scholarly journals Developmental trajectory of communication repair in children with Fragile X Syndrome

2020 ◽  
Vol 5 ◽  
pp. 239694152090901 ◽  
Author(s):  
Heather Fielding-Gebhardt ◽  
Steven F Warren ◽  
Nancy C Brady
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Developmental Trajectory

Caregiver Reports of Sleep Problems on a Convenience Sample of Children With Fragile X Syndrome

2009 ◽  
Vol 114 (6) ◽  
pp. 383-392 ◽  
Author(s):  
Rebecca Kronk ◽  
Ronald Dahl ◽  
Robert Noll
Keyword(s):  
Fragile X Syndrome ◽  
Assessment Tool ◽  
Sleep Problems ◽  
Fragile X ◽  
Demographic Variables ◽  
Developmental Trajectory ◽  
Sleep Diary ◽  
Sleep Habits ◽  
Convenience Sample ◽  
Children’S Sleep

Abstract Caregivers reported on sleep in a convenience sample of 90 children with fragile X syndrome utilizing a standardized assessment tool, the Children's Sleep Habits Questionnaire (CSHQ), and a 14-day sleep diary. CSHQ data indicated that 47% of participants had sleep problems at a level that suggested referral and further evaluation. Sleep diary data indicated high rates of several sleep problems. These problems did not appear to follow a typical developmental trajectory and were not related to gender or demographic variables. Nineteen percent of the sample was currently receiving medication to improve sleep; however, there were no significant differences between those receiving medications and those not receiving medications.

scholarly journals Sertraline May Improve Language Developmental Trajectory in Young Children with Fragile X Syndrome: A Retrospective Chart Review

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Tri Indah Winarni ◽  
Weerasak Chonchaiya ◽  
Evan Adams ◽  
Jacky Au ◽  
Yi Mu ◽  
...  
Keyword(s):  
Young Children ◽  
Fragile X Syndrome ◽  
Chart Review ◽  
Fragile X ◽  
Retrospective Chart Review ◽  
Developmental Trajectory

scholarly journals Sensory hypo-excitability in a rat model of fetal development in Fragile X Syndrome

2016 ◽  
Vol 6 (1) ◽  
Author(s):  
Julia Berzhanskaya ◽  
Marnie A. Phillips ◽  
Jing Shen ◽  
Matthew T. Colonnese
Keyword(s):  
Fragile X Syndrome ◽  
Rat Model ◽  
Fragile X ◽  
Gamma Oscillations ◽  
Developmental Trajectory ◽  
Inhibitory Neurons ◽  
Visual Responses ◽  
Age Appropriate ◽  
Eye Opening ◽  
Hyper Sensitivity

Abstract Fragile X syndrome (FXS) is characterized by sensory hyper-sensitivity, and animal models suggest that neuronal hyper-excitability contributes to this phenotype. To understand how sensory dysfunction develops in FXS, we used the rat model (FMR-KO) to quantify the maturation of cortical visual responses from the onset of responsiveness prior to eye-opening, through age equivalents of human juveniles. Rather than hyper-excitability, visual responses before eye-opening had reduced spike rates and an absence of early gamma oscillations, a marker for normal thalamic function at this age. Despite early hypo-excitability, the developmental trajectory of visual responses in FMR-KO rats was normal, and showed the expected loss of visually evoked bursting at the same age as wild-type, two days before eye-opening. At later ages, during the third and fourth post-natal weeks, signs of mild hyper-excitability emerged. These included an increase in the visually-evoked firing of regular spiking, presumptive excitatory, neurons, and a reduced firing of fast-spiking, presumptive inhibitory, neurons. Our results show that early network changes in the FMR-KO rat arise at ages equivalent to fetal humans and have consequences for excitability that are opposite those found in adults. This suggests identification and treatment should begin early, and be tailored in an age-appropriate manner.

scholarly journals Development of Neural Response to Novel Sounds in Fragile X Syndrome: Potential Biomarkers

