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Challenging Behaviors in Individuals with Fragile X Syndrome
 From the December 2001 Disabilities newsletter.Children and adults with fragile X syndrome can exhibit many challenging behaviors. According to Randi Hagerman, the Tsakopoulos-Vismara Professor of Pediatrics at the University of California at Davis School of Medicine, these behaviors can include hyperactivity, mood instability, sensory integration problems, anxiety, outbursts such as temper tantrums, and aggressive behaviors such as kicking and biting. People with fragile X also tend to have difficulties with executive function, leading to disorganization and obsessive-compulsive behaviors. Dr. Hagerman, who is also the medical director of the university's M.I.N.D. (Medical Investigation of Neurodevelopmental Disorders) Institute, notes that about 30% of children with fragile X also have autism or autism-like behaviors, such as poor eye contact or hand flapping. She also points out that girls with fragile X may have less hyperactivity than boys but can still have problems with organization as well as social anxiety and shyness. The Genetic Link Research into the genetic roots of behavior in people with fragile X has uncovered a connection to a lack of normal protein production of the fragile X mental retardation 1 (FMR1) gene, sequenced in 1991. It was also discovered that the amount of protein produced by the FMR1 gene (called FMRP) varied, depending on the individual. Since low FMRP levels have been found to cause changes in brain structure, challenging behaviors, as well as variations in behaviors, in people with fragile X are thought to be due to variations of FMRP. The next step in fragile X research, says Dr. Hagerman, is to develop molecular interventions. Researchers are working on finding ways to "turn on" the FMR1 gene and translate messenger RNA into protein. The goal is to get more protein into neurons to replace what the FMR1 gene does not produce naturally. Intervention In the meantime, Dr. Hagerman notes that both medical and behavioral interventions are available to help children and adults with fragile X syndrome. For children age 3 and older, a variety of medications (often in combination) is sometimes used. Stimulant medications, such as Ritalin (for hyperactivity), SSRIs, such as Paxil (for anxiety), and mood stabilizers, such as Risperdol, are usually prescribed depending on the person's specific needs. Since, according to Dr. Hagerman, about 20% of people with fragile X also have seizures, anti-convulsive medications are sometimes needed. Dr. Hagerman emphasizes that medicine is not the only recommended intervention for people with fragile X syndrome. Sensory integration, occupational, speech-language, and physical therapies may all be needed. Since many children with fragile X have language deficits and/or difficulty with math, computer programs that enhance abstract reasoning and comprehension skills can be helpful tools. As children with fragile X grow older, Dr. Hagerman reports that hyperactivity tends to decrease. Anxiety tends to worsen in adolescence, but eventually improves, as does mood instability. Dr. Hagerman notes that she would also like to see more research on improving vocational outcomes for adults with fragile X syndrome.
For information, see the M.I.N.D. Institute and its research and education programs.
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