Skip to main content
Advertisement
  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Inclusion, Diversity, Equity, Anti-racism, & Social Justice (IDEAS)
    • Innovations in Care Delivery
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • COVID-19
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Neurology Future Forecasting Series
    • Null Hypothesis
    • Patient Pages
    • Topics A-Z
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit a Manuscript
    • Author Center

Advanced Search

Main menu

  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Inclusion, Diversity, Equity, Anti-racism, & Social Justice (IDEAS)
    • Innovations in Care Delivery
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • COVID-19
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Neurology Future Forecasting Series
    • Null Hypothesis
    • Patient Pages
    • Topics A-Z
    • Translations
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit a Manuscript
    • Author Center
  • Home
  • Latest Articles
  • Current Issue
  • Past Issues
  • Neurology Video Journal Club
  • Residents & Fellows

User menu

  • Subscribe
  • My Alerts
  • Log in

Search

  • Advanced search
Neurology
Home
The most widely read and highly cited peer-reviewed neurology journal
  • Subscribe
  • My Alerts
  • Log in
Site Logo
  • Home
  • Latest Articles
  • Current Issue
  • Past Issues
  • Neurology Video Journal Club
  • Residents & Fellows

Share

November 03, 2020; 95 (18) Article

Cortical microstructure in the amyotrophic lateral sclerosis–frontotemporal dementia continuum

View ORCID ProfileIgnacio Illán-Gala, Victor Montal, Jordi Pegueroles, View ORCID ProfileEduard Vilaplana, View ORCID ProfileDaniel Alcolea, Oriol Dols-Icardo, Noemi de Luna, Janina Turón-Sans, Elena Cortés-Vicente, Luis Martinez-Roman, Maria Belén Sánchez-Saudinós, Andrea Subirana, Laura Videla, Isabel Sala, Isabel Barroeta, Sílvia Valldeneu, Rafael Blesa, Jordi Clarimón, Alberto Lleó, Juan Fortea, View ORCID ProfileRicard Rojas-García
First published September 10, 2020, DOI: https://doi.org/10.1212/WNL.0000000000010727
Ignacio Illán-Gala
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Ignacio Illán-Gala
Victor Montal
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Jordi Pegueroles
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Eduard Vilaplana
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Eduard Vilaplana
Daniel Alcolea
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Daniel Alcolea
Oriol Dols-Icardo
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Noemi de Luna
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Janina Turón-Sans
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Elena Cortés-Vicente
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Luis Martinez-Roman
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Maria Belén Sánchez-Saudinós
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Andrea Subirana
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Laura Videla
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Isabel Sala
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Isabel Barroeta
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Sílvia Valldeneu
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Rafael Blesa
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Jordi Clarimón
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Alberto Lleó
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Juan Fortea
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Ricard Rojas-García
From 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.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Ricard Rojas-García
Full PDF
Short Form
Citation
Cortical microstructure in the amyotrophic lateral sclerosis–frontotemporal dementia continuum
Ignacio Illán-Gala, Victor Montal, Jordi Pegueroles, Eduard Vilaplana, Daniel Alcolea, Oriol Dols-Icardo, Noemi de Luna, Janina Turón-Sans, Elena Cortés-Vicente, Luis Martinez-Roman, Maria Belén Sánchez-Saudinós, Andrea Subirana, Laura Videla, Isabel Sala, Isabel Barroeta, Sílvia Valldeneu, Rafael Blesa, Jordi Clarimón, Alberto Lleó, Juan Fortea, Ricard Rojas-García
Neurology Nov 2020, 95 (18) e2565-e2576; DOI: 10.1212/WNL.0000000000010727

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero
Permissions

Make Comment

See Comments

Downloads
132

Share

  • Article
  • Figures & Data
  • Info & Disclosures
Loading

This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.

Abstract

Objective To characterize the cortical macrostructure and microstructure of behavioral and cognitive changes along the amyotrophic lateral sclerosis (ALS)–frontotemporal dementia (FTD) continuum.

Methods We 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.

Results The 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.

Conclusions Cortical 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.

Glossary

ALS=
amyotrophic lateral sclerosis;
ALScbi=
ALS with cognitive or behavioral impairment;
ALSno-cbi=
ALS without cognitive or behavioral impairment;
bvFTD=
behavioral variant of FTD;
ECAS=
Edinburgh Cognitive and Behavioral ALS Screen;
FTD=
frontotemporal dementia;
FTLD=
frontotemporal lobar degeneration;
TDP-43=
TAR DNA-binding protein 43

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • ↵* These authors contributed equally to this work as first authors.