2020 ◽  
Vol 125 (6) ◽  
pp. 449-464
Author(s):  
Lauren Ethridge ◽  
Andrew Thaliath ◽  
Jeremy Kraff ◽  
Karan Nijhawan ◽  
Elizabeth Berry-Kravis
Keyword(s):  
Fragile X Syndrome ◽  
Auditory Processing ◽  
Fragile X ◽  
Event Related Potentials ◽  
Developmental Trajectory ◽  
Oddball Paradigm ◽  
Retest Reliability ◽  
Gamma Power ◽  
Related Potentials ◽  
Potential Biomarkers

Abstract Auditory processing abnormalities in fragile X syndrome (FXS) may contribute to difficulties with language development, pattern identification, and contextual updating. Participants with FXS (N = 41) and controls (N = 27) underwent auditory event-related potentials during presentation of an oddball paradigm. Data was adequate for analysis for 33 participants with FXS and 27 controls (age 4–51 y, 13 females [FXS]; 4–54 y, 11 females [control]). Participants with FXS showed larger N1 and P2 amplitudes, abnormal lack of modulation of P1 and P2 amplitudes and P2 latency in response to oddball stimuli ) relative to controls: Females with FXS were more similar to controls. Participants with FXS showed a marginal speeding of the P2 latency, suggesting potentiation to oddball stimuli rather than habituation. Participants with FXS showed a heightened N1 habituation effect compared to controls. Gamma power was significantly higher for participants with FXS. Groups did not differ on mismatch negativity. Both controls and participants with FXS showed similar developmental trajectories in P1 and N1 amplitude, P2 latency, and gamma power, but not for P2 amplitude. One month retest analyses performed in 14 participants suggest strong test-retest reliability for most measures. Individuals with FXS show previously demonstrated increased response amplitude and high frequency neural activity. Despite an overall normal developmental trajectory for most measures, individuals with FXS show age-independent but gender-dependent decreases in complex processing of novel stimuli. Many markers show strong retest reliability even in children and thus are potential biomarkers for clinical trials in FXS.

scholarly journals Sustained correction of associative learning deficits after brief, early treatment in a rat model of Fragile X Syndrome

2019 ◽  
Vol 11 (494) ◽  
pp. eaao0498 ◽  
Author(s):  
Antonis Asiminas ◽  
Adam D. Jackson ◽  
Susana R. Louros ◽  
Sally M. Till ◽  
Teresa Spano ◽  
...  
Keyword(s):  
Associative Learning ◽  
Fragile X Syndrome ◽  
Associative Memory ◽  
Rat Model ◽  
Fragile X ◽  
Time Windows ◽  
Treatment Strategies ◽  
Developmental Time ◽  
Developmental Trajectory ◽  
Pharmacological Intervention

Fragile X Syndrome (FXS) is one of the most common monogenic forms of autism and intellectual disability. Preclinical studies in animal models have highlighted the potential of pharmaceutical intervention strategies for alleviating the symptoms of FXS. However, whether treatment strategies can be tailored to developmental time windows that define the emergence of particular phenotypes is unknown. Similarly, whether a brief, early intervention can have long-lasting beneficial effects, even after treatment cessation, is also unknown. To address these questions, we first examined the developmental profile for the acquisition of associative learning in a rat model of FXS. Associative memory was tested using a range of behavioral paradigms that rely on an animal’s innate tendency to explore novelty. Fmr1 knockout (KO) rats showed a developmental delay in their acquisition of object-place recognition and did not demonstrate object-place-context recognition paradigm at any age tested (up to 23 weeks of age). Treatment of Fmr1 KO rats with lovastatin between 5 and 9 weeks of age, during the normal developmental period that this associative memory capability is established, prevents the emergence of deficits but has no effect in wild-type animals. Moreover, we observe no regression of cognitive performance in the FXS rats over several months after treatment. This restoration of the normal developmental trajectory of cognitive function is associated with the sustained rescue of both synaptic plasticity and altered protein synthesis. The findings provide proof of concept that the impaired emergence of the cognitive repertoire in neurodevelopmental disorders may be prevented by brief, early pharmacological intervention.

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