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

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

Share

July 2020; 7 (4) Editor's CornerOpen Access

“Time to recharge”

Josep Dalmau
First published June 11, 2020, DOI: https://doi.org/10.1212/NXI.0000000000000779
Josep Dalmau
From the ICREA-IDIBAPS Hospital Clínic, University of Barcelona, Barcelona, Spain; and Department of Neurology, University of Pennsylvania, Pennsylvania.
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Full PDF
Citation
“Time to recharge”
Josep Dalmau
Neurol Neuroimmunol Neuroinflamm Jul 2020, 7 (4) e779; DOI: 10.1212/NXI.0000000000000779

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
226

Share

  • Article
  • Info & Disclosures
Loading

Embedded Image

This has been a challenging spring, and we all hope that the summer will bring the needed relief. To help you unwind, the July issue of Neurology® Neuroimmunology & Neuroinflammation contains excellent articles on a wide variety of topics that should hold your interest and help charge your brain batteries. Here I highlight 4 studies that I choose for the common theme of dealing with rare inflammatory disorders. I took the liberty of including a study of pregnant and lactating women with MS/NMOSD because investigations of this cohort are relatively rare.

Antimyelin-associated glycoprotein (MAG) antibody neuropathy is a slowly progressive, chronic polyneuropathy, associated with IgM monoclonal gammopathy. The current standard of care is rituximab, but approximately only 50% of patients benefit from it use. It was recently shown that the MYD88L265P mutation is the most common mutation in patients with Waldenström macroglobulinemia and IgM-monoclonal gammopathy of undetermined significance. This mutation results in constitutive activation of Bruton's tyrosine kinase (BTK) and NF-kB signaling. Ibrutinib is the first in-class inhibitor of BTK and has shown promising activity in a few patients with B-cell lymphomas especially those with MYD88L265P. Further support for the efficacy of ibrutinib is now provided in the article by Castellani et al.1 reporting the successful treatment of 3 patients with anti-MAG neuropathy associated with Waldenström macroglobulinemia with MYD88L265P. All 3 patients had stable improvement of their neuropathy that in one patient it was described as a dramatic improvement in gait stability. Ibrutunib was well tolerated, and all patients remain on treatment. Although a larger number of patients are needed to confirm these results, it is exciting that we may have an efficacious option for this disabling neuropathy.

CTLA4 deficiency is a rare primary immune deficiency disorder with a wide range of systemic manifestations. Neurologic manifestations have been reported in approximately 30% of cases, including autoimmune encephalitis or encephalomyelitis with perivascular lymphocytic infiltration, inflammatory demyelinating processes, and optic neuritis, among others. Ayrignac et al.2 add to this list in their case descriptions of 3 patients. Two of the patients were siblings who had symptom onset during childhood, including recurrent episodes of brain or spinal cord inflammatory processes, as has been described before. However, the third patient became symptomatic in her early 40s and developed progressive cerebellar ataxia and visual loss with bilaterally symmetric white matter changes similar to that seen in inherited leukodystrophies. Although the neurologic symptoms of these 3 patients occurred after the diagnosis of the CTLA4 deficiency, in 5% of patients, neurologic symptoms predate the diagnosis, supporting the importance of keeping in mind the broad neurologic phenotype.

Sarcoidosis is an enigmatic disorder that may present with a wide range of clinical manifestations. Neurologic involvement occurs in approximately 5% of cases and in approximately half of these patients is the initial manifestation of the disease. Sarcoidosis-associated myelopathy has features that may overlap with other inflammatory spinal cord disorders that can confound the diagnosis. To determine if there is a clinical and imaging phenotype of sarcoidosis-associated myelopathy, Murphy et al.3 reviewed the characteristics of 62 patients with this complication. Most of the patients had a chronic course with predominant sensory symptoms. Four imaging patterns were identified on spine MRI with longitudinally extensive myelitis with predominantly dorsal subpial and/or meningeal enhancement being the most common. This has been previously reported, and the authors consider that this pattern should be considered the “classic” imaging phenotype of sarcoidosis-associated myelopathy. Enhancement was present in all but one case and across all lesion types; subpial enhancement frequently occurred at locations with co-existing structural changes such as disc herniations or cervical spondylosis. This novel observation lead the authors to hypothesize that increased permeability of the spinal cord barrier at the sites of mechanical stress may be a key step in the evolution of the inflammatory lesions in sarcoidosis-associated myelopathy.

