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December 2015; 2 (6) ArticleOpen Access

Fulminant demyelinating encephalomyelitis

Insights from antibody studies and neuropathology

Franziska Di Pauli, Romana Höftberger, Markus Reindl, Ronny Beer, Paul Rhomberg, Kathrin Schanda, Douglas Sato, Kazuo Fujihara, Hans Lassmann, Erich Schmutzhard, Thomas Berger
First published November 4, 2015, DOI: https://doi.org/10.1212/NXI.0000000000000175
Franziska Di Pauli
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Romana Höftberger
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Markus Reindl
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Ronny Beer
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Paul Rhomberg
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Kathrin Schanda
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Douglas Sato
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Kazuo Fujihara
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Hans Lassmann
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Erich Schmutzhard
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Thomas Berger
From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
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Full PDF
Citation
Fulminant demyelinating encephalomyelitis
Insights from antibody studies and neuropathology
Franziska Di Pauli, Romana Höftberger, Markus Reindl, Ronny Beer, Paul Rhomberg, Kathrin Schanda, Douglas Sato, Kazuo Fujihara, Hans Lassmann, Erich Schmutzhard, Thomas Berger
Neurol Neuroimmunol Neuroinflamm Dec 2015, 2 (6) e175; DOI: 10.1212/NXI.0000000000000175

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Article Information

vol. 2 no. 6 e175
DOI: 
https://doi.org/10.1212/NXI.0000000000000175
PubMed: 
26587556

Published By: 
Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology
Online ISSN: 
2332-7812
History: 
  • Received July 28, 2015
  • Accepted in final form September 18, 2015
  • First Published November 4, 2015.

Copyright & Usage: 
© 2015 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.

Author Disclosures

    1. Franziska Di Pauli, MD,
    2. Romana Höftberger, MD,
    3. Markus Reindl, PhD,
    4. Ronny Beer, MD,
    5. Paul Rhomberg, MD,
    6. Kathrin Schanda, MSc,
    7. Douglas Sato, MD,
    8. Kazuo Fujihara, MD,
    9. Hans Lassmann, MD,
    10. Erich Schmutzhard, MD and
    11. Thomas Berger, MD
  1. Franziska Di Pauli, MD,
  2. Scientific Advisory Boards:
    1. NONE

    Gifts:
    1. NONE

    Funding for Travel or Speaker Honoraria:
    1. Biogen idec, funding for travel to conference and speaker honoraria Genzyme, funding for travel to conference and speaker honoraria