  • ↵† These authors contributed equally to this work as senior authors.

  • Class of Evidence: NPub.org/coe

  • Received January 14, 2020.
  • Accepted in final form June 4, 2020.
  • © 2020 American Academy of Neurology
View Full Text

AAN Members

We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.

Google Safari Microsoft Edge Firefox

Click here to login

AAN Non-Member Subscribers

Click here to login

Purchase access

For assistance, please contact:
AAN Members (800) 879-1960 or (612) 928-6000 (International)
Non-AAN Member subscribers (800) 638-3030 or (301) 223-2300 option 3, select 1 (international)

Sign Up
Information on how to subscribe to Neurology and Neurology: Clinical Practice can be found here 

Purchase
Individual access to articles is available through the Add to Cart option on the article page.  Access for 1 day (from the computer you are currently using) is US$ 39.00.  Pay-per-view content is for the use of the payee only, and content may not be further distributed by print or electronic means.  The payee may view, download, and/or print the article for his/her personal, scholarly, research, and educational use.  Distributing copies (electronic or otherwise) of the article is not allowed.

Letters: Rapid online correspondence

No comments have been published for this article.
Comment

REQUIREMENTS

If you are uploading a letter concerning an article:
You must have updated your disclosures within six months: http://submit.neurology.org

Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.

If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.

Submission specifications:

  • Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
  • Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
  • Submit only on articles published within 6 months of issue date.
  • Do not be redundant. Read any comments already posted on the article prior to submission.
  • Submitted comments are subject to editing and editor review prior to posting.

More guidelines and information on Disputes & Debates

Compose Comment

More information about text formats

Plain text

  • No HTML tags allowed.
  • Web page addresses and e-mail addresses turn into links automatically.
  • Lines and paragraphs break automatically.
Author Information
NOTE: The first author must also be the corresponding author of the comment.
First or given name, e.g. 'Peter'.
Your last, or family, name, e.g. 'MacMoody'.
Your email address, e.g. higgs-boson@gmail.com
Your role and/or occupation, e.g. 'Orthopedic Surgeon'.
Your organization or institution (if applicable), e.g. 'Royal Free Hospital'.
Publishing Agreement
NOTE: All authors, besides the first/corresponding author, must complete a separate Publishing Agreement Form and provide via email to the editorial office before comments can be posted.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.

Vertical Tabs

You May Also be Interested in

Back to top
  • Article
    • Abstract
    • Glossary
    • Methods
    • Results
    • Discussion
    • Study funding
    • Disclosure
    • Acknowledgment
    • Appendix Authors
    • Footnotes
    • References
  • Figures & Data
  • Info & Disclosures
Advertisement

Hemiplegic Migraine Associated With PRRT2 Variations A Clinical and Genetic Study

Dr. Robert Shapiro and Dr. Amynah Pradhan

► Watch

Related Articles

  • No related articles found.

Topics Discussed

  • Amyotrophic lateral sclerosis
  • MRI
  • Frontotemporal dementia
  • Cognitive neuropsychology in dementia

Alert Me

  • Alert me when eletters are published
Neurology: 100 (5)

Articles

  • Ahead of Print
  • Current Issue
  • Past Issues
  • Popular Articles
  • Translations

About

  • About the Journals
  • Ethics Policies
  • Editors & Editorial Board
  • Contact Us
  • Advertise

Submit

  • Author Center
  • Submit a Manuscript
  • Information for Reviewers
  • AAN Guidelines
  • Permissions

Subscribers

  • Subscribe
  • Activate a Subscription
  • Sign up for eAlerts
  • RSS Feed
Site Logo
  • Visit neurology Template on Facebook
  • Follow neurology Template on Twitter
  • Visit Neurology on YouTube
  • Neurology
  • Neurology: Clinical Practice
  • Neurology: Education
  • Neurology: Genetics
  • Neurology: Neuroimmunology & Neuroinflammation
  • AAN.com
  • AANnews
  • Continuum
  • Brain & Life
  • Neurology Today

Wolters Kluwer Logo

Neurology | Print ISSN:0028-3878
Online ISSN:1526-632X

© 2023 American Academy of Neurology

  • Privacy Policy
  • Feedback
  • Advertise