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All fields Title Creator / Publisher Keyword Year AND OR AND NOT All fields Title Creator / Publisher Keyword Year AND OR AND NOT All fields Title Creator / Publisher Keyword Year AND OR AND NOT All fields Title Creator / Publisher Keyword Year AND OR AND NOT All fields Title Creator / Publisher Keyword Year Sort by creator [A → Z]' creator [Z → A]' publishing year ↑ (asc)' publishing year ↓ (desc)' title [A → Z]' title [Z → A]' Simple Search Page: 1 2 3 4 5...186 Hits 1 – 20 of 3.701 1 Family history of FXTAS is associated with age-related cognitive-linguistic decline among mothers with the FMR1 premutation. Klusek, Jessica; Fairchild, Amanda; Moser, Carly; Mailick, Marsha R; Thurman, Angela John; Abbeduto, Leonard 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 BASE Hide details 2 Single-word severity and intelligibility in CAS (Chenausky et al., 2022) ... Chenausky, Karen V.; Gagné, Danielle; Stipancic, Kaila L.. - : ASHA journals, 2022 BASE Show details 3 Specific speech errors predict literacy skills (Boada et al., 2022) ... Boada, Katheryn L.; Boada, Richard; F. Pennington, Bruce. - : ASHA journals, 2022 BASE Show details 4 Specific speech errors predict literacy skills (Boada et al., 2022) ... Boada, Katheryn L.; Boada, Richard; F. Pennington, Bruce. - : ASHA journals, 2022 BASE Show details 5 Processing Phonotactic Illegality (Steinberg et al., 2016) ... Steinberg, Johanna; Jacobsen, Thomas Konstantin; Jacobsen, Thomas. - : ASHA journals, 2022 BASE Show details 6 Intoxication and pitch control in tonal and non-tonal language speakers ... Chang, Charles. - : Open Science Framework, 2022 BASE Show details 7 Local temporal regularities in Spanish child-directed speech ... Pérez-Navarro, Jose. - : Open Science Framework, 2022 BASE Show details 8 Materials for Tang et al. (2022) ... Chang, Charles. - : Open Science Framework, 2022 BASE Show details 9 Speech production in very preterm children (van Noort-van der Spek et al., 2022) ... van Noort-van der Spek, Inge L.; Dudink, Jeroen; Reiss, Irwin K.. - : ASHA journals, 2022 BASE Show details 10 A Mandarin Chinese–Oriented Sound Test (Hung et al., 2016) ... Hung, Yu-Chen; Lin, Chun-Yi; Tsai, Li-Chiun. - : ASHA journals, 2022 BASE Show details 11 Processing Phonotactic Illegality (Steinberg et al., 2016) ... Steinberg, Johanna; Jacobsen, Thomas Konstantin; Jacobsen, Thomas. - : ASHA journals, 2022 BASE Show details 12 Speech production in very preterm children (van Noort-van der Spek et al., 2022) ... van Noort-van der Spek, Inge L.; Dudink, Jeroen; Reiss, Irwin K.. - : ASHA journals, 2022 BASE Show details 13 A Mandarin Chinese–Oriented Sound Test (Hung et al., 2016) ... Hung, Yu-Chen; Lin, Chun-Yi; Tsai, Li-Chiun. - : ASHA journals, 2022 BASE Show details 14 On the benefits of speech-language therapy for cleft lip and/or palate (Sand et al., 2022) ... Sand, Anders; Hagberg, Emilie; Lohmander, Anette. - : ASHA journals, 2022 BASE Show details 15 Dataset for Tang et al. (2022) ... Chang, Charles. - : Open Science Framework, 2022 BASE Show details 16 On the benefits of speech-language therapy for cleft lip and/or palate (Sand et al., 2022) ... Sand, Anders; Hagberg, Emilie; Lohmander, Anette. - : ASHA journals, 2022 BASE Show details 17 COLORINCO ... , Caudrelier. - : Open Science Framework, 2022 BASE Show details 18 Single-word severity and intelligibility in CAS (Chenausky et al., 2022) ... Chenausky, Karen V.; Gagné, Danielle; Stipancic, Kaila L.. - : ASHA journals, 2022 BASE Show details 19 Corpus of British Isles Spoken English ... Coats, Steven; Englantilainen Filologia. - : Steven Coats, 2022 BASE Show details 20 Task and language in Spanish–English narratives (Wofford et al., 2022) ... Wofford, Mary Claire; Cano, Jessica; Goodrich, J. Marc. - : ASHA journals, 2022 BASE Show details Page: 1 2 3 4 5...186

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