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1
Family history of FXTAS is associated with age-related cognitive-linguistic decline among mothers with the FMR1 premutation.
In: Journal of neurodevelopmental disorders, vol 14, iss 1 (2022)
Abstract: BackgroundWomen who carry a premutation allele of the FMR1 gene are at increased vulnerability to an array of age-related symptoms and disorders, including age-related decline in select cognitive skills. However, the risk factors for age-related decline are poorly understood, including the potential role of family history and genetic factors. In other forms of pathological aging, early decline in syntactic complexity is observed and predicts the later onset of neurodegenerative disease. To shed light on the earliest signs of degeneration, the present study characterized longitudinal changes in the syntactic complexity of women with the FMR1 premutation across midlife, and associations with family history of fragile X-associated tremor/ataxia syndrome (FXTAS) and CGG repeat length.MethodsForty-five women with the FMR1 premutation aged 35-64years at study entry participated in 1-5 longitudinal assessments spaced approximately a year apart (130 observations total). All participants were mothers of children with confirmed fragile X syndrome. Language samples were analyzed for syntactic complexity and participants provided information on family history of FXTAS. CGG repeat length was determined via molecular genetic testing.ResultsHierarchical linear models indicated that women who reported a family history of FXTAS exhibited faster age-related decline in syntactic complexity than those without a family history, with that difference emerging as the women reached their mid-50s. CGG repeat length was not a significant predictor of age-related change.ConclusionsResults suggest that women with the FMR1 premutation who have a family history of FXTAS may be at increased risk for neurodegenerative disease, as indicated by age-related loss of syntactic complexity. Thus, family history of FXTAS may represent a personalized risk factor for age-related disease. Follow-up study is needed to determine whether syntactic decline is an early indicator of FXTAS specifically, as opposed to being a more general age-related cognitive decline associated with the FMR1 premutation.
Keyword: 2.1 Biological and endogenous factors; Acquired Cognitive Impairment; Adult; Aging; Alleles; Alzheimer's Disease; Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD); Ataxia; Behavioral and Social Science; Brain Disorders; Child; Clinical Research; Cognitive Dysfunction; Dementia; Female; Fragile X Mental Retardation Protein; Fragile X premutation; Fragile X Syndrome; Genetics; Grammatical complexity; Humans; Intellectual and Developmental Disabilities (IDD); Language Disorders; Language production; Middle Aged; Mothers; Neurodegenerative; Neurodegenerative Diseases; Neurological; Neurosciences; Pediatric; Psychology; Rare Diseases; Tremor
URL: https://escholarship.org/uc/item/2m78r62b
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2
Providing a parent-administered outcome measure in a bilingual family of a father and a mother of two adolescents with ASD: brief report.
In: Developmental neurorehabilitation, vol 25, iss 2 (2022)
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3
Tablet Word Learning (TabletSwitch) ...
Tsui, Rachel. - : Open Science Framework, 2022
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4
Supplemental Materials from Long-range sequential dependencies precede complex syntactic production in language acquisition ...
Sainburg, Tim; Mai, Anna; Gentner, Timothy Q.. - : The Royal Society, 2022
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5
Supplemental Materials from Long-range sequential dependencies precede complex syntactic production in language acquisition ...
Sainburg, Tim; Mai, Anna; Gentner, Timothy Q.. - : The Royal Society, 2022
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6
Exploring stimulus onset timings: The effect of repeating object and word pairings on adults novel object recognition ...
Brown, Anna. - : Open Science Framework, 2022
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7
Potential of automatic speech processing technologies for early detection of oral language disorders: a meta-analytic review ...
Bonnet, Camille. - : Open Science Framework, 2022
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8
Two-year-olds' eye movements reflect confidence in their understanding of words ...
Dautriche, Isabelle. - : Open Science Framework, 2022
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9
Monolinguals and Bilinguals’ Visual Recognition Memory of Socially Relevant Stimuli at 8-10 Months. ...
Freda, Kate. - : Open Science Framework, 2022
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10
Gender Does Not Equal Genitalia: A Review of the Implications of Inclusivity in School-Based Sexual Health Education on the Identity Development of Non-Binary and Transgender Adolescents
In: University Honors Theses (2022)
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11
Visual generics: How children understand generic language with different visualizations ...
Menendez, David. - : Open Science Framework, 2022
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12
Writing Competence and Grammatical Errors of the Written Discourses of HUMSS 11 Students ...
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13
a charanga da LILI ...
Costa, Patricia Luíza. - : figshare, 2022
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14
Writing Competence and Grammatical Errors of the Written Discourses of HUMSS 11 Students ...
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15
a charanga da LILI ...
Costa, Patricia Luíza. - : figshare, 2022
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16
Writing Competence and Grammatical Errors of the Written Discourses of HUMSS 11 Students ...
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17
Phonological and semantic specialization in 9- to 10-year-old children during auditory word processing ...
Wang, Jin. - : Open Science Framework, 2022
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18
Neural pathways of phonological and semantic processing and its relations to children’s reading skills ...
Wagley, Neelima. - : Open Science Framework, 2022
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19
Homophone acquisition across semantic categories based on limited exposure ...
Gomes, Victor. - : Open Science Framework, 2022
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20
SemPhonD ...
Campbell, Erin. - : Open Science Framework, 2022
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