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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
Cortical microstructure in primary progressive aphasia: a multicenter study.
In: Alzheimer's research & therapy, vol 14, iss 1 (2022)
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3
Many Changes in Speech through Aging Are Actually a Consequence of Cognitive Changes
In: International Journal of Environmental Research and Public Health; Volume 19; Issue 4; Pages: 2137 (2022)
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4
Chemotherapy-Related Cognitive Impairment in Patients with Breast Cancer Based on Functional Assessment and NIRS Analysis
In: Journal of Clinical Medicine; Volume 11; Issue 9; Pages: 2363 (2022)
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5
Speech- and Language-Based Classification of Alzheimer’s Disease: A Systematic Review
In: Bioengineering; Volume 9; Issue 1; Pages: 27 (2022)
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6
Validation of the Visual Cognitive Assessment Test (VCAT) for the Early Diagnosis of Cognitive Impairment in Multilingual Population in Malaysia
In: Psych; Volume 4; Issue 1; Pages: 38-48 (2022)
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7
Síntomas cognitivos en la COVID-19 persistente: un análisis neuropsicológico
Pérez Sánchez, María del Carmen. - : Universitat Oberta de Catalunya (UOC), 2022
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8
Altered Inhibitory Mechanisms in Parkinson’s Disease: Evidence From Lexical Decision and Simple Reaction Time Tasks
In: ISSN: 1662-5161 ; Frontiers in Human Neuroscience ; https://hal.archives-ouvertes.fr/hal-03236439 ; Frontiers in Human Neuroscience, Frontiers, 2021, 15, ⟨10.3389/fnhum.2021.624026⟩ (2021)
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9
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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10
What (more) can verbal fluency tell us about multiple sclerosis?
In: ISSN: 1877-0657 ; Annals of Physical and Rehabilitation Medicine ; https://hal-univ-fcomte.archives-ouvertes.fr/hal-03630141 ; Annals of Physical and Rehabilitation Medicine, Elsevier Masson, 2021, 64 (2), pp.101394. ⟨10.1016/j.rehab.2020.05.002⟩ (2021)
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11
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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12
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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13
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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14
What Do We Mean by Behavioral Disinhibition in Frontotemporal Dementia?
Magrath Guimet, Nahuel; Miller, Bruce L; Allegri, Ricardo F. - : eScholarship, University of California, 2021
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15
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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16
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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17
A New Test of Irony and Indirect Requests Comprehension—The IRRI Test: Validation and Normative Data in French-Speaking Adults
In: ISSN: 0887-6177 ; Archives of Clinical Neuropsychology ; https://hal.archives-ouvertes.fr/hal-03337764 ; Archives of Clinical Neuropsychology, Oxford University Press (OUP), 2021, ⟨10.1093/arclin/acab043⟩ (2021)
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18
Cross-cultural cognitive assessment of dementia: a meta-analysis of the impact of illiteracy on dementia screening and an evaluation of a transcultural short-term memory assessment ...
Maher, Caragh. - : The University of Edinburgh, 2021
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19
Conceptual framework for the definition of preclinical and prodromal frontotemporal dementia. ...
Benussi, Alberto; Alberici, Antonella; Samra, Kiran. - : Apollo - University of Cambridge Repository, 2021
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20
Impact of stimulus variability on the understanding of reversible sentences in adolescents with Developmental Language Disorder: learning vs. generalisation. ...
Parker, Adam. - : Open Science Framework, 2021
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