Ciplea et al.4 identified 23 patients with MS or NMOSD who received monoclonal antibodies during pregnancy and/or lactation to determine possible adverse effects on the infants. After a median follow-up of 1 year, they found no negative effects on overall health and development. Those infants who were exposed to natalizumab during the third trimester had lower birth weight and more hospitalizations in the first year of life but still had normal development. Several infants had transient hematologic abnormalities including mild or moderate anemia and/or thrombocytopenia. There was a slightly higher than expected rate of premature deliveries noted in the cohort without a clear attribution to the underlying demyelinating disease or exposure to monoclonal antibody. Based on this study and previous reports and with the caution that the overall sample size is limited, the authors conclude that there is a low probability of harmful effects for infants exposed to breastmilk after monoclonal antibody treatment of the mother.

In addition to these studies, the July issue of Neurology: Neuroimmunology & Neuroinflammation contains a review on the current knowledge of the transfer of monoclonal antibodies into breastmilk that complements nicely the study of Ciplea et al. and 2 clinical/scientific notes describing COVID-19 infection concurrent with Guillain-Barré syndrome and MS.5,–,7 I hope that these and all the other interesting articles in this issue will catch your attention.

Study funding

No targeted funding reported.

Disclosure

J. Dalmau holds the patents for the use of Ma2, NMDAR, GABABR, GABAAR, DPPX, and IgLON5 as autoantibody tests and receives royalties from the use of these tests. He is the editor of Neurology: Neuroimmunology & Neuroinflammation. Go to Neurology.org/NN for full disclosures.

Footnotes

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

  • Copyright © 2020 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.

References

  1. 1.↵
    1. Castellani F,
    2. Visentin A,
    3. Campagnolo M, et al
    . The Bruton tyrosine kinase inhibitor ibrutinib improves anti-MAG antibody polyneuropathy. Neuroimmunol Neuroinflamm 2020;7:e720. doi: 10.1212/NXI.0000000000000720.
    OpenUrlAbstract/FREE Full Text
  2. 2.↵
    1. Ayrignac X,
    2. Goulabchand R,
    3. Jeziorski E, et al
    . Two neurologic facets of CTLA4-related haploinsufficiency. Neuroimmunol Neuroinflamm 2020;7:e751. doi: 10.1212/NXI.0000000000000751.
    OpenUrlAbstract/FREE Full Text
  3. 3.↵
    1. Murphy OC,
    2. Salazar-Camelo RA,
    3. Jimenez Arango JA, et al
    . Clinical and MRI phenotypes of sarcoidosis-associated myelopathy. Neuroimmunol Neuroinflamm 2020;7:e722. doi: 10.1212/NXI.0000000000000722.
    OpenUrlCrossRef
  4. 4.↵
    1. Ciplea AI,
    2. Langer-Gould AS,
    3. Thiel S, et al
    . Safety of potential breast milk exposure to IFN-β or glatiramer acetate: One-year infant outcomes. Neurol Neuroimmunol Neuroinflamm 2020;7:e757. doi: 10.1212/NXI.0000000000000757.
    OpenUrlAbstract/FREE Full Text
  5. 5.↵
    1. LaHue SC,
    2. Anderson A,
    3. Krusko KM, et al
    . Transfer of monoclonal antibodies into breastmilk in neurologic and non-neurologic diseases. Neuroimmunol Neuroinflamm 2020;7:e769. doi: 10.1212/NXI.0000000000000769.
    OpenUrlCrossRef
  6. 6.↵
    1. Alberti P,
    2. Beretta S,
    3. Piatti M, et al
    . Guillain-Barré syndrome related to COVID-19 infection. Neuroimmunol Neuroinflamm 2020;7:e741. doi: 10.1212/NXI.0000000000000741.
    OpenUrlAbstract/FREE Full Text
  7. 7.↵
    1. Barzegar M,
    2. Mirmosayyeb O,
    3. Nehzat N, et al
    . COVID-19 infection in a patient with multiple sclerosis treated with fingolimod. Neuroimmunol Neuroinflamm 2020;7:e753. doi: 10.1212/NXI.0000000000000753.
    OpenUrlAbstract/FREE 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
    • Study funding
    • Disclosure
    • Footnotes
    • References
  • Info & Disclosures

Related Articles

  • No related articles found.

Alert Me

  • Alert me when eletters are published
Advertisement
Neurology - Neuroimmunology Neuroinflammation: 9 (4)

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: Genetics
  • Neurology: Neuroimmunology & Neuroinflammation
  • Neurology: Education
  • AAN.com
  • AANnews
  • Continuum
  • Brain & Life
  • Neurology Today

Wolters Kluwer Logo

Neurology: Neuroimmunology & Neuroinflammation
Online ISSN: 2332-7812

© 2022 American Academy of Neurology

  • Privacy Policy
  • Feedback
  • Advertise