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

June 2015; 2 (3) Clinical/Scientific NotesOpen Access

Aquaporin-4–positive myelitis associated with Sjögren syndrome and colonic adenocarcinoma

Thomas Rossel, Anastasia Zekeridou, Francois Ochsner, Susanne Renaud
First published April 9, 2015, DOI: https://doi.org/10.1212/NXI.0000000000000103
Thomas Rossel
From the Division of Neurology (T.R., A.Z., S.R.), Pourtalès Hospital, Neuchatel, Switzerland; and Department of Clinical Neurosciences (F.O.), University Hospital of Lausanne, Switzerland.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Anastasia Zekeridou
From the Division of Neurology (T.R., A.Z., S.R.), Pourtalès Hospital, Neuchatel, Switzerland; and Department of Clinical Neurosciences (F.O.), University Hospital of Lausanne, Switzerland.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Francois Ochsner
From the Division of Neurology (T.R., A.Z., S.R.), Pourtalès Hospital, Neuchatel, Switzerland; and Department of Clinical Neurosciences (F.O.), University Hospital of Lausanne, Switzerland.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Susanne Renaud
From the Division of Neurology (T.R., A.Z., S.R.), Pourtalès Hospital, Neuchatel, Switzerland; and Department of Clinical Neurosciences (F.O.), University Hospital of Lausanne, Switzerland.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Full PDF
Citation
Aquaporin-4–positive myelitis associated with Sjögren syndrome and colonic adenocarcinoma
Thomas Rossel, Anastasia Zekeridou, Francois Ochsner, Susanne Renaud
Neurol Neuroimmunol Neuroinflamm Jun 2015, 2 (3) e103; DOI: 10.1212/NXI.0000000000000103

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
519

Share

  • Article
  • Figures & Data
  • Info & Disclosures
Loading

Neuromyelitis optica (NMO) is an inflammatory disorder of the CNS with prominent involvement of the optic nerve and the spinal cord. An autoantibody against aquaporin-4 (AQP4), a water channel abundant on astrocytic foot processes, is the biomarker of the disease.1

The term neuromyelitis optica spectrum disorders (NMOSD) includes AQP4-IgG-positive syndromes that may only partially fulfill contemporary criteria for diagnosis of NMO.2

NMOSD are frequently associated with other autoimmune disorders and there are increasing reports of NMOSD in patients with cancer.3,4

Case report.

We report a case of an 80-year-old nonsmoking Caucasian woman with a diagnosis of primary Sjögren syndrome 1.5 years prior to presentation (seropositive for SSA [Ro60 and Ro52 > 100 U/mL]; SSB-negative) confirmed by salivary gland biopsy and a positive Schirmer test. The diagnosis was established during workup for a sensory neuropathy; peroneal nerve biopsy showed involvement of both myelin and axons with perivascular T-lymphocytic infiltration (figure). She was treated with rituximab with an excellent clinical improvement.

Figure
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure Nerve biopsy findings and spinal MRI

(A–C) Superficial peroneal nerve biopsy (hematoxylin & eosin stain). (A) Fibrous nerve fascicle without myelin reflecting myelino-axonal involvement (green circle) associated with slight vasculitic infiltrate (red circle) without arteriole lesion (blue circle). (B, C) Predominance of T-lymphocyte infiltration (CD3 staining, B) in comparison to B lymphocytes (CD20 staining, C). (D–F) Spinal MRI with predominantly central longitudinally transverse myelitis from C1 to T10 (T2-weighted image, D) with heterogeneous gadolinium enhancement (T1-weighted images, E and F).

The patient presented to the emergency department with sudden loss of strength in her left lower limb. The cerebral CT scan showed chronic microvascular leukoencephalopathy and she was admitted for suspicion of anterior cerebral artery stroke. The next day, symmetric paraparesis and bilateral hyperreflexia developed, with a T4 sensory level and loss of voluntary sphincter control. The spinal MRI showed a longitudinally extensive transverse myelitis with diffuse inhomogeneous gadolinium enhancement (figure). The CSF showed pleocytosis with monocytic predominance (387 cells/mm3), no atypical cells, slightly low glucose (40% of the serum), and elevated protein (2,989 mg/L) without any oligoclonal bands. CSF culture and viral PCR (herpes simplex virus 1 and 2) were negative. The cerebral MRI showed chronic vascular leukoencephalopathy lesions. Serologies for Lyme disease, syphilis, HIV, and tuberculosis were negative. Detected non-organ-specific autoantibodies were SSA (Ro 52 > 240 U/mL and Ro 60 > 108 U/mL; normal < 5 U/mL). Neural autoantibodies were not detected (specifically acetylcholine receptor muscle and ganglionic type, voltage-gated calcium channel P/Q-type and N-type, GAD65, voltage-gated potassium channel, alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor, γ-aminobutyric acid B receptor, NMDA receptor, collapsin response-mediator protein 5, amphiphysin, anti-neuronal nuclear antibody 1 and 2, Purkinje cell cytoplasmic antibody type 1, and Ma1/Ma2) except for AQP4-IgG that was positive at 1:1,000 serum dilution by cell-binding assay (commercially available, M23 isoform). A serum specimen collected a year earlier that was retrospectively tested was also positive for AQP4-IgG at a lower dilution (1:500).

