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1
Development of a standard of care for patients with valosin-containing protein associated multisystem proteinopathy.
In: Orphanet journal of rare diseases, vol 17, iss 1 (2022)
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2
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)
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3
Cortical microstructure in primary progressive aphasia: a multicenter study.
In: Alzheimer's research & therapy, vol 14, iss 1 (2022)
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4
Rare Disorders: Diagnosis and Therapeutic Planning for Patients Seeking Orthodontic Treatment
In: Journal of Clinical Medicine; Volume 11; Issue 6; Pages: 1527 (2022)
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5
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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6
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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7
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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8
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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9
B Cell-Restricted Depletion of Dnmt3a Activates Notch Signaling and Causes Chronic Lymphocytic Leukemia
In: Blood, vol 138, iss Supplement 1 (2021)
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10
Concurrent Associations between Expressive Language Ability and Independence in Adolescents and Adults with Fragile X Syndrome.
In: Brain sciences, vol 11, iss 9 (2021)
Abstract: BackgroundFew individuals with fragile X syndrome (FXS) successfully meet adult normative expectations in education, employment, peer relations, and habitation, although there is within-syndrome variability in this regard. The primary goal of this study was to determine whether expressive language skills contribute to the capacity for independent functioning in adulthood even after controlling for nonverbal cognitive ability.MethodsParticipants were 18- to 23-year-olds with FXS. Expressive language was assessed using the psychometrically validated Expressive Language Sampling (ELS) conversation and narration procedures. The language produced was transcribed and analyzed to yield measures of expressive vocabulary, syntax, and intelligibility. Parents concurrently completed questionnaires on the independent functioning of the participants with FXS.ResultsAll three ELS measures were significantly corelated with multiple measures of independence. The magnitudes of the correlations were reduced when nonverbal IQ was controlled through partial correlation. Nonetheless, many of the partial correlations were medium to large and several were statistically significant.ConclusionsExpressive language skills appear to contribute uniquely to the capacity for independence, although longitudinal data are needed to evaluate the possibility of a bidirectional relationship between these domains. Thus, language intervention may be a prerequisite for preparing youth with FXS for an independent adult life.
Keyword: Behavioral and Social Science; Brain Disorders; Clinical Research; Cognitive Sciences; daily living skills; expressive language; fragile X syndrome; independent functioning; Intellectual and Developmental Disabilities (IDD); Mental Health; Neurosciences; Pediatric; Psychology; Rare Diseases
URL: https://escholarship.org/uc/item/4g58z81c
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11
Concurrent Associations between Expressive Language Ability and Independence in Adolescents and Adults with Fragile X Syndrome.
In: Brain sciences, vol 11, iss 9 (2021)
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12
Expressive language development in adolescents with Down syndrome and fragile X syndrome: change over time and the role of family-related factors.
In: Journal of neurodevelopmental disorders, vol 12, iss 1 (2020)
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13
New directions in clinical trials for frontotemporal lobar degeneration: Methods and outcome measures.
In: Alzheimer's & dementia : the journal of the Alzheimer's Association, vol 16, iss 1 (2020)
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14
Comparing two facets of emotion perception across multiple neurodegenerative diseases.
In: Social cognitive and affective neuroscience, vol 15, iss 5 (2020)
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15
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
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16
Use of the CDR® plus NACC FTLD in mild FTLD: Data from the ARTFL/LEFFTDS consortium.
In: Alzheimer's & dementia : the journal of the Alzheimer's Association, vol 16, iss 1 (2020)
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17
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
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18
New directions in clinical trials for frontotemporal lobar degeneration: Methods and outcome measures.
In: Alzheimer's & dementia : the journal of the Alzheimer's Association, vol 16, iss 1 (2020)
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19
Graded, multidimensional intra- and intergroup variations in primary progressive aphasia and post-stroke aphasia.
In: Brain : a journal of neurology, vol 143, iss 10 (2020)
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20
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
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