Skip to main content
Advertisement
  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Genetics
    • Neuroimmunology & Neuroinflammation
    • Education
  • 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
    • 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
    • Genetics
    • Neuroimmunology & Neuroinflammation
    • Education
  • 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
    • 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
  • 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
  • Residents & Fellows

Share

March 22, 2022; 98 (12) NeuroImage

Primary Lateral Sclerosis Presenting With Focal Onset Spreading Through Contiguous Neuroanatomic Regions

View ORCID ProfileParide Schito, View ORCID ProfileEdoardo Gioele Spinelli, Antonio Malvaso, View ORCID ProfileTommaso Russo, View ORCID ProfileYuri Matteo Falzone, View ORCID ProfileFederica Agosta, View ORCID ProfileAngelo Quattrini, View ORCID ProfileMassimo Filippi, View ORCID ProfileNilo Riva
First published February 7, 2022, DOI: https://doi.org/10.1212/WNL.0000000000200011
Paride Schito
From the Neurology Unit, Neuroimaging Research Unit (P.S., T.R., Y.M.F., F.A., M.F., N.R.), and Experimental Neuropathology Unit (A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience (E.G.S., A.M., F.A., M.F.), Neurorehabilitation Unit (M.F., N.R.), and Neurophysiology Service (M.F.), IRCCS San Raffaele Scientific Institute; and Vita-Salute San Raffaele University (F.A., M.F.); Milan, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Paride Schito
Edoardo Gioele Spinelli
From the Neurology Unit, Neuroimaging Research Unit (P.S., T.R., Y.M.F., F.A., M.F., N.R.), and Experimental Neuropathology Unit (A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience (E.G.S., A.M., F.A., M.F.), Neurorehabilitation Unit (M.F., N.R.), and Neurophysiology Service (M.F.), IRCCS San Raffaele Scientific Institute; and Vita-Salute San Raffaele University (F.A., M.F.); Milan, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Edoardo Gioele Spinelli
Antonio Malvaso
From the Neurology Unit, Neuroimaging Research Unit (P.S., T.R., Y.M.F., F.A., M.F., N.R.), and Experimental Neuropathology Unit (A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience (E.G.S., A.M., F.A., M.F.), Neurorehabilitation Unit (M.F., N.R.), and Neurophysiology Service (M.F.), IRCCS San Raffaele Scientific Institute; and Vita-Salute San Raffaele University (F.A., M.F.); Milan, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Tommaso Russo
From the Neurology Unit, Neuroimaging Research Unit (P.S., T.R., Y.M.F., F.A., M.F., N.R.), and Experimental Neuropathology Unit (A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience (E.G.S., A.M., F.A., M.F.), Neurorehabilitation Unit (M.F., N.R.), and Neurophysiology Service (M.F.), IRCCS San Raffaele Scientific Institute; and Vita-Salute San Raffaele University (F.A., M.F.); Milan, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Tommaso Russo
Yuri Matteo Falzone
From the Neurology Unit, Neuroimaging Research Unit (P.S., T.R., Y.M.F., F.A., M.F., N.R.), and Experimental Neuropathology Unit (A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience (E.G.S., A.M., F.A., M.F.), Neurorehabilitation Unit (M.F., N.R.), and Neurophysiology Service (M.F.), IRCCS San Raffaele Scientific Institute; and Vita-Salute San Raffaele University (F.A., M.F.); Milan, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Yuri Matteo Falzone
Federica Agosta
From the Neurology Unit, Neuroimaging Research Unit (P.S., T.R., Y.M.F., F.A., M.F., N.R.), and Experimental Neuropathology Unit (A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience (E.G.S., A.M., F.A., M.F.), Neurorehabilitation Unit (M.F., N.R.), and Neurophysiology Service (M.F.), IRCCS San Raffaele Scientific Institute; and Vita-Salute San Raffaele University (F.A., M.F.); Milan, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Federica Agosta
Angelo Quattrini
From the Neurology Unit, Neuroimaging Research Unit (P.S., T.R., Y.M.F., F.A., M.F., N.R.), and Experimental Neuropathology Unit (A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience (E.G.S., A.M., F.A., M.F.), Neurorehabilitation Unit (M.F., N.R.), and Neurophysiology Service (M.F.), IRCCS San Raffaele Scientific Institute; and Vita-Salute San Raffaele University (F.A., M.F.); Milan, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Angelo Quattrini
Massimo Filippi
From the Neurology Unit, Neuroimaging Research Unit (P.S., T.R., Y.M.F., F.A., M.F., N.R.), and Experimental Neuropathology Unit (A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience (E.G.S., A.M., F.A., M.F.), Neurorehabilitation Unit (M.F., N.R.), and Neurophysiology Service (M.F.), IRCCS San Raffaele Scientific Institute; and Vita-Salute San Raffaele University (F.A., M.F.); Milan, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Massimo Filippi
Nilo Riva
From the Neurology Unit, Neuroimaging Research Unit (P.S., T.R., Y.M.F., F.A., M.F., N.R.), and Experimental Neuropathology Unit (A.Q., N.R.), Institute of Experimental Neurology (INSPE), Division of Neuroscience (E.G.S., A.M., F.A., M.F.), Neurorehabilitation Unit (M.F., N.R.), and Neurophysiology Service (M.F.), IRCCS San Raffaele Scientific Institute; and Vita-Salute San Raffaele University (F.A., M.F.); Milan, Italy.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Nilo Riva
Full PDF
Citation
Primary Lateral Sclerosis Presenting With Focal Onset Spreading Through Contiguous Neuroanatomic Regions
Paride Schito, Edoardo Gioele Spinelli, Antonio Malvaso, Tommaso Russo, Yuri Matteo Falzone, Federica Agosta, Angelo Quattrini, Massimo Filippi, Nilo Riva
Neurology Mar 2022, 98 (12) 503-504; DOI: 10.1212/WNL.0000000000200011

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
281

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.

A 66-year-old woman complained of a 3-year history of left ankle focal spasticity. Brain MRI revealed subcortical white matter hyperintensity in the medial region of the right precentral gyrus. Subsequently, motor impairment spread proximally, to the contralateral lower limb and then to ipsilateral arm, ultimately resulting in a progressive asymmetric tetraparesis and a mild dysarthria. After a 4-year follow-up, a diagnosis of definite primary lateral sclerosis was established.1 Clinical progression was mirrored by corresponding spreading of the MRI hyperintensity through motor areas (Figure, A) and extensive microstructural damage of the corticospinal tract shown by diffusion tensor MRI tractography (Figure, B), consistently with a model of TDP-43 protein prion-like transmission through the complex 3D anatomy of the motor system.2

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.

  • © 2022 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.

Disputes & Debates: 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
    • Study Funding
    • Disclosure
    • Acknowledgment
    • Appendix Authors
    • Footnotes
    • References
  • Figures & Data
  • Info & Disclosures
Advertisement

Related Articles

  • No related articles found.

Alert Me

  • Alert me when eletters are published
Neurology: 99 (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: Genetics
  • Neurology: Neuroimmunology & Neuroinflammation
  • Neurology: Education
  • AAN.com
  • AANnews
  • Continuum
  • Brain & Life
  • Neurology Today

Wolters Kluwer Logo

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

© 2022 American Academy of Neurology

  • Privacy Policy
  • Feedback
  • Advertise