    Editorial Boards:
    1. NONE

    Patents:
    1. NONE

    Publishing Royalties:
    1. NONE

    Employment, Commercial Entity:
    1. NONE

    Consultancies:
    1. NONE

    Speakers' Bureaus:
    1. NONE

    Other Activities:
    1. NONE

    Clinical Procedures or Imaging Studies:
    1. NONE

    Research Support, Commercial Entities:
    1. NONE

    Research Support, Government Entities:
    1. NONE

    Research Support, Academic Entities:
    1. NONE

    Research Support, Foundations and Societies:
    1. NONE

    Stock/Stock Options/Board of Directors Compensation:
    1. NONE

    License Fee Payments, Technology or Inventions:
    1. NONE

    Royalty Payments, Technology or Inventions:
    1. NONE

    Stock/Stock Options, Research Sponsor:
    1. NONE

    Stock/Stock Options, Medical Equipment & Materials:
    1. NONE

    Legal Proceedings:
    1. NONE

  3. Romana Höftberger, MD,
  4. Scientific Advisory Boards:
    1. NONE

    Gifts:
    1. NONE

    Funding for Travel or Speaker Honoraria:
    1. NONE

    Editorial Boards:
    1. NONE

    Patents:
    1. NONE

    Publishing Royalties:
    1. NONE

    Employment, Commercial Entity:
    1. NONE

    Consultancies:
    1. NONE

    Speakers' Bureaus:
    1. NONE

    Other Activities:
    1. NONE

    Clinical Procedures or Imaging Studies:
    1. NONE

    Research Support, Commercial Entities:
    1. NONE

    Research Support, Government Entities:
    1. NONE

    Research Support, Academic Entities:
    1. NONE

    Research Support, Foundations and Societies:
    1. NONE

    Stock/Stock Options/Board of Directors Compensation:
    1. NONE

    License Fee Payments, Technology or Inventions:
    1. NONE

    Royalty Payments, Technology or Inventions:
    1. NONE

    Stock/Stock Options, Research Sponsor:
    1. NONE

    Stock/Stock Options, Medical Equipment & Materials:
    1. NONE

    Legal Proceedings:
    1. NONE

  5. Markus Reindl, PhD,
  6. Scientific Advisory Boards:
    1. NONE

    Gifts:
    1. NONE

    Funding for Travel or Speaker Honoraria:
    1. NONE

    Editorial Boards:
    1. PLOS ONE, academic editor, 2012-Current Medicinal Chemistry, editorial board, 2006-2012 Autoimmune Diseases, 2012-2013

    Patents:
    1. NONE

    Publishing Royalties:
    1. NONE

    Employment, Commercial Entity:
    1. NONE

    Consultancies:
    1. NONE

    Speakers' Bureaus:
    1. NONE

    Other Activities:
    1. The University Hospital and Medical University of Innsbruck (Austria, Markus Reindl) receive payments for antibody assays (NMDAR, AQP4 and other autoantibodies) and for AQP4 antibody validation experiments organized by Euroimmun.

    Clinical Procedures or Imaging Studies:
    1. NONE

    Research Support, Commercial Entities:
    1. NONE

    Research Support, Government Entities:
    1. (1) This study was supported by a research grant from the Austrian Federal Ministery of Science and Economy (grant BIG WIG MS).

    Research Support, Academic Entities:
    1. NONE

    Research Support, Foundations and Societies:
    1. NONE

    Stock/Stock Options/Board of Directors Compensation:
    1. NONE

    License Fee Payments, Technology or Inventions:
    1. NONE

    Royalty Payments, Technology or Inventions:
    1. NONE

    Stock/Stock Options, Research Sponsor:
    1. NONE

    Stock/Stock Options, Medical Equipment & Materials:
    1. NONE

    Legal Proceedings:
    1. NONE

  7. Ronny Beer, MD,
  8. Scientific Advisory Boards:
    1. NONE

    Gifts:
    1. NONE

    Funding for Travel or Speaker Honoraria:
    1. NONE

    Editorial Boards:
    1. NONE

    Patents:
    1. NONE

    Publishing Royalties:
    1. NONE

    Employment, Commercial Entity:
    1. NONE

    Consultancies:
    1. NONE

    Speakers' Bureaus:
    1. NONE

    Other Activities:
    1. NONE

    Clinical Procedures or Imaging Studies:
    1. NONE

    Research Support, Commercial Entities:
    1. NONE

    Research Support, Government Entities:
    1. (1) Austrian Science Fund, KLI 375-B00, Principal Investigator, 2014-2016.

