ from the typically developing, mental-age-matched boys in the percentage of correct early-, middle-, and late-developing consonants; phonological processes; or whole-word proximity scores. Furthermore, boys with fragile X syndrome had fewer errors on early-, middle-, and late-developing consonants; fewer syllable structure processes; and higher whole-word proximity scores than did boys with Down syndrome. Boys with Down syndrome also were delayed in their speech development, yet their phonological inventories, occurrences of phonological processes, and proportion of whole-word proximity scores indicated greater delays in their phonological development than the younger, typically developing boys. These results suggest that males with fragile X syndrome display phonological characteristics in isolated words similar to younger, typically developing children, whereas males with Down syndrome show greater delays as well as some developmental differences compared with both the males with fragile X syndrome and typically developing males.

2021 ◽  
Vol 12 ◽  
Author(s):  
Eliseo Diez-Itza ◽  
Patricio Vergara ◽  
María Barros ◽  
Manuela Miranda ◽  
Verónica Martínez

In the context of comparing linguistic profiles across neurodevelopmental disorders, Down syndrome (DS) has captured growing attention for its uneven profile. Although specific weaknesses in grammatical and phonological processing have been reported, research evidence on phonological development remains scarce, particularly beyond early childhood. The purpose of this study was to explore the phonological profiles of children and adolescents with Down syndrome. The profiles were based on the frequency and relative proportion of the processes observed by classes, and they were compared to those of typically developing preschool children of similar verbal age. A complementary goal was to assess the effect of two different methods of elicitation: a test of articulation and spontaneous speech sampling. Finally, intergroup and intragroup differences in full match percentages between three positions at syllable-level (complex onset, medial coda, and final coda) were assessed. The results of the present study confirmed that the frequency of phonological processes in children and adolescents with DS is atypically high and is above what is expected for lexical age and at the same level as grammatical age. Highly increased frequency of processes, consistent in all kinds of processes and positions at the syllable-level, and asynchronous with verbal age and mental age suggest atypical developmental trajectories of phonological development in the Down syndrome population.


2017 ◽  
Vol 33 (3) ◽  
pp. 660
Author(s):  
Anibal Puente ◽  
Jesús M. Alvarado ◽  
Virginia Jiménez ◽  
Lourdes Martínez

<p>Fragile X Syndrome (FXS) and Down Syndrome (DS) read better than expected for their mental age. We have measured three basic reading skills (word recognition, phonological awareness, and reading comprehension), and two standard intellectual and verbal measures: the McCarthy Scales of Children’s Abilities and Peabody Picture Vocabulary Tests. The tests were applied to 16 adolescents classified as FXS (<em>M</em> = 14.74 years old, <em>SD</em> = 4.03) and 16 adolescents classified as DS (<em>M</em> = 15.59 years old, <em>SD</em> = 2.35). For comparison purposes, the reading tests were also applied to a typical develop group of 70 children aged between 4.8 and 7.0 years (<em>M</em> = 6.11, <em>SD</em> = 0.71).  Children with DS and FXS exhibited verbal skills superior to their cognitive development, especially in the FXS group. In reading performance, FXS showed a reading level corresponding to 5/6 their age, and for SD 6/7 of the equivalent reading age, however, both groups exhibited a similar reading performance in the three reading skills measured, when nonverbal mental age was controlled.</p>


2021 ◽  
Vol 126 (2) ◽  
pp. 167-181
Author(s):  
Kayla Smith ◽  
Abigail L. Hogan ◽  
Elizabeth Will ◽  
Jane E. Roberts

Abstract Early identification of behavioral risk markers for anxiety is essential to optimize long-term outcomes in children with neurodevelopmental disorders. This study analyzed attentional avoidance and its relation to anxiety and autism spectrum disorder (ASD) symptomatology during social and nonsocial fear conditions in toddlers with fragile X syndrome (FXS) and Down syndrome (DS). Toddlers with FXS and DS exhibited increased nonsocial attentional avoidance relative to typically developing (TD) toddlers. Attentional avoidance was not related to anxiety symptom severity in any group; however, higher ASD symptom severity was related to more social attentional avoidance in the FXS and TD groups. Findings suggest that there may be different underlying mechanisms driving attentional avoidance across neurodevelopmental disorders.


