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Psych: When being ‘Fragile’ takes on a whole new meaning: An overview for teachers
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Fragile X syndrome is the most frequent cause of hereditary mental retardation around the world, with one in 2,500 boys experiencing the cognitive, behavioural and physical challenges of this genetic disorder. Fragile X occurs when the FMR-1 gene is absent on the X chromosome.
The disorder creates some autism-like characteristics in terms of anxiety, attention problems, and the ability to process information sequentially, but people with Fragile X are often quite social but can appear shy and introvert.
The profile of learning problems in Fragile X are more severe in boys, who do not benefit from the buffering of a second X chromosome as girls do. Many young adults with Fragile X with the right training opportunities can make a meaningful transition to the workforce and live productive lives. The key is to ensure that all individuals with Fragile X have educational plans that target their specific strengths and weaknesses across development.
Fragile X syndrome is passed down through the generations. The expansion of the CGG gene sequence on the X chromosome can lead to the absence of the FMR-1 gene, which is important for brain functioning and development. The normal level of CGG repeats is approximately 30, but carriers will have between 55 and 200 repeats, the upper limit being the point at which the full mutation occurs and a person is born with Fragile X syndrome. CGG repeats increase as the syndrome moves further down the family line.
Approximately one in 600 women and one in 800 men are Fragile X carriers, and although it was once thought that these carriers experienced no symptoms of the disorder, Professor Kim Cornish now promotes the idea of a “spectrum of Fragile X”. For example, a male Fragile X carrier may function intellectually at a normal level while having problems with inhibition.
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