    Research Support, Academic Entities:
    1. NONE

    Research Support, Foundations and Societies:
    1. NONE

    Stock/Stock Options/Board of Directors Compensation:
    1. NONE

    License Fee Payments, Technology or Inventions:
    1. NONE

    Royalty Payments, Technology or Inventions:
    1. NONE

    Stock/Stock Options, Research Sponsor:
    1. NONE

    Stock/Stock Options, Medical Equipment & Materials:
    1. NONE

    Legal Proceedings:
    1. NONE

  9. Paul Rhomberg, MD,
  10. Scientific Advisory Boards:
    1. NONE

    Gifts:
    1. NONE

    Funding for Travel or Speaker Honoraria:
    1. NONE

    Editorial Boards:
    1. NONE

    Patents:
    1. NONE

    Publishing Royalties:
    1. NONE

    Employment, Commercial Entity:
    1. NONE

    Consultancies:
    1. NONE

    Speakers' Bureaus:
    1. NONE

    Other Activities:
    1. NONE

    Clinical Procedures or Imaging Studies:
    1. NONE

    Research Support, Commercial Entities:
    1. NONE

    Research Support, Government Entities:
    1. NONE

    Research Support, Academic Entities:
    1. NONE

    Research Support, Foundations and Societies:
    1. NONE

    Stock/Stock Options/Board of Directors Compensation:
    1. NONE

    License Fee Payments, Technology or Inventions:
    1. NONE

    Royalty Payments, Technology or Inventions:
    1. NONE

    Stock/Stock Options, Research Sponsor:
    1. NONE

    Stock/Stock Options, Medical Equipment & Materials:
    1. NONE

    Legal Proceedings:
    1. NONE

  11. Kathrin Schanda, MSc,
  12. Scientific Advisory Boards:
    1. NONE

    Gifts:
    1. NONE

    Funding for Travel or Speaker Honoraria:
    1. NONE

    Editorial Boards:
    1. NONE

    Patents:
    1. NONE

    Publishing Royalties:
    1. NONE

    Employment, Commercial Entity:
    1. NONE

    Consultancies:
    1. NONE

    Speakers' Bureaus:
    1. NONE

    Other Activities:
    1. NONE

    Clinical Procedures or Imaging Studies:
    1. NONE

    Research Support, Commercial Entities:
    1. NONE

    Research Support, Government Entities:
    1. NONE

    Research Support, Academic Entities:
    1. NONE

    Research Support, Foundations and Societies:
    1. NONE

    Stock/Stock Options/Board of Directors Compensation:
    1. NONE

    License Fee Payments, Technology or Inventions:
    1. NONE

    Royalty Payments, Technology or Inventions:
    1. NONE

    Stock/Stock Options, Research Sponsor:
    1. NONE

    Stock/Stock Options, Medical Equipment & Materials:
    1. NONE

    Legal Proceedings:
    1. NONE

  13. Douglas Sato, MD,
  14. Scientific Advisory Boards:
    1. NONE

    Gifts:
    1. NONE

    Funding for Travel or Speaker Honoraria:
    1. Speaker honoraria from Novartis

    Editorial Boards:
    1. Associate editor of Arquivos de Neuropsiquiatria (2013-present)

    Patents:
    1. NONE

    Publishing Royalties:
    1. NONE

    Employment, Commercial Entity:
    1. NONE

    Consultancies:
    1. NONE

    Speakers' Bureaus:
    1. NONE

    Other Activities:
    1. NONE

    Clinical Procedures or Imaging Studies:
    1. NONE

    Research Support, Commercial Entities:
    1. NONE

    Research Support, Government Entities:
    1. (1) Ministry of Education, Culture, Sports, Science & Technology (MEXT) in Japan, Japanese Government Scholarship Program, 4 years (2010 - 2014)

    Research Support, Academic Entities:
    1. NONE

    Research Support, Foundations and Societies:
    1. (1) Grant-in-aid for scientific research from the Japan Society for the Promotion of Science (KAKENHI 15K19472) (2) Research support from CAPES/Brasil (88881.068012/2014)

    Stock/Stock Options/Board of Directors Compensation:
    1. NONE

    License Fee Payments, Technology or Inventions:
    1. NONE

    Royalty Payments, Technology or Inventions:
    1. NONE

    Stock/Stock Options, Research Sponsor:
    1. NONE

    Stock/Stock Options, Medical Equipment & Materials:
    1. NONE

    Legal Proceedings:
    1. NONE

  15. Kazuo Fujihara, MD,
  16. Scientific Advisory Boards:
    1. Serves on scientific advisory boards for Bayer Schering Pharma, Biogen Idec, Mitsubishi Tanabe Pharma Corporation, Novartis Pharma, Chugai Pharmaceutical, Ono Pharmaceutical, Nihon Pharmaceutical, Merck Serono, Alexion Pharmaceuticals, Medimmune and Medical Review.

    Gifts:
    1. NONE

    Funding for Travel or Speaker Honoraria:
    1. has received funding for travel and speaker honoraria from Bayer Schering Pharma, Biogen Idec, Eisai Inc., Mitsubishi Tanabe Pharma Corporation, Novartis Pharma, Astellas Pharma Inc., Takeda Pharmaceutical Company Limited, Asahi Kasei Medical Co., Daiichi Sankyo, and Nihon Pharmaceutical.

