ArticleNeurology2020
Cortical microstructure in the amyotrophic lateral sclerosis-frontotemporal dementia continuum.
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Article in Neurology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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21citing papers in PubMed
1.8field-weighted citation impact, top 11% of its field
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
21 citing papers in PubMed, 39 citations in OpenAlex.
- Methodological insights and clinical outcomes in ALS and FTLD: lessons from the SPIN cohort.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2026Article
- Plasma growth-associated protein 43 correlates with synaptic loss in Alzheimer's disease.Cell reports. Medicine · 2025Article
- Cortical microstructure in familial frontotemporal dementia associated with MAPT, GRN, and C9orf72 pathogenic variants: Looking beyond atrophy.The journal of prevention of Alzheimer's disease · 2025Article
- Cortical microstructure is associated with disease severity and clinical progression in genetic frontotemporal dementia: a GENFI study.Molecular psychiatry · 2025Article
- The Relationship of Prior Concussion and Contact Sport Exposure With Cortical Macro and Microstructure.Human brain mapping · 2025Article
- Cortical macro- and microstructural changes in isolated rapid eye movement sleep behavior disorder.NPJ Parkinson's disease · 2025Article
- The spectrum of behavioral disorders in amyotrophic lateral sclerosis: current view.Journal of neural transmission (Vienna, Austria : 1996) · 2025Review
- Surface-based Analyses of Diffusional Kurtosis Imaging in Amyotrophic Lateral Sclerosis: Relationship with Onset Subtypes.Magnetic resonance in medical sciences : MRMS : an official journal of Japan Society of Magnetic Resonance in Medicine · 2025Article
- Spatial and temporal patterns of cortical mean diffusivity in Alzheimer's disease and suspected non-Alzheimer's disease pathophysiology.Alzheimer's & dementia : the journal of the Alzheimer's Association · 2024Article
- Cortical microstructural abnormalities in amyotrophic lateral sclerosis: a gray matter-based spatial statistics study.Quantitative imaging in medicine and surgery · 2024Article
- Structural and Functional Brain Network Connectivity at Different King's Stages in Patients With Amyotrophic Lateral Sclerosis.Neurology · 2024Observational
- Evidence based on Mendelian randomization and colocalization analysis strengthens causal relationships between structural changes in specific brain regions and risk of amyotrophic lateral sclerosis.Frontiers in neuroscience · 2024Article
- Association of cortical and subcortical microstructure with disease severity: impact on cognitive decline and language impairments in frontotemporal lobar degeneration.Alzheimer's research & therapy · 2023Article
- Low Fluctuation of Symptoms May Delay Diagnosis of Myasthenia Gravis: A Case Series.Neurology and therapy · 2022Article
- Amyotrophic Lateral Sclerosis-Frontotemporal Dementia: Shared and Divergent Neural Correlates Across the Clinical Spectrum.Neurology · 2022Article
- Association of cortical microstructure with amyloid-β and tau: impact on cognitive decline, neurodegeneration, and clinical progression in older adults.Molecular psychiatry · 2021Article
- Regional grey matter microstructural changes and volume loss according to disease duration in multiple sclerosis patients.Scientific reports · 2021Article
- Feature selection from magnetic resonance imaging data in ALS: a systematic review.Therapeutic advances in chronic disease · 2021Review
- Frontotemporal Pathology in Motor Neuron Disease Phenotypes: Insights From Neuroimaging.Frontiers in neurology · 2021Review
- Pathophysiological Underpinnings of Extra-Motor Neurodegeneration in Amyotrophic Lateral Sclerosis: New Insights From Biomarker Studies.Frontiers in neurology · 2021Review
Corrections and comments
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Authors and funding
21 authors at 1 institution in 1 country.
Ignacio Illán-GalaFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain. iillan@santpau.cat jfortea@santpau.cat.ORCID 0000-0002-5418-2052
Victor MontalFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Jordi PeguerolesFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Eduard VilaplanaFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.ORCID 0000-0002-1809-8435
Daniel AlcoleaFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.ORCID 0000-0002-3819-3245
Oriol Dols-IcardoFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Noemi de LunaFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Janina Turón-SansFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Elena Cortés-VicenteFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Luis Martinez-RomanFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Maria Belén Sánchez-SaudinósFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Andrea SubiranaFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Laura VidelaFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Isabel SalaFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Isabel BarroetaFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Sílvia ValldeneuFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Rafael BlesaFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Jordi ClarimónFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Alberto LleóFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.
Juan ForteaFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain. iillan@santpau.cat jfortea@santpau.cat.
Ricard Rojas-GarcíaFrom the Sant Pau Memory Unit (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., L.M.-R., M.B.S.-S., A.S., L.V., I.S., I.B., S.V., R.B., J.C., A.L., J.F.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona; Neuromuscular Diseases Unit (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Raras (CIBERER) (N.d.L., J.T.-S., E.C.-V., R.R.-G.), Valencia; Barcelona Down Medical Center (I.I.G., L.V., J.F.), Fundació Catalana de Síndrome de Down, Barcelona; and Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (I.I.-G., V.M., J.P., E.V., D.A., O.D.-I., I.B., R.B., J.C., A.L., J.F.), CIBERNED, Madrid, Spain.ORCID 0000-0003-1411-5573
Hospital de Sant Pau · ES
Funding
Biological Correlates of Alzheimer in Down Syndrome.R21AG056974 · NIA · UNIVERSITY OF DENVER (COLORADO SEMINARY) · PI GRANHOLM-BENTLEY, ANN-CHARLOTTE ESTHER · 2017 to 2018
$414kNIA NIH HHS R21 AG056974
Abstract
objectiveTo characterize the cortical macrostructure and microstructure of behavioral and cognitive changes along the amyotrophic lateral sclerosis (ALS)-frontotemporal dementia (FTD) continuum.
methodsWe prospectively recruited 88 participants with a 3T MRI structural and diffusion-weighted imaging sequences: 31 with ALS, 20 with the behavioral variant of FTD (bvFTD), and 37 cognitively normal controls. Participants with ALS underwent a comprehensive cognitive and behavioral assessment and were dichotomized into ALS without cognitive or behavioral impairment (ALSno-cbi; n = 12) and ALS with cognitive or behavioral impairment (ALScbi; n = 19). We computed cortical thickness and cortical mean diffusivity using a surface-based approach and explored the cortical correlates of cognitive impairment with the Edinburgh Cognitive and Behavioral ALS Screen.
resultsThe ALSno-cbi and ALScbi groups showed different patterns of reduced cortical thickness and increased cortical mean diffusivity. In the ALSno-cbi group, cortical thinning was restricted mainly to the dorsal motor cortex. In contrast, in the ALScbi group, cortical thinning was observed primarily on frontoinsular and temporal regions bilaterally. There were progressive cortical mean diffusivity changes along the ALSno-cbi, ALScbi, and bvFTD clinical continuum. Participants with ALS with either cognitive or behavioral impairment showed increased cortical mean diffusivity in the prefrontal cortex in the absence of cortical thickness.
conclusionsCortical mean diffusivity might be a useful biomarker for the study of extramotor cortical neurodegeneration in the ALS-FTD clinical spectrum. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that the cortical microstructure correlates with cognitive impairment in the ALS-FTD continuum.
Indexed as
AgedAmyotrophic Lateral SclerosisCerebral CortexCognition DisordersFemaleFrontotemporal DementiaHumansMagnetic Resonance ImagingMaleMiddle AgedNeuroimagingNeuropsychological TestsProblem Behavior
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