2002 ◽  
Vol 2 (3) ◽  
pp. 244-265 ◽  
Author(s):  
Annick Comblain ◽  
Mouna Elbouz

The language profile of individuals with fragile-X syndrome looks like that of individuals with Down syndrome, except for phonological and pragmatic abilities. Whereas the pragmatic aspect of language is relatively preserved in Down syndrome, it is one of the most impaired language components in fragile-X syndrome. Discourse of persons with fragile X generally contains repetitions, perseverations, and stereotypies. These behaviors are well documented in the literature but we do not know, for now, the reasons for these repetitions and perseverations. Are they the consequence of a real pragmatic disorder or the consequence of motor difficulties? Another aspect of the pragmatic component of language (in this pathology) remains almost unexplored: the common ground management and the organization of the old and the new information in conversation. It is this last point that we explore. We conducted a preliminary study with four boys with fragile-X aged from 6 to 12 years. Pairs of boys worked together on referential communication tasks. We presented traditional experimental situations in a non-eye contact condition between participants. We compared the results of our subjects with those of typically developing children matched for lexical age. Boys with Fragile-X were less efficient than were typically developing children when the message to give or receive contained spatial terms or “ordinal” attributes. They also managed less efficiently with an incomplete message, especially when it was given by an adult.


2021 ◽  
Vol 11 (5) ◽  
pp. 636
Author(s):  
Martina Fontana ◽  
Maria Carmen Usai ◽  
Sandra Pellizzoni ◽  
Maria Chiara Passolunghi

While previous research on inhibition in people with Down syndrome (DS) reported contradictory results, with no explicit theoretical model, on the other hand, a more homogeneous impaired profile on the delay of gratification skills emerged. The main goal of the present study was to investigate response inhibition, interference suppression, and delay of gratification in 51 individuals with DS matched for a measure of mental age (MA) with 71 typically developing (TD) children. Moreover, we cross-sectionally explored the strengths and weaknesses of these components in children and adolescents vs. adults with DS with the same MA. A battery of laboratory tasks tapping on inhibitory sub-components and delay of gratification was administrated. Results indicated that individuals with DS showed an overall worse performance compared to TD children on response inhibition and delay of gratification, while no differences emerged between the two samples on the interference suppression. Additionally, our results suggested that older individuals with DS outperformed the younger ones both in response inhibition and in the delay of gratification, whereas the interference suppression still remains impaired in adulthood. This study highlights the importance of evaluating inhibitory sub-components considering both MA and chronological age in order to promote more effective and evidence-based training for this population.


Author(s):  
Jane E. Roberts ◽  
Bridgette Tonnsen ◽  
Ashley Robinson ◽  
Svetlana V. Shinkareva

Abstract The present study contrasted physiological arousal in infants and toddlers with fragile X syndrome to typically developing control participants and examined physiological predictors early in development to autism severity later in development in fragile X syndrome. Thirty-one males with fragile X syndrome (ages 8–40 months) and 25 age-matched control participants were included. The group with fragile X syndrome showed shorter interbeat intervals (IBIs), lower vagal tone (VT), and less modulation of IBI. Data suggested a nonlinear effect with IBI and autistic behavior; however, a linear effect with VT and autistic behavior emerged. These findings suggest that atypical physiological arousal emerges within the first year and predicts severity of autistic behavior in fragile X syndrome. These relationships are complex and dynamic, likely reflecting endogenous factors assumed to reflect atypical brain function secondary to reduced fragile X mental retardation protein. This research has important implications for the early identification and treatment of autistic behaviors in young children with fragile X syndrome.


2016 ◽  
Vol 30 (5) ◽  
pp. 970-974 ◽  
Author(s):  
Wilmar Saldarriaga ◽  
Fabian Andres Ruiz ◽  
Flora Tassone ◽  
Randi Hagerman

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