Introduction

Arousal refers to a general state of nervous system activation that is reflected in behavior, physiological activity and emotional experience. There is an optimum level of arousal that is necessary for controlled behavior, and this level is usually maintained by internal homeostatic mechanisms. One of the characteristics of Fragile X syndrome is an impairment of this homeostatic control of arousal. Another is a heightened sensitivity to environmental and social stimulation. Together, these factors combine to cause boys and girls with Fragile X syndrome to become aroused more easily, and to a greater extent, than others, and to remain in such a “hyperaroused” state for prolonged periods of time. While hyperaroused, such individuals are less able to control their behavior. They may act impulsively, have difficulty focusing and sustaining attention to tasks, exhibit speech and language impairments, and emit other “problem” behaviors.

Diagnosis & Recognition

The hyperarousal discussed above is believed to underlie many of the phenotypical behaviors commonly associated with Fragile X syndrome. Individuals experiencing hyperarousal may become flushed, their ears and cheeks may become red, and their palms may begin to sweat. Their behavior becomes disorganized and they may begin to engage in repetitive movements. Some may become obstinate and not wish to continue an activity or to remain in the stimulating environment. They may become unfocused, inattentive, impulsive, aggressive, and display symptoms that mimic anxiety. Their behavior may become disorganized, repetitive and less efficient. Their speech may also become repetitive and perseverative.

Current Treatment Recommendations

When problem behaviors occur, it is important to recognize that they may be caused by hyperarousal, and to first try to identify any sources that may be overstimulating the person. The initial intervention should always be to try to reduce environmental stimulation. When that is not possible, it may be advisable to remove the person from the overstimulating environment. A non-stimulating, quiet and “safe” place should be designated, so that when hyperarousal occurs, the individual can have a place to quickly retreat to where he will be able to dearouse, regain his composure, and re-establish a sense of well-being before returning to his previous activity.

Proactive strategies to prevent hyperarousal include modifying the physical environments of individuals with FXS (e.g., their homes, classrooms, and workplaces) to reduce their stimulating quality. Examples might include providing natural or incandescent lighting instead of fluorescent lighting, and introducing sound-dampening material such as curtains or acoustic tiles in order to minimize sound reverberation and echo.

It is important to recognize that the sources of stimulation vary within environments such as classrooms, and to situate individuals away from arousing stimuli. For example, seat the child away from the bell that signals class changes, or away from open doors that admit distracting sounds from adjunct rooms or hallways.

When exposure to intense stimulation is unavoidable, such as during assemblies, fire drills or other busy events, a familiar person should accompany the individual to help him remain calm and provide continuity.

Unfamiliarity and unpredictability can be arousing, and attempts to reduce them for the person with Fragile X syndrome are highly advisable. Strategies along these lines may include engaging in role play to help an individual anticipate social situations, or providing picture schedules to prepare her for changes in activities or routines.

An occupational therapist can provide valuable insight and advice regarding accommodations to improve an individual’s physical and social environment to reduce its arousing quality, and can also recommend ways to provide an appropriate sensory diet to help her maintain control and feel calm and focused throughout the day.

Teaching individuals with Fragile X syndrome personal stress-reducing strategies such as “square breathing,” muscle relaxation techniques, and visualization of tranquil places, can also help them cope with unavoidable exposures to stimulation.

Medication may also be helpful in some cases, by reducing the person’s threshold for becoming hyperaroused when exposed to stimulating environments. See Medications for Individuals with Fragile X Syndrome.

Frequently Asked Questions

Additional Resources

We have articles and other materials on sensory integration and hyperarousal that can provide additional insights and suggestions regarding the effects of hyperarousal and strategies for preventing and coping with it.

Behavioral Challenges in Fragile X Syndrome — Practitioners
From our Treatment and Interventions Recommendations: The most common behavioral challenges seen in Fragile X syndrome include behaviors associated with generalized anxiety, social interaction difficulties (this includes social anxiety-withdrawal and social aloofness), attention deficit hyperactivity disorder (ADHD), self-injury, and aggression. Addressing these challenges needs to be done with consideration to the person’s level of intellectual functioning, speech/language abilities, and ASD symptoms, if applicable.

The Top 10 Things Teachers Should Know About Fragile X Syndrome: Strengths, Challenges, and How They Learn — Educators
There’s really only one thing every teacher needs to know about Fragile X syndrome: Students with Fragile X syndrome are prone to hyperarousal and anxiety. It’s how their nervous systems are wired.The most effective way to help a student get the most from their time at school is to minimize the likelihood of hyperarousal and anxiety, thus maximizing their focus and cooperation.

Questions?