Children with fragile X syndrome have a bias toward threatening emotion

(Elsevier) Anxiety occurs at high rates in children with fragile X syndrome (FXS), the most common form of inherited intellectual disability. Children with co-occurring anxiety tend to fare worse, but it can be hard to identify in infants. A new study in Biological Psychiatry: Cognitive Neuroscience and Neuroimaging reports that infants and children with FXS show bias toward threatening emotion, rather than positive emotion, a pattern highly linked with anxiety.
Source: EurekAlert! - Social and Behavioral Science - Category: International Medicine & Public Health Source Type: news

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Introduction: Fragile X Syndrome (FXS) is the leading form of inherited intellectual disability and autism spectrum disorder, caused by a tri-nucleotide CGG repeat expansion in the promoter region of the FMR1 gene [1]. The cognitive profile in FXS includes deficits in executive control and in visuospatial abilities, as well as in language and severe behavioural alterations with hyperactivity, impulsivity, anxiety: the condition often is associated with medical comorbidities among which epilepsy [1].
Source: Gait and Posture - Category: Orthopaedics Authors: Source Type: research
We report remarkably similar neural oscillation phenotypes in the Fmr1 KO mouse including enhanced resting state gamma power and reduced evoked gamma synchronization. The gamma band inter-trial coherence of neural response was reduced in both auditory and frontal cortex of Fmr1 KO mice stimulated with a sound whose envelope was modulated from 1 to 100 Hz, similar to that seen in humans with FXS. These deficits suggest a form of enhanced 'resting state noise' that interferes with the ability of the circuit to mount a synchronized response to sensory input, predicting specific sensory and cognitive deficits in FXS. The abn...
Source: Neurobiology of Disease - Category: Neurology Authors: Tags: Neurobiol Dis Source Type: research
This study investigates whether a newly discovered STEP inhibitor (TC-2153) could attenuate the behavioral and synaptic abnormalities in Fmr1 KO mice. TC-2153 reversed audiogenic seizure incidences, reduced hyperactivity, normalized anxiety states, and increased sociability in Fmr1 KO mice. Moreover, TC-2153 reduced dendritic spine density and improved synaptic aberrations in Fmr1 KO neuronal cultures as well as in vivo. TC-2153 also reversed the mGluR-mediated exaggerated LTD in brain slices derived from Fmr1 KO mice. These studies suggest that STEP inhibition may have therapeutic benefit in FXS. PMID: 28943283 [PubM...
Source: Neuropharmacology - Category: Drugs & Pharmacology Authors: Tags: Neuropharmacology Source Type: research
ConclusionsIn women with the FMR1 premutation, elevated optimism may reduce the occurrence or severity of MDD and anxiety disorders. These findings underscore the importance of supporting mental health across the FMR1 spectrum of involvement.
Source: Journal of Intellectual Disability Research - Category: Disability Authors: Tags: Original Manuscript Source Type: research
American Journal on Intellectual and Developmental Disabilities,Volume 122, Issue 5, Page 359-373, September 2017.
Source: American Journal on Intellectual and Developmental Disabilities - Category: Disability Authors: Source Type: research
ConclusionThese findings highlight the importance of including female subjects in preclinical studies, as simply studying the impact of genetic mutations in males does not yield a complete picture of the phenotype. Further research should explore these marked phenotypic differences among the sexes. Moreover, given that treatment strategies are typically equivalent between the sexes, the results highlight a potential need for sex‐specific therapeutics. Few investigations into the behavioral phenotype of the Fmr1 knockout mouse have also investigated females. The results of this study provide evidence for a sex‐specific...
Source: Brain and Behavior - Category: Neurology Authors: Tags: ORIGINAL RESEARCH Source Type: research
This study ’s other authors are Daniel Cantu, Anubhuti Goel, Shilpa Mantri and William Zeiger, all of UCLA.The study was funded by a Paul and Daisy Soros Fellowship for New Americans and an NIH NINDS F30 Fellowship (NS093719); UCLA Neural Microcircuits training grant (T32-NS058280); a Eugene V. Cota-Robles Fellowship; the UCLA Medical Scientist Training Program (NIH NIGMS training grant GM08042; and a Developmental Disabilities Translational Research Program grant (20160969) from the John Merck Fund; a SFARI grant from the Simons Foundation (295438); and NIH NICHD grant (RO1 HD054453).
Source: UCLA Newsroom: Health Sciences - Category: Universities & Medical Training Source Type: news
Authors: Martin LA, Porter AG, Pelligrini VA, Schnatz PA, Jiang X, Kleinstreuer N, Hall JE, Verbiest S, Olmstead J, Fair R, Falorni A, Persani L, Rajkovic A, Mehta K, Nelson LM Abstract Most clinicians are not prepared to provide integrated personal care to address all the clinical needs of women with primary ovarian insufficiency. Design thinking is an engineering methodology used to develop and evaluate novel concepts for systems operation. Here we articulate the need for a seamlessly integrated mobile health system to support genomic research as well as patient care. We also review the pathophysiology and manage...
Source: Panminerva Medica - Category: Journals (General) Tags: Panminerva Med Source Type: research
Advances in human genetics have identified a significant number of genetic disorders associated with intellectual disability. As a result, appropriate clinical management of these affected individuals and their family members have become critical in addressing medical needs to improve quality of life. We examine the importance of a Fragile X Clinic for individuals with fragile X syndrome (FXS) and their family members by conducting a retrospective chart review of 123 new patients with FXS evaluated at the Fragile X Clinic at Emory University. After the initial diagnosis of a proband with FXS with cascade testing, there wer...
Source: American Journal of Medical Genetics Part A - Category: Genetics & Stem Cells Authors: Tags: Original Article Source Type: research
Abstract Fragile X syndrome (FXS) is an inheritable neuropsychological disease caused by expansion of the CGG trinucleotide repeat affecting the fmr1 gene on X chromosome, resulting in silence of the fmr1 gene and failed expression of FMRP. Patients with FXS suffer from cognitive impairment, sensory integration deficits, learning disability, anxiety, autistic traits, and so forth. Specifically, the morbidity of anxiety in FXS individuals remains high from childhood to adulthood. By and large, it is common that the change of brain plasticity plays a key role in the progression of disease. But for now, most studies ...
Source: Neural Plasticity - Category: Neurology Authors: Tags: Neural Plast Source Type: research
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