| 41 |
The Relationship between Expressive Language Sampling and Clinical Measures in Fragile X Syndrome and Typical Development.
|
|
|
|
In: Brain sciences, vol 10, iss 2 (2020)
|
|
BASE
|
|
Show details
|
|
|
| 42 |
Artificial intelligence approaches to predicting and detecting cognitive decline in older adults: A conceptual review.
|
|
|
|
BASE
|
|
Show details
|
|
|
| 43 |
Thalamo-cortical network hyperconnectivity in preclinical progranulin mutation carriers.
|
|
Lee, Suzee E; Sias, Ana C; Kosik, Eena L; Flagan, Taru M; Deng, Jersey; Chu, Stephanie A; Brown, Jesse A; Vidovszky, Anna A; Ramos, Eliana Marisa; Gorno-Tempini, Maria Luisa; Karydas, Anna M; Coppola, Giovanni; Geschwind, Daniel H; Rademakers, Rosa; Boeve, Bradley F; Boxer, Adam L; Rosen, Howard J; Miller, Bruce L; Seeley, William W. - : eScholarship, University of California, 2019
|
|
Abstract:
Mutations in progranulin (GRN) cause heterogeneous clinical syndromes, including behavioral variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), corticobasal syndrome (CBS) and Alzheimer-type dementia (AD-type dementia). Human studies have shown that presymptomatic GRN carriers feature reduced connectivity in the salience network, a system targeted in bvFTD. Mice with homozygous deletion of GRN, in contrast, show thalamo-cortical hypersynchrony due to aberrant pruning of inhibitory synapses onto thalamo-cortical projection neurons. No studies have systematically explored the intrinsic connectivity networks (ICNs) targeted by the four GRN-associated clinical syndromes, or have forged clear links between human and mouse model findings. We compared 17 preclinical GRN carriers (14 "presymptomatic" clinically normal and three "prodromal" with mild cognitive symptoms) to healthy controls to assess for differences in cognitive testing and gray matter volume. Using task-free fMRI, we assessed connectivity in the salience network, a non-fluent variant primary progressive aphasia network (nfvPPA), the perirolandic network (CBS), and the default mode network (AD-type dementia). GRN carriers and controls showed similar performance on cognitive testing. Although carriers showed little evidence of brain atrophy, markedly enhanced connectivity emerged in all four networks, and thalamo-cortical hyperconnectivity stood out as a unifying feature. Voxelwise assessment of whole brain degree centrality, an unbiased graph theoretical connectivity metric, confirmed thalamic hyperconnectivity. These results show that human GRN disease and the prevailing GRN mouse model share a thalamo-cortical network hypersynchrony phenotype. Longitudinal studies will determine whether this network physiology represents a compensatory response as carriers approach symptom onset, or an early and sustained preclinical manifestation of lifelong progranulin haploinsufficiency.
|
|
Keyword:
2.1 Biological and endogenous factors; Acquired Cognitive Impairment; Adult; Aged; Aging; Alzheimer's Disease; Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD); Alzheimer's Disease Related Dementias (ADRD); Behavioral and Social Science; Brain Disorders; Cerebral Cortex; Clinical Research; Cognitive Dysfunction; Connectome; Dementia; Female; Frontotemporal Dementia; Frontotemporal Dementia (FTD); GRN; Heterozygote; Humans; Magnetic Resonance Imaging; Male; Middle Aged; MRI; Nerve Net; Neurodegenerative; Neurological; Neurosciences; Prodromal Symptoms; Progranulin; Progranulins; Rare Diseases; Thalamus
|
|
URL: https://escholarship.org/uc/item/6vv8g8qt
|
|
BASE
|
|
Hide details
|
|
|
| 44 |
Physiological, behavioral and subjective sadness reactivity in frontotemporal dementia subtypes.
|
|
|
|
In: Social cognitive and affective neuroscience, vol 14, iss 12 (2019)
|
|
BASE
|
|
Show details
|
|
|
| 45 |
Factors that predict diagnostic stability in neurodegenerative dementia.
|
|
|
|
In: Journal of neurology, vol 266, iss 8 (2019)
|
|
BASE
|
|
Show details
|
|
|
| 46 |
Relationship Turmoil and Emotional Empathy in Frontotemporal Dementia.
|
|
|
|
In: Alzheimer disease and associated disorders, vol 33, iss 3 (2019)
|
|
BASE
|
|
Show details
|
|
|
| 47 |
Divergent patterns of loss of interpersonal warmth in frontotemporal dementia syndromes are predicted by altered intrinsic network connectivity.
|
|
|
|
BASE
|
|
Show details
|
|
|
| 48 |
Thalamo-cortical network hyperconnectivity in preclinical progranulin mutation carriers.
|
|
|
|
BASE
|
|
Show details
|
|
|
| 49 |
Cortical developmental abnormalities in logopenic variant primary progressive aphasia with dyslexia.
|
|
|
|
In: Brain communications, vol 1, iss 1 (2019)
|
|
BASE
|
|
Show details
|
|
|
| 50 |
Divergent patterns of loss of interpersonal warmth in frontotemporal dementia syndromes are predicted by altered intrinsic network connectivity.
|
|
|
|
BASE
|
|
Show details
|
|
|
| 51 |
Physiological, behavioral and subjective sadness reactivity in frontotemporal dementia subtypes.
|
|
|
|
In: Social cognitive and affective neuroscience, vol 14, iss 12 (2019)
|
|
BASE
|
|
Show details
|
|
|
| 53 |
Neural correlates of abnormal auditory feedback processing during speech production in Alzheimer's disease.
|
|
|
|
In: Scientific reports, vol 9, iss 1 (2019)
|
|
BASE
|
|
Show details
|
|
|
| 54 |
Neural correlates of abnormal auditory feedback processing during speech production in Alzheimer's disease.
|
|
|
|
In: Scientific reports, vol 9, iss 1 (2019)
|
|
BASE
|
|
Show details
|
|
|
| 55 |
Relationships Between Domain-Specific Cognition and Regional Brain Structure in a Typically Developing Cohort
|
|
|
|
BASE
|
|
Show details
|
|
|
| 56 |
Linguistic and Cultural Acceptability of a Spanish Translation of the Ohio State University Traumatic Brain Injury Identification Method Among Community-Dwelling Spanish-Dominant Older Adults.
|
|
|
|
In: Archives of rehabilitation research and clinical translation, vol 1, iss 3-4 (2019)
|
|
BASE
|
|
Show details
|
|
|
| 57 |
Muggle or magical?: Electrophysiological investigations of variation in the language-knowledge interface during reading
|
|
|
|
BASE
|
|
Show details
|
|
|
| 58 |
A Randomized Controlled Trial of Sertraline in Young Children With Autism Spectrum Disorder.
|
|
|
|
BASE
|
|
Show details
|
|
|
| 59 |
Pathogenic variants in USP7 cause a neurodevelopmental disorder with speech delays, altered behavior, and neurologic anomalies.
|
|
|
|
In: Genetics in medicine : official journal of the American College of Medical Genetics, vol 21, iss 8 (2019)
|
|
BASE
|
|
Show details
|
|
|
| 60 |
A Randomized Controlled Trial of Sertraline in Young Children With Autism Spectrum Disorder.
|
|
|
|
BASE
|
|
Show details
|
|
|
|
|
