Adaptive behavior important in inherited intellectual disability syndrome
Standard scores measuring "adaptive behavior" in boys with fragile X syndrome tend to decline during childhood and adolescence, the largest longitudinal study of the inherited disorder to date has found.
Adaptive behavior covers a range of everyday social and practical skills, including communication, socialization, and completing tasks of daily living such as getting dressed. In this study, socialization emerged as a relative strength in boys with fragile X, in that it did not decline as much as the other two domains of adaptive behavior measured: communication and daily living skills.
Fragile X syndrome is the leading inherited cause of intellectual disability and the leading single-gene risk factor for autism spectrum disorder. It affects one in 4000 boys and one in 8000 girls, and boys tend to be more severely affected, because boys have just one X chromosome while girls have two. Measured IQ shows a range from average to severe intellectual disability, with an average IQ of 40 in males who have complete silencing of the FMR1 gene.
The results are published in the journal Pediatrics.
The study's findings will help child development specialists refine educational programs for individuals with fragile X syndrome, and could inform the design of clinical trials now underway for drugs that target the molecular deficiencies arising in fragile X syndrome.
Adaptive behavior is often studied in individuals with intellectual disability, because it indicates someone's ability to function independently.
"Adaptive behavior is separate from measures of intelligence such as IQ, but it can be a more important predictor of success," says lead author Cheryl Klaiman, PhD, formerly of the Stanford University Center for Interdisciplinary Brain Sciences Center, now senior psychologist at Marcus Autism Center and assistant professor of pediatrics at Emory University School of Medicine.
"Our findings highlight the need for continued attention to adaptive behavior skills for individuals with fragile X syndrome, in both special education programs and home-based care."
Researchers from Stanford and the University of North Carolina followed the families of 275 children and adolescents with fragile X syndrome (186 were boys) and 225 typically developing children and adolescents. 60 percent of the typical controls were siblings of study participants with fragile X.
Study participants' caregivers were interviewed using the Vineland Adaptive Behavior Scales every two to four years. The Vineland framework asks caregivers whether the child never, sometimes/partially or always performs a given task, and was divided into socialization, communication and daily living realms.
For typically developing children, their average standard score in each area was around 100 and the average generally remained steady with age. For boys with fragile X, the average standard score for all three areas starts at around 60 in early childhood and declines significantly into the teens. Daily living skills did increase in males with fragile X after 14 years of age. This gain could perhaps be due to the increased efforts focused on teaching these skills prior to graduating from high school.
The decline doesn't mean that boys with fragile X are losing skills over time, Klaiman says.
"It does mean that they are not keeping pace with their typically developing peers of the same age, or expectations based on their initial scores in early childhood," she says.
Adaptive behavior scores for girls with fragile X start lower, compared to typically developing children, but do not decline as much as males' scores do over time. As a group, their scores also have a wider range. For girls, communication declines more than other domains.
Quinn Eastman | Eurek Alert!
The importance of biodiversity in forests could increase due to climate change
17.11.2017 | Deutsches Zentrum für integrative Biodiversitätsforschung (iDiv) Halle-Jena-Leipzig
Win-win strategies for climate and food security
02.10.2017 | International Institute for Applied Systems Analysis (IIASA)
The WHO reports an estimated 429,000 malaria deaths each year. The disease mostly affects tropical and subtropical regions and in particular the African continent. The Fraunhofer Institute for Silicate Research ISC teamed up with the Fraunhofer Institute for Molecular Biology and Applied Ecology IME and the Institute of Tropical Medicine at the University of Tübingen for a new test method to detect malaria parasites in blood. The idea of the research project “NanoFRET” is to develop a highly sensitive and reliable rapid diagnostic test so that patient treatment can begin as early as possible.
Malaria is caused by parasites transmitted by mosquito bite. The most dangerous form of malaria is malaria tropica. Left untreated, it is fatal in most cases....
The formation of stars in distant galaxies is still largely unexplored. For the first time, astron-omers at the University of Geneva have now been able to closely observe a star system six billion light-years away. In doing so, they are confirming earlier simulations made by the University of Zurich. One special effect is made possible by the multiple reflections of images that run through the cosmos like a snake.
Today, astronomers have a pretty accurate idea of how stars were formed in the recent cosmic past. But do these laws also apply to older galaxies? For around a...
Just because someone is smart and well-motivated doesn't mean he or she can learn the visual skills needed to excel at tasks like matching fingerprints, interpreting medical X-rays, keeping track of aircraft on radar displays or forensic face matching.
That is the implication of a new study which shows for the first time that there is a broad range of differences in people's visual ability and that these...
Computer Tomography (CT) is a standard procedure in hospitals, but so far, the technology has not been suitable for imaging extremely small objects. In PNAS, a team from the Technical University of Munich (TUM) describes a Nano-CT device that creates three-dimensional x-ray images at resolutions up to 100 nanometers. The first test application: Together with colleagues from the University of Kassel and Helmholtz-Zentrum Geesthacht the researchers analyzed the locomotory system of a velvet worm.
During a CT analysis, the object under investigation is x-rayed and a detector measures the respective amount of radiation absorbed from various angles....
The quantum world is fragile; error correction codes are needed to protect the information stored in a quantum object from the deteriorating effects of noise. Quantum physicists in Innsbruck have developed a protocol to pass quantum information between differently encoded building blocks of a future quantum computer, such as processors and memories. Scientists may use this protocol in the future to build a data bus for quantum computers. The researchers have published their work in the journal Nature Communications.
Future quantum computers will be able to solve problems where conventional computers fail today. We are still far away from any large-scale implementation,...
15.11.2017 | Event News
15.11.2017 | Event News
30.10.2017 | Event News
21.11.2017 | Physics and Astronomy
21.11.2017 | Physics and Astronomy
21.11.2017 | Life Sciences