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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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Computational Models in Electroencephalography.
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In: Brain topography, vol 35, iss 1 (2022)
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Bilingualism, Dementia, and the Neurological Mechanisms in Between: The Need for a More Critical Look Into Dementia Subtypes ...
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Lee, Yan-Yi. - : Apollo - University of Cambridge Repository, 2022
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CEREBRAL ABSCESS AND MYCOTIC ANEURYSM AS A CONSEQUENCE OF INFECTIVE ENDOCARDITIS: A CASE REPORT ...
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CEREBRAL ABSCESS AND MYCOTIC ANEURYSM AS A CONSEQUENCE OF INFECTIVE ENDOCARDITIS: A CASE REPORT ...
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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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Development of thalamus mediates paternal age effect on offspring reading: A preliminary investigation.
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In: Human brain mapping, vol 42, iss 14 (2021)
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Functional maps of direct electrical stimulation-induced speech arrest and anomia: a multicentre retrospective study.
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In: Brain : a journal of neurology, vol 144, iss 8 (2021)
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Understanding Dyslexia as a Neurological Learning Disability: A Plan for an Instructive Website for Parents and Early Elementary Teachers
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In: Culminating Experience Projects (2021)
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Caregiver burden in patients with behavioural variant frontotemporal dementia and non-fluent variant and semantic variant primary progressive aphasia
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In: J Neural Transm (Vienna) (2021)
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Grieving a disrupted biography: an interpretative phenomenological analysis exploring barriers to the use of mindfulness after neurological injury or impairment
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The language profile in multiple system atrophy: an exploratory study
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In: J Neural Transm (Vienna) (2021)
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University Scholar Series: Pei-Tzu Tsai
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In: University Scholar Series (2021)
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Rehabilitation of unilateral peripheral facial paralysis in Guillain-Barré: a case study
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In: Revista CEFAC, Vol 23, Iss 6 (2021) (2021)
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Associations between cognitive performance and the rehabilitation, medical care and social support provided to French children with Prader-Willi syndrome
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In: ISSN: 1769-7212 ; European Journal of Medical Genetics ; https://hal.umontpellier.fr/hal-03338019 ; European Journal of Medical Genetics, Elsevier, 2020, 63 (12), pp.104064. ⟨10.1016/j.ejmg.2020.104064⟩ (2020)
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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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