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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
Cortical microstructure in primary progressive aphasia: a multicenter study.
In: Alzheimer's research & therapy, vol 14, iss 1 (2022)
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3
Improving the diagnostic accuracy of primary progressive aphasia using cognitive tests
Foxe, David Gordon. - : The University of Sydney, 2022. : Faculty of Science, School of Psychology, 2022
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4
A Meta-Analysis of Semantic Memory in Mild Cognitive Impairment
In: ISSN: 1040-7308 ; EISSN: 1573-6660 ; Neuropsychology Review ; https://hal.archives-ouvertes.fr/hal-03015335 ; Neuropsychology Review, Springer Verlag, 2021, ⟨10.1007/s11065-020-09453-5⟩ (2021)
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5
Cognitive Impairment in Late-Life Depression: A Comparative Study of Healthy Older People, Late-Life Depression, and Mild Alzheimer's Disease Using Multivariate Base Rates of Low Scores
In: EISSN: 1664-1078 ; Frontiers in Psychology ; https://hal-univ-fcomte.archives-ouvertes.fr/hal-03630024 ; Frontiers in Psychology, Frontiers, 2021, 12, pp.724731. ⟨10.3389/fpsyg.2021.724731⟩ (2021)
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6
Uniform data set language measures for bvFTD and PPA diagnosis and monitoring.
In: Alzheimer's & dementia (Amsterdam, Netherlands), vol 13, iss 1 (2021)
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7
Social Behavior Observer Checklist: Patterns of Spontaneous Behaviors Differentiate Patients With Neurodegenerative Disease From Healthy Older Adults.
Rankin, Katherine P; Toller, Gianina; Gavron, Lauren. - : eScholarship, University of California, 2021
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8
Resting functional connectivity in the semantic appraisal network predicts accuracy of emotion identification.
Yang, Winson FZ; Toller, Gianina; Shdo, Suzanne. - : eScholarship, University of California, 2021
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9
Uniform data set language measures for bvFTD and PPA diagnosis and monitoring.
In: Alzheimer's & dementia (Amsterdam, Netherlands), vol 13, iss 1 (2021)
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10
Asian Americans and Pacific Islanders' perspectives on participating in the CARE recruitment research registry for Alzheimer's disease and related dementias, aging, and caregiving research.
In: Alzheimer's & dementia (New York, N. Y.), vol 7, iss 1 (2021)
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11
APOE alleles' association with cognitive function differs across Hispanic/Latino groups and genetic ancestry in the study of Latinos-investigation of neurocognitive aging (HCHS/SOL).
In: Alzheimer's & dementia : the journal of the Alzheimer's Association, vol 17, iss 3 (2021)
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12
The New Face of Caregiving: Multidimensional Factors of Caregiver Burden Among Young Adult Caregivers
Demko, Courtney Marie. - : eScholarship, University of California, 2021
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13
Data for: Correlates of Anomia in non-semantic Variants of Primary Progressive Aphasia Converge over Time ...
Leyton, Cristian. - : Mendeley, 2021
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14
Data for: Correlates of Anomia in non-semantic Variants of Primary Progressive Aphasia Converge over Time ...
Leyton, Cristian. - : Mendeley, 2021
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15
Student Project: Semantic Priming and Alzheimer's Disease (Oliver Rimkus)- Template ...
Nowak, Alexander. - : Open Science Framework, 2021
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16
Aprendre idiomes de gran crea connexions neuronals beneficioses
Bueno i Torrens, David, 1965-. - : Edició de Premsa Periòdica Ara, 2021
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17
Deterioros lingüísticos asociados a un caso clínico de demencia tipo alzhéimer ; Language impairments associated with a clinical case of Alzheimer’s dementia
Jiménez Ruiz, Juan Luis; Palenzuela Sánchez, Ángeles. - : Universidad Nacional Autónoma de México, 2021
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18
Network Effects Of Reduced Audibility (NEORA) and its Significance for Cognitive Decline
Alcock, C. - : University of Exeter, 2021. : College of Engineering, Mathematics, and Physical Sciences, 2021
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19
Edinburgh Cognitive and Behavioural ALS Screen (ECAS) in the assessment of early onset dementia
De Icaza Valenzuela, Mónica Mariana. - : The University of Edinburgh, 2021
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20
Distinctive Oculomotor Behaviors in Alzheimer's Disease and Frontotemporal Dementia
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