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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 Hits 1 – 20 of 20 1 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 2 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 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 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 5 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 6 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 7 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 8 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 9 Thalamo-cortical network hyperconnectivity in preclinical progranulin mutation carriers. Lee, Suzee E; Sias, Ana C; Kosik, Eena L. - : eScholarship, University of California, 2019 BASE Show details 10 Factors that predict diagnostic stability in neurodegenerative dementia. Perry, David C; Datta, Samir; Miller, Zachary A... In: Journal of neurology, vol 266, iss 8 (2019) BASE Show details 11 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 12 Cortical developmental abnormalities in logopenic variant primary progressive aphasia with dyslexia. Miller, Zachary A; Spina, Salvatore; Pakvasa, Mikhail... In: Brain communications, vol 1, iss 1 (2019) BASE Show details 13 Physiological, behavioral and subjective sadness reactivity in frontotemporal dementia subtypes. Hua, Alice Y; Chen, Kuan-Hua; Brown, Casey L... In: Social cognitive and affective neuroscience, vol 14, iss 12 (2019) BASE Show details 14 Cerebrospinal fluid biomarkers predict frontotemporal dementia trajectory. Ljubenkov, Peter A; Staffaroni, Adam M; Rojas, Julio C... In: Annals of clinical and translational neurology, vol 5, iss 10 (2018) BASE Show details 15 Rates of Amyloid Imaging Positivity in Patients With Primary Progressive Aphasia. Santos-Santos, Miguel A; Rabinovici, Gil D; Iaccarino, Leonardo... In: JAMA neurology, vol 75, iss 3 (2018) BASE Show details 16 Longitudinal white matter change in frontotemporal dementia subtypes and sporadic late onset Alzheimer's disease. Elahi, Fanny M; Marx, Gabe; Cobigo, Yann. - : eScholarship, University of California, 2017 BASE Show details 17 Longitudinal white matter change in frontotemporal dementia subtypes and sporadic late onset Alzheimer's disease. Elahi, Fanny M; Marx, Gabe; Cobigo, Yann. - : eScholarship, University of California, 2017 BASE Show details 18 Increased prevalence of autoimmune disease within C9 and FTD/MND cohorts: Completing the picture. Miller, Zachary A; Sturm, Virginia E; Camsari, Gamze Balci... In: Neurology(R) neuroimmunology & neuroinflammation, vol 3, iss 6 (2016) BASE Show details 19 Atrophy patterns in early clinical stages across distinct phenotypes of Alzheimer's disease. Ossenkoppele, Rik; Cohn-Sheehy, Brendan I; La Joie, Renaud... In: Human brain mapping, vol 36, iss 11 (2015) BASE Show details 20 Neural substrates of socioemotional self-awareness in neurodegenerative disease. Sollberger, Marc; Rosen, Howard J; Shany-Ur, Tal... In: Brain and behavior, vol 4, iss 2 (2014) BASE Show details

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