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Family history of FXTAS is associated with age-related cognitive-linguistic decline among mothers with the FMR1 premutation.
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In: Journal of neurodevelopmental disorders, vol 14, iss 1 (2022)
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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.
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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
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URL: https://escholarship.org/uc/item/2m78r62b
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Spectatoriale Geschlechterkonstruktionen: Geschlechtsspezifische Wissens- und Welterzeugung in den französisch- und spanischsprachigen Moralischen Wochenschriften des 18. Jahrhunderts
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Völkl, Yvonne. - : transcript Verlag, 2022. : DEU, 2022. : Bielefeld, 2022
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In: Gender Studies ; 355 (2022)
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Impact of factors specific to forced migration on German language acquisition: Family constellation, health status and housing situation
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In: 4-2020 ; BAMF-Brief Analysis ; 13 (2022)
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Neural dynamics of semantic categorization in semantic variant of primary progressive aphasia.
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Resting functional connectivity in the semantic appraisal network predicts accuracy of emotion identification.
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Adolescent Protective and Risk Factors for Incarceration through Early Adulthood
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In: JOURNAL OF CHILD AND FAMILY STUDIES, vol 30, iss 6 (2021)
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Development of thalamus mediates paternal age effect on offspring reading: A preliminary investigation.
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In: Human brain mapping, vol 42, iss 14 (2021)
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The effect of distance and frequency factors on the prominence of referential entities ; L’effet des facteurs de distance et de fréquence sur la saillance des entités référentielles
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Evidence of the interplay of genetics and culture in Ethiopia. ...
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Web indirect language assessment on monolingual Italian toddlers ...
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Sexualität - Geschlecht - Affekt: Sexuelle Scripts als Palimpsest in literarischen Erzähltexten und zeitgenössischen theoretischen Debatten
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In: Gender Studies ; 326 (2021)
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Do women and men use language differently in spoken face-to-face interaction? A scoping review
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In: Review of Communication Research ; 9 ; 43-79 (2021)
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The emotional impact of mixed proficiency levels in the corporate language in multinational teams
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Evidence of the interplay of genetics and culture in Ethiopia.
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In: essn: 2041-1723 ; nlmid: 101528555 (2021)
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Problems in Foreign Language Education in the Turkish Education System: Pre-Service Teachers’ Accounts
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In: Eurasian Journal of Applied Linguistics, Vol 7, Iss 1, Pp 397-419 (2021) (2021)
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Cognitive and environmental predictors of academic skills in French middle school students ; Prédicteurs cognitifs et environnementaux des apprentissages scolaires chez les élèves de collège en France
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In: https://tel.archives-ouvertes.fr/tel-03375054 ; Psychology. Sorbonne Université, 2020. English. ⟨NNT : 2020SORUS240⟩ (2020)
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| 18 |
Expressive language development in adolescents with Down syndrome and fragile X syndrome: change over time and the role of family-related factors.
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In: Journal of neurodevelopmental disorders, vol 12, iss 1 (2020)
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Comparing two facets of emotion perception across multiple neurodegenerative diseases.
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In: Social cognitive and affective neuroscience, vol 15, iss 5 (2020)
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Amount and delay insensitivity during intertemporal choice in three neurodegenerative diseases reflects dorsomedial prefrontal atrophy.
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