Influence of stimulants on electrodermal studies in Fragile X syndrome

Hagerman, Randi J.; Miller, Lucy; McGrath-Clarke, Jude; Riley, Karen; Goldson, Edward; Harris, Susan; Simon, Jody; Church, Kelly; Bonnell, Julie; Ognibene, Todd C.; McIntosh, Daniel N.

doi:10.1002/jemt.10067

Cited by 37 publications

(28 citation statements)

References 25 publications

(24 reference statements)

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“…One cannot rule out the possibility that the fmr1 KO mouse might exhibit impaired transfer of learning in some other types of tasks, but the available data indicate that this aspect of cognition is intact in this mouse model and, by extension, in humans with FXS. If so, this finding would add to the growing evidence that the dysfunction in FXS is quite specific, limited primarily to aspects of executive functioning (including inhibitory control and attention), arousal regulation, and social behavior (see Cornish et al, 2004c;Hagerman et al, 2002).…”

Section: Discussionmentioning

confidence: 90%

Attentional dysfunction, impulsivity, and resistance to change in a mouse model of fragile X syndrome.

Moon¹,

Beaudin²,

Verosky³

et al. 2006

Behavioral Neuroscience

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Fragile X syndrome (FXS) is the most common inherited form of mental retardation, occurring in roughly 1/4000 males and 1/8000 females. An abnormal expansion of a trinucleotide CGG repeat sequence in the fmr1 gene results in transcriptional silencing of this gene, which codes for the Fragile X Mental Retardation Protein (FMRP). The loss of FMRP, directly and/or indirectly, gives rise to the FXS phenotype, which includes a characteristic set of anatomic and cognitive/behavioral features. The present studies were designed to test the hallmark areas of dysfunction (i.e. attention, inhibitory control, hyperarousal, and emotional regulation) seen in human FXS and further characterize spared and impaired functions in fmr1 KO mice. The performance of F1 hybrid fmr1 KO mice (a C57BL/6J x FVB/NJ cross) and wild-type (WT) littermate controls were evaluated on a series of tasks designed to assess inhibitory control and various aspects of attention, Reversal Learning Task, and Associate Learning Task. Regulation of arousal and emotion, two domains affected in FXS, was also evaluated in these tasks by examining the animals' reaction to the unexpected presentation of potent olfactory distractors (in the Distraction task), as well as their reaction to committing an error on the previous trial.The present studies provided the first evidence that the hallmark deficits in human FXS --impaired attention, inhibitory control, and arousal regulation -are also impaired in the fmr1 mouse model of FXS. In addition, these findings demonstrate that attentional dysfunction and impaired inhibitory control are most prominent when task contingencies change and when the animal has just committed an errorsituations that arouse or disturb the mice. Analysis of videotapes further demonstrates that arousal regulation is impaired in the fmr1 KO mice. Additionally, the fmr1 KO mice were not impaired in associative learning, transfer of learning, or reversal Jisook's stidies in New Generation stimulated her desire for a deeper and moe systematic understanding of the neural mechanisms of human brain. She was accepted into the Department of Psychology at Yonsei University, which is considered one of the best programs in Korea. This allowed her the unique combination of being able to study at Yonsei University and to work part time for New Generation C&C. Working and studying at the same time allowed her many opportunities to apply academic knowledge to real-world situations.To the end, Jisook assisted Dr. Kang in her lab, the Cognitive Neuroscience and Medical Physics Lab at the Seoul National University Hospital. There, she conducted studies on memory and learning in normal human conditions, as well as abnormal states of perception such as epilepsy and dementia. Through cutting-edge imaging methods (i.e. fMRI, PET, and MRI) coupled with some behavioral tests, she furthered her knowledge of functional and structural workings of the human brain iv during learning, memorizing, and recognizing. She also ganed insight into how the activity of the brain dif...

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Section: Discussionmentioning

confidence: 90%

Attentional dysfunction, impulsivity, and resistance to change in a mouse model of fragile X syndrome.

Moon¹,

Beaudin²,

Verosky³

et al. 2006

Behavioral Neuroscience

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“…Roberts et al [2002] recently reported that boys with FXS on stimulants had better attention, lower motor activity levels, and higher academic test scores on medicated days versus unmedicated days, although levels of physiological arousal were unaffected by stimulant treatment. Electrodermal studies measuring an enhanced sweat response to stimuli in children with FXS did show a decrease in response toward normal after stimulant treatment [Hagerman et al, 2002]. Taken together, currently available information suggests that stimulants are quite helpful in managing distractibility and hyperactivity symptoms in a subgroup of boys and girls with FXS presenting with prominent difficulty in these behavioral domains.…”

Section: Stimulantsmentioning

confidence: 95%

Psychopharmacology in fragile X syndrome—Present and future

Berry‐Kravis

Potanos

2004

Ment. Retard. Dev. Disabil. Res. Rev.

