A tool to assess language, called expressive language sampling (ELS), found that people with fragile X syndrome have less complex speech, more disruptions in speech, greater talkativeness, and a lesser ability to be understood than those with typical development, a study reported.
Besides showing greater deficits in males than in females with fragile X, these results also validate ELS as an accurate measure of language abilities in this disorder.
The study, “The Relationship between Expressive Language Sampling and Clinical Measures in Fragile X Syndrome and Typical Development,” was published in the journal Brain Sciences.
Delayed language development in areas such as vocabulary, putting words together to form phrases and sentences (morphosyntax), and how context contributes to meaning (pragmatics) are well-known in people with fragile X.
High rates of verbal perseverance, such as repetitive topics and words, are also common to these patients.
As standard methods lack sensitivity, focus groups run by the National Institutes of Health identified the need for testing strategies to accurately measure the development of language in those with fragile X.
One such strategy is ELS, which offers a more natural measurement of language compared to typical standardized assessments.
Researchers at the University of Cincinnati College of Medicine used ELS in 25 fragile X patients as well as 23 people with typical development, ages 5 to 36, serving as controls.
Two techniques are used in ELS: a conversation between two people (dyadic) and communicating a narrative. The team chose the narrative form (ELS-N), with participants given the wordless picture book “Frog Goes to Dinner.” After an introduction, they were encouraged to detail the story page by page in their own words.
Recordings were transcribed and analyzed for outcome measures that included sentence structure, complexity of syntax, number of different root words used (lexical diversity), talkativeness, speech planning, and whether the words were comprehensible (intelligibility).
In addition, IQ was measured using the Stanford-Binet 5 Brief. Parents or caregivers completed the Vineland Adaptive Behavior Scales, the Aberrant Behavior Checklist (ABC, which assesses irritability, social withdrawal, repetitive behavior, hyperactivity, and inappropriate speech), the Anxiety, Depression, and Mood Scale (ADAMS), and the Social Communication Questionnaire (SCQ).
Results showed that, compared to controls, those with fragile X had significantly lesser syntax complexity, lower diversity in words used, more disruptions of ongoing speech, greater talkativeness, and lesser intelligibility.
Within the fragile X group, males were significantly more talkative and less intelligible than females. No gender differences were found within the control group.
Cognitive tests in the fragile X group showed that females performed better than males, with males having higher hyperactivity and anxiety scores. Males also scored lower on the SCQ, a measure for autistic-like characteristics.
A statistical analysis associated better cognitive skills with higher lexical diversity, more complex syntax, and greater intelligibility in those with fragile X. Hyperactivity was linked to increased talkativeness and being less intelligible, while autistic characteristics were also linked to poorer intelligibility in speech.
Increased talkativeness was associated with more compulsive behaviors, inappropriate speech, hyperactivity, and higher SCQ scores. Again, no significant links were found among the control group.
“This study adds to the growing research evidence supporting the use of ELS-N as an outcome measure for individuals with FXS [fragile X syndrome],” the scientists wrote.
“Future studies should examine the relationship between ELS-N variables and CGG repeat numbers [the genetic basis of fragile X], seek to replicate the relationships between ELS-N and the clinical measures included in this study, particularly with measures of hyperactivity and talkativeness, and examine ELS-N in a larger sample size,” they added.
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