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Development of a standard of care for patients with valosin-containing protein associated multisystem proteinopathy.
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In: Orphanet journal of rare diseases, vol 17, iss 1 (2022)
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Family history of FXTAS is associated with age-related cognitive-linguistic decline among mothers with the FMR1 premutation.
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In: Journal of neurodevelopmental disorders, vol 14, iss 1 (2022)
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Cortical microstructure in primary progressive aphasia: a multicenter study.
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Illán-Gala, Ignacio; Montal, Victor; Borrego-Écija, Sergi; Mandelli, Maria Luisa; Falgàs, Neus; Welch, Ariane E; Pegueroles, Jordi; Santos-Santos, Miguel; Bejanin, Alexandre; Alcolea, Daniel; Dols-Icardo, Oriol; Belbin, Olivia; Sánchez-Saudinós, Mª Belén; Bargalló, Nuria; González-Ortiz, Sofía; Lladó, Albert; Blesa, Rafael; Dickerson, Bradford C; Rosen, Howard J; Miller, Bruce L; Lleó, Alberto; Gorno-Tempini, Maria Luisa; Sánchez-Valle, Raquel; Fortea, Juan
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In: Alzheimer's research & therapy, vol 14, iss 1 (2022)
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Abstract:
BackgroundCortical mean diffusivity is a novel imaging metric sensitive to early changes in neurodegenerative syndromes. Higher cortical mean diffusivity values reflect microstructural disorganization and have been proposed as a sensitive biomarker that might antedate macroscopic cortical changes. We aimed to test the hypothesis that cortical mean diffusivity is more sensitive than cortical thickness to detect cortical changes in primary progressive aphasia (PPA).MethodsIn this multicenter, case-control study, we recruited 120 patients with PPA (52 non-fluent, 31 semantic, and 32 logopenic variants; and 5 GRN-related PPA) as well as 89 controls from three centers. The 3-Tesla MRI protocol included structural and diffusion-weighted sequences. Disease severity was assessed with the Clinical Dementia Rating scale. Cortical thickness and cortical mean diffusivity were computed using a surface-based approach.ResultsThe comparison between each PPA variant and controls revealed cortical mean diffusivity increases and cortical thinning in overlapping regions, reflecting the canonical loci of neurodegeneration of each variant. Importantly, cortical mean diffusivity increases also expanded to other PPA-related areas and correlated with disease severity in all PPA groups. Cortical mean diffusivity was also increased in patients with very mild PPA when only minimal cortical thinning was observed and showed a good correlation with measures of disease severity.ConclusionsCortical mean diffusivity shows promise as a sensitive biomarker for the study of the neurodegeneration-related microstructural changes in PPA.
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Keyword:
2.1 Biological and endogenous factors; Acquired Cognitive Impairment; Aging; Alzheimer's disease; Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD); Alzheimer’s disease; Aphasia; Biomedical Imaging; Brain Disorders; Case-Control Studies; Clinical Research; Dementia; Diffusion; Diffusion Magnetic Resonance Imaging; Frontotemporal Dementia (FTD); Frontotemporal lobar degeneration; Humans; Magnetic resonance; Magnetic Resonance Imaging; Medical and Health Sciences; Neurodegenerative; Neurological; Neurosciences; Primary Progressive; Primary progressive aphasia; Rare Diseases
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URL: https://escholarship.org/uc/item/4td346vg
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Social Behavior Observer Checklist: Patterns of Spontaneous Behaviors Differentiate Patients With Neurodegenerative Disease From Healthy Older Adults.
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Resting functional connectivity in the semantic appraisal network predicts accuracy of emotion identification.
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What Do We Mean by Behavioral Disinhibition in Frontotemporal Dementia?
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Comparing two facets of emotion perception across multiple neurodegenerative diseases.
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In: Social cognitive and affective neuroscience, vol 15, iss 5 (2020)
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Amount and delay insensitivity during intertemporal choice in three neurodegenerative diseases reflects dorsomedial prefrontal atrophy.
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Depressive Symptom Profiles Predict Specific Neurodegenerative Disease Syndromes in Early Stages.
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Depressive Symptom Profiles Predict Specific Neurodegenerative Disease Syndromes in Early Stages.
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Deformation-based shape analysis of the hippocampus in the semantic variant of primary progressive aphasia and Alzheimer's disease.
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Regional and hemispheric susceptibility of the temporal lobe to FTLD-TDP type C pathology.
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Amount and delay insensitivity during intertemporal choice in three neurodegenerative diseases reflects dorsomedial prefrontal atrophy.
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A neurophysiological model of speech production deficits in fragile X syndrome.
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In: Brain communications, vol 2, iss 1 (2020)
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Speaker-normalized sound representations in the human auditory cortex.
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In: Nature communications, vol 10, iss 1 (2019)
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Thalamo-cortical network hyperconnectivity in preclinical progranulin mutation carriers.
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Factors that predict diagnostic stability in neurodegenerative dementia.
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In: Journal of neurology, vol 266, iss 8 (2019)
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Relationship Turmoil and Emotional Empathy in Frontotemporal Dementia.
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In: Alzheimer disease and associated disorders, vol 33, iss 3 (2019)
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Divergent patterns of loss of interpersonal warmth in frontotemporal dementia syndromes are predicted by altered intrinsic network connectivity.
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Thalamo-cortical network hyperconnectivity in preclinical progranulin mutation carriers.
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