137

135

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In addition to cognitive disability, fragile X syndrome (FXS) is associated with behavioral problems that are often functionally limiting. There are few controlled trials to guide treatment; however, available information does suggest that medications can be quite helpful for a number of categories of behavioral disturbance in FXS. Specifically, stimulants appear to be quite useful for management of distractibility, hyperactivity, and impulsive behavior; antidepressants help with anxiety, obsessive-compulsive behaviors and mood dysregulation; and antipsychotics can reduce aggression. These medications are supportive and help minimize dysfunctional behaviors and maximize functioning. As more is learned about the neural functions of FMRP, medications in the future will be expected to target specific synaptic mechanisms dysregulated in FXS brain and thus ameliorate the cognitive deficit with resultant behavioral improvements. This article summarizes knowledge about effectiveness and approaches to management of currently available psychopharmacology for behavior in FXS and discusses early leads to future treatments for cognition.

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“…Although both reflect sensory gating mechanisms, available evidence suggests that different neural circuits mediate the two responses (Hong et al 2007). In addition to these gating paradigms, measurement of response to sensory stimuli has been conducted using electrodermal responses (EDR) (Blair 1999;Crowell et al 2006;Hagerman et al 2002;McIntosh et al 1999;Miller et al 1999;Venables 1977).…”

Section: Electrophysiological Measurementmentioning

confidence: 99%

Diagnostic Validity of Sensory Over-Responsivity: A Review of the Literature and Case Reports

Reynolds

Lane

2007

J Autism Dev Disord

137

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Atypical responses to sensory stimulation are frequently reported to co-occur with diagnoses such as autism, ADHD, and Fragile-X syndrome. It has also been suggested that children and adults may present with atypical sensory responses while failing to meet the criteria for other medical or psychological diagnoses. This may be particularly true for individuals with over-responsivity to sensation. This article reviews the literature related to sensory over-responsivity and presents three pediatric cases that present a profile of having sensory over-responsivity without a co-occurring diagnosis. Findings from these cases provide very preliminary evidence to support the suggestion that sensory over-responsivity can occur as a sole diagnosis. Within this small group, tactile overresponsivity was the most common and pervasive form of this condition.Diminished or exaggerated responses to typical sensory stimulation have been described as a feature of many prominent diagnostic conditions. The terms under-responsiveness and over-responsiveness have appeared as the most current descriptors of these behaviors (Miller et al. 2007). Attempts to understand, classify, research, and treat both under and over-responsiveness have been ongoing since they were first described by Ayres (1965). None-theless, after 40 years of work, our understanding of sensory processing patterns within and across groups, and their relationship to functional behaviors, continues to be imprecise.The umbrella term Sensory Modulation Dysfunction (SMD) is currently being used to encompass both over and under-responsivity, along with the overlapping or fluctuating responsivity. Sensory seeking has also been suggested as a subtype of SMD (Miller et al. 2007). This has been questioned, however, since seeking behaviors have been found in populations exhibiting both over and under-responsive (Liss et al. 2006). Liss and colleagues have suggested that sensory seeking is more a compensatory mechanism used to moderate high arousal levels, at least by children with Autism Spectrum Disorder.Some investigators have attempted to identify subgroups of individuals with sensory processing disorder, with the most prominent model presented by Dunn (Dunn and Brown 1997;Dunn 1999). Dunn's model suggests that individuals could be classified based on their neurological threshold and behavioral responses to incoming stimuli. According to this model high neuronal thresholds are indicative of a nervous system that requires a stronger or more intense input to elicit a behavioral response, suggestive of an under-responsive nervous system. Lower neuronal thresholds, on the other hand, are indicative of a nervous system that requires less intense or less frequent stimulation to fire, suggestive of an over-responsive nervous system. The model further highlights that individuals can respond either in accordance with their neurological threshold, or act to counteract their threshold. For example, individuals with high thresholds acting in accordance with

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Influence of stimulants on electrodermal studies in Fragile X syndrome

Cited by 37 publications

References 25 publications

Attentional dysfunction, impulsivity, and resistance to change in a mouse model of fragile X syndrome.

Attentional dysfunction, impulsivity, and resistance to change in a mouse model of fragile X syndrome.

Psychopharmacology in fragile X syndrome—Present and future

Diagnostic Validity of Sensory Over-Responsivity: A Review of the Literature and Case Reports

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