Skip to main content
Advertisement
  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Neurology: Neuroimmunology & Neuroinflammation COVID-19 Article Hub
    • 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
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Neurology: Neuroimmunology & Neuroinflammation COVID-19 Article Hub
    • 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
  • Articles
  • Issues
  • COVID-19 Article Hub
  • Infographics & Video Summaries

User menu

  • My Alerts
  • Log in

Search

  • Advanced search
Neurology Neuroimmunology & Neuroinflammation
Home
A peer-reviewed clinical and translational neurology open access journal
  • My Alerts
  • Log in
Site Logo
  • Home
  • Articles
  • Issues
  • COVID-19 Article Hub
  • Infographics & Video Summaries

Share

July 2022; 9 (4) Research ArticleOpen Access

Neurosarcoidosis of the Cauda Equina

Clinical Course, Radiographic and Electrodiagnostic Findings, Response to Treatment, and Outcomes

View ORCID ProfileGabriela Alejandra Bou, View ORCID ProfileRocio Garcia-Santibanez, View ORCID ProfileAlexander Jordan Castilho, View ORCID ProfileSpencer Kristian Hutto
First published April 29, 2022, DOI: https://doi.org/10.1212/NXI.0000000000001170
Gabriela Alejandra Bou
From the Neurology Residency Program (G.A.B.), and Division of Neuromuscular Medicine (R.G.-S.), Department of Neurology, Division of Neuroradiology (A.J.C.), Department of Radiology, and Division of Hospital Neurology (S.K.H.), Department of Neurology, Emory University School of Medicine, Atlanta, GA.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Gabriela Alejandra Bou
  • For correspondence: gabriela.alejandra.bou@emory.edu
Rocio Garcia-Santibanez
From the Neurology Residency Program (G.A.B.), and Division of Neuromuscular Medicine (R.G.-S.), Department of Neurology, Division of Neuroradiology (A.J.C.), Department of Radiology, and Division of Hospital Neurology (S.K.H.), Department of Neurology, Emory University School of Medicine, Atlanta, GA.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Rocio Garcia-Santibanez
  • For correspondence: rocio.c.garcia.santibanez@emory.edu
Alexander Jordan Castilho
From the Neurology Residency Program (G.A.B.), and Division of Neuromuscular Medicine (R.G.-S.), Department of Neurology, Division of Neuroradiology (A.J.C.), Department of Radiology, and Division of Hospital Neurology (S.K.H.), Department of Neurology, Emory University School of Medicine, Atlanta, GA.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Alexander Jordan Castilho
  • For correspondence: alexander.jordan.castilho@emory.edu
Spencer Kristian Hutto
From the Neurology Residency Program (G.A.B.), and Division of Neuromuscular Medicine (R.G.-S.), Department of Neurology, Division of Neuroradiology (A.J.C.), Department of Radiology, and Division of Hospital Neurology (S.K.H.), Department of Neurology, Emory University School of Medicine, Atlanta, GA.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • ORCID record for Spencer Kristian Hutto
Full PDF
Citation
Neurosarcoidosis of the Cauda Equina
Clinical Course, Radiographic and Electrodiagnostic Findings, Response to Treatment, and Outcomes
Gabriela Alejandra Bou, Rocio Garcia-Santibanez, Alexander Jordan Castilho, Spencer Kristian Hutto
Neurol Neuroimmunol Neuroinflamm Jul 2022, 9 (4) e1170; DOI: 10.1212/NXI.0000000000001170

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
1045

Share

  • Article
  • Figures & Data
  • Info & Disclosures
Loading

Abstract

Background and Objectives Sarcoidosis is a multisystem granulomatous disease affecting the nervous system in 3%–5% of cases. It can affect almost any component of the nervous system. Involvement of the cauda equina is an understudied phenotype, and questions remain regarding its natural history and optimal approach to management. This study aims to study the long-term clinical evolution of neurosarcoidosis affecting the cauda equina, response to treatment, and clinical and radiographic outcomes.

Methods Patients with neurosarcoidosis treated at Emory University between January 1, 2011, and December 8, 2021, were retrospectively evaluated for manifestations of cauda equina disease and included if disease of the cauda equina could be substantiated by MRI or EMG.

Results Of 216 cases, 14 (6.5%) involved the cauda equina. The median age was 49.5 years, and most were female (85.7%) and African American (64.3%). Chronic (>28 days) presentations were most common (78.6%), but acute (<7 days, 14.3%) and subacute (7–28 days, 7.1%) were also seen. The median modified Rankin Scale (mRS) score at nadir was 3 (range 2–4). Symptoms were asymmetric in 78.6% and included leg numbness (85.7%), leg weakness (64.3%), perineal numbness (35.7%), pain (42.3%), and incontinence (21.4%). On MRI, the cauda equina enhanced in 100%, appeared nodular in 78.6%, and was diffusely involved in 71.4%. Coexisting myelitis was common (cervical 28.6%, thoracic 35.7%, and conus medullaris 28.6%). Intracranial inflammation included leptomeningitis (71.4%) and cranial neuropathies (57.1%). Electrodiagnostic studies were conducted in 3 with only one showing features consistent with a radicular process. Serum and CSF angiotensin-converting enzyme levels were elevated in 38.5% and 0.0%, respectively. CSF white blood cell and protein were elevated in 92.9%. Corticosteroids were tried in all patients with durable stabilization or improvement in only 3 (21.4%). Second-line agents associated with improvement included methotrexate/infliximab (3/4, 75%), methotrexate (3/4, 75.0%), and azathioprine (1/1, 100%). During a median follow-up of 22.5 months, the final median mRS score was 3. Relapses occurred at a median of 6 months in 21.4%. In 9 patients with MRI follow-up, 6 improved (66.7%), 1 stabilized (11.1%), and 2 worsened (22.2%).

Discussion Characteristic features of cauda equina involvement by neurosarcoidosis include chronically delayed presentations, nodular enhancement on MRI, poor response to corticosteroids, and substantial resultant neurologic disability.

Glossary

ACE=
angiotensin-converting enzyme;
Ig=
immunoglobulin;
mRS=
modified Rankin Scale;
NCS=
nerve conduction study;
OCB=
oligoclonal band

Footnotes

  • Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.

  • The Article Processing Charge was funded by the authors.

  • Submitted and externally peer reviewed. The handling editor was Josep O. Dalmau, MD, PhD, FAAN.

  • Received December 27, 2021.
  • Accepted in final form March 10, 2022.
  • Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

View Full Text

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
    • Appendix Authors
    • Footnotes
    • References
  • Figures & Data
  • Info & Disclosures
Advertisement

Preferences and User Experiences of Wearable Devices in Epilepsy A Systematic Review and Mixed-Methods Synthesis

Dr. Daniel Friedman and Dr. Sharon Chiang

► Watch

Related Articles

  • No related articles found.

Alert Me

  • Alert me when eletters are published
Neurology - Neuroimmunology Neuroinflammation: 10 (2)

Articles

  • Articles
  • Issues
  • Popular Articles

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
  • 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: Neuroimmunology & Neuroinflammation
Online ISSN: 2332-7812

© 2023 American Academy of Neurology

  • Privacy Policy
  • Feedback
  • Advertise