The patient was treated with high-dose methylprednisolone followed by oral tapering with prednisone. Plasma exchange was initiated because the patient developed tetraplegia; the patient's motor skills in lower extremities subsequently improved rapidly.

Due to the patient's age and history of diverticulitis in the previous year, total-body CT was performed in search of a tumor and revealed a perforated sigmoid mass.

The patient underwent exploratory laparotomy and left hemicolectomy. Histopathology confirmed a locally invasive colonic adenocarcinoma (pT4aN1aG2L1) without evidence of systemic metastasis. The patient died 2 days later from hemorrhagic shock. The family denied permission for autopsy.

Discussion.

This is a case of an elderly woman presenting with AQP4-IgG-seropositive transverse myelitis in the context of primary Sjögren syndrome with sensory neuropathy and colonic adenocarcinoma.

In a cohort of 2,743 AQP4-IgG-seropositive patients with NMOSD, only 12.1% were older than 65 years.5 However, the clinical presentation with longitudinally extensive transverse myelitis at disease onset, AQP4-IgG seropositivity, and severe neurologic impairment is similar to presentations reported in elderly women.5

AQP4 autoimmunity is strongly associated with Sjögren syndrome, representing the coexistence of 2 different disorders rather than NMOSD as a symptom of the systemic autoimmune disorder.3

This patient was positive for AQP4-IgG a year prior to the presentation of transverse myelitis, without any signs of an NMO-type clinical presentation. The existence of AQP4-IgG before NMO onset has been described in 4 patients with coexisting myasthenia gravis; higher AQP4-IgG levels were noticed at NMO clinical presentation, as in this patient.6

Cancer coexistence with NMOSD was 5% in large cohorts but up to 15% in cohorts with older patients. Breast, thymus, lung, nasopharynx, cervix, bladder, gastrointestinal tract, thyroid, pituitary gland, ovary, prostate, skin neoplasms, carcinoid tumors, and hematologic cancers are reported.4,7 AQP4 is not expressed in colonic tissue, but to our knowledge colonic adenocarcinomas have not been studied for AQP4 expression at the RNA or the protein level. As this patient's tumor was not tested for AQP4 expression, we can only speculate that the presence of AQP4-IgG a year prior to disease onset could be a marker of the underlying oncologic disease. A simple coexistence of the cancer and the NMOSD is also possible.

Acknowledgments

Acknowledgment: The authors thank Prof. Vanda Lennon, MD, PhD, Mayo Clinic, Rochester, MN, for revising the manuscript.

Footnotes

  • Author contributions: Thomas Rossel: concept and design of the paper, acquisition of data, analysis and interpretation, and drafting of the manuscript. Anastasia Zekeridou: concept and design of the paper, analysis and interpretation of the data, drafting of the manuscript, and critical revision for important intellectual content. François Ochsner: acquisition of data, analysis and interpretation, and critical revision for important intellectual content. Susanne Renaud: acquisition of data, analysis and interpretation, and critical revision of the draft for important intellectual content.

  • Study funding: No targeted funding reported.

  • Disclosure: The authors report no disclosures. Go to Neurology.org/nn for full disclosure forms.

  • Received January 8, 2015.
  • Accepted in final form March 9, 2015.
  • © 2015 American Academy of Neurology

This is an open access article distributed under the terms of the Creative Commons Attribution-Noncommercial No Derivative 3.0 License, which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially.

References

  1. 1.↵
    1. Lennon VA,
    2. Wingerchuk DM,
    3. Kryzer TJ,
    4. et al
    . A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 2004;364:2106–2112.
    OpenUrlCrossRefPubMed
  2. 2.↵
    1. Wingerchuk DM,
    2. Lennon VA,
    3. Lucchinetti CF,
    4. Pittock SJ,
    5. Weinshenker BG
    . The spectrum of neuromyelitis optica. Lancet Neurol 2007;6:805–815.
    OpenUrlCrossRefPubMed
  3. 3.↵
    1. Pittock SJ,
    2. Lennon VA,
    3. de Seze J,
    4. et al
    . Neuromyelitis optica and non organ-specific autoimmunity. Arch Neurol 2008;65:78–83.
    OpenUrlCrossRefPubMed
  4. 4.↵
    1. Pittock SJ,
    2. Lennon VA
    . Aquaporin-4 autoantibodies in a paraneoplastic context. Arch Neurol 2008;65:629–632.
    OpenUrlCrossRefPubMed
  5. 5.↵
    1. Quek AML,
    2. McKeon A,
    3. Lennon VA,
    4. et al
    . Effects of age and sex on aquaporin-4 autoimmunity. Arch Neurol 2012;69:1039–1043.
    OpenUrlCrossRefPubMed
  6. 6.↵
    1. Leite MI,
    2. Coutinho E,
    3. Lana-Peixoto M,
    4. et al
    . Myasthenia gravis and neuromyelitis optica spectrum disorder: a multicenter study of 16 patients. Neurology 2012;78:1601–1607.
    OpenUrlCrossRef
  7. 7.↵
    1. Ontaneda D,
    2. Fox RJ
    . Is neuromyelitis optica with advanced age of onset a paraneoplastic disorder? Int J Neurosci 2014;124:509–511.
    OpenUrlCrossRefPubMed

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
    • Acknowledgments
    • 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