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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 Hits 1 – 20 of 57 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 Cortical microstructure in primary progressive aphasia: a multicenter study. Illán-Gala, Ignacio; Montal, Victor; Borrego-Écija, Sergi... In: Alzheimer's research & therapy, vol 14, iss 1 (2022) BASE Show details 3 Uniform data set language measures for bvFTD and PPA diagnosis and monitoring. Staffaroni, Adam M; Weintraub, Sandra; Rascovsky, Katya... In: Alzheimer's & dementia (Amsterdam, Netherlands), vol 13, iss 1 (2021) BASE Show details 4 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 BASE Show details 5 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 BASE Show details 6 What Do We Mean by Behavioral Disinhibition in Frontotemporal Dementia? Magrath Guimet, Nahuel; Miller, Bruce L; Allegri, Ricardo F. - : eScholarship, University of California, 2021 BASE Show details 7 Uniform data set language measures for bvFTD and PPA diagnosis and monitoring. Staffaroni, Adam M; Weintraub, Sandra; Rascovsky, Katya... In: Alzheimer's & dementia (Amsterdam, Netherlands), vol 13, iss 1 (2021) BASE Show details 8 New directions in clinical trials for frontotemporal lobar degeneration: Methods and outcome measures. Boxer, Adam L; Gold, Michael; Feldman, Howard... In: Alzheimer's & dementia : the journal of the Alzheimer's Association, vol 16, iss 1 (2020) BASE Show details 9 Comparing two facets of emotion perception across multiple neurodegenerative diseases. Brown, Casey L; Hua, Alice Y; De Coster, Lize... In: Social cognitive and affective neuroscience, vol 15, iss 5 (2020) BASE Show details 10 Amount and delay insensitivity during intertemporal choice in three neurodegenerative diseases reflects dorsomedial prefrontal atrophy. Beagle, Alexander J; Zahir, Ali; Borzello, Mia. - : eScholarship, University of California, 2020 BASE Show details 11 Depressive Symptom Profiles Predict Specific Neurodegenerative Disease Syndromes in Early Stages. Shdo, Suzanne M; Ranasinghe, Kamalini G; Sturm, Virginia E. - : eScholarship, University of California, 2020 BASE Show details 12 Utility of the global CDR® plus NACC FTLD rating and development of scoring rules: Data from the ARTFL/LEFFTDS Consortium. Miyagawa, Toji; Brushaber, Danielle; Syrjanen, Jeremy... In: Alzheimer's & dementia : the journal of the Alzheimer's Association, vol 16, iss 1 (2020) BASE Show details 13 Use of the CDR® plus NACC FTLD in mild FTLD: Data from the ARTFL/LEFFTDS consortium. Miyagawa, Toji; Brushaber, Danielle; Syrjanen, Jeremy... In: Alzheimer's & dementia : the journal of the Alzheimer's Association, vol 16, iss 1 (2020) BASE Show details 14 Depressive Symptom Profiles Predict Specific Neurodegenerative Disease Syndromes in Early Stages. Shdo, Suzanne M; Ranasinghe, Kamalini G; Sturm, Virginia E. - : eScholarship, University of California, 2020 BASE Show details 15 New directions in clinical trials for frontotemporal lobar degeneration: Methods and outcome measures. Boxer, Adam L; Gold, Michael; Feldman, Howard... In: Alzheimer's & dementia : the journal of the Alzheimer's Association, vol 16, iss 1 (2020) BASE Show details 16 Deformation-based shape analysis of the hippocampus in the semantic variant of primary progressive aphasia and Alzheimer's disease. Chapleau, Marianne; Bedetti, Christophe; Devenyi, Gabriel A. - : eScholarship, University of California, 2020 BASE Show details 17 Graded, multidimensional intra- and intergroup variations in primary progressive aphasia and post-stroke aphasia. Ingram, Ruth U; Halai, Ajay D; Pobric, Gorana... In: Brain : a journal of neurology, vol 143, iss 10 (2020) BASE Show details 18 Utility of the global CDR® plus NACC FTLD rating and development of scoring rules: Data from the ARTFL/LEFFTDS Consortium. Miyagawa, Toji; Brushaber, Danielle; Syrjanen, Jeremy... In: Alzheimer's & dementia : the journal of the Alzheimer's Association, vol 16, iss 1 (2020) BASE Show details 19 Regional and hemispheric susceptibility of the temporal lobe to FTLD-TDP type C pathology. Borghesani, V; Battistella, G; Mandelli, ML. - : eScholarship, University of California, 2020 BASE Show details 20 Use of the CDR® plus NACC FTLD in mild FTLD: Data from the ARTFL/LEFFTDS consortium. Miyagawa, Toji; Brushaber, Danielle; Syrjanen, Jeremy... In: Alzheimer's & dementia : the journal of the Alzheimer's Association, vol 16, iss 1 (2020) BASE Show details Page: 1 2 3

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