    Editorial Boards:
    1. Serve as an editorial board member of Clinical and Experimental Neuroimmunology (2009-present) and a advisory board member of Sri Lanka journal of Neurology.

    Patents:
    1. NONE

    Publishing Royalties:
    1. NONE

    Employment, Commercial Entity:
    1. NONE

    Consultancies:
    1. NONE

    Speakers' Bureaus:
    1. NONE

    Other Activities:
    1. NONE

    Clinical Procedures or Imaging Studies:
    1. NONE

    Research Support, Commercial Entities:
    1. has received research support from Bayer Schering Pharma, Biogen Idec Japan, Asahi Kasei Medical, The Chemo-Sero-Therapeutic Research Institute, Teva Pharmaceutical, Mitsubishi Tanabe Pharma, Teijin Pharma, Chugai Pharmaceutical,Ono Pharmaceutical, Nihon Pharmaceutical, and Genzyme Japan.

    Research Support, Government Entities:
    1. funded as the investigator (2010?present) by the Grants-in-Aid for Scientific Research from the Ministry of Education, Science and Technology of Japan and as the secondary investigator by the Grants-in-Aid for Scientific Research from the Ministry of Health, Welfare and Labor of Japan (2010?present).

    Research Support, Academic Entities:
    1. NONE

    Research Support, Foundations and Societies:
    1. NONE

    Stock/Stock Options/Board of Directors Compensation:
    1. NONE

    License Fee Payments, Technology or Inventions:
    1. NONE

    Royalty Payments, Technology or Inventions:
    1. NONE

    Stock/Stock Options, Research Sponsor:
    1. NONE

    Stock/Stock Options, Medical Equipment & Materials:
    1. NONE

    Legal Proceedings:
    1. NONE

  17. Hans Lassmann, MD,
  18. Scientific Advisory Boards:
    1. NONE

    Gifts:
    1. NONE

    Funding for Travel or Speaker Honoraria:
    1. I have received speaker and travel honoraria for several scientific meetings from Biogen Idec, Novartis and Teva.

    Editorial Boards:
    1. I am member of the Editorial Board of several Journals in the fields of Neurology and Neuroscience.

    Patents:
    1. NONE

    Publishing Royalties:
    1. NONE

    Employment, Commercial Entity:
    1. NONE

    Consultancies:
    1. I have served as a consultant for Biogen Idec and AMGEN.

    Speakers' Bureaus:
    1. NONE

    Other Activities:
    1. NONE

    Clinical Procedures or Imaging Studies:
    1. NONE

    Research Support, Commercial Entities:
    1. NONE

    Research Support, Government Entities:
    1. Austrian Science Fund, Projects P19854, P 24245;principal investigator; 2007-2012 European Union FP7 Project Neuroprmise Pl 018637 2007-2011; Subproject; E-Rare Project EDEN; 2011-2014; subproject This funding is not directly related to the respective article

    Research Support, Academic Entities:
    1. NONE

    Research Support, Foundations and Societies:
    1. NONE

    Stock/Stock Options/Board of Directors Compensation:
    1. NONE

    License Fee Payments, Technology or Inventions:
    1. NONE

    Royalty Payments, Technology or Inventions:
    1. NONE

    Stock/Stock Options, Research Sponsor:
    1. NONE

    Stock/Stock Options, Medical Equipment & Materials:
    1. NONE

    Legal Proceedings:
    1. NONE

  19. Erich Schmutzhard, MD and
  20. Scientific Advisory Boards:
    1. NONE

    Gifts:
    1. NONE

    Funding for Travel or Speaker Honoraria:
    1. UCB and ZOLL Medical: speaker honoraria

    Editorial Boards:
    1. NONE

    Patents:
    1. NONE

    Publishing Royalties:
    1. NONE

    Employment, Commercial Entity:
    1. NONE

    Consultancies:
    1. NONE

    Speakers' Bureaus:
    1. NONE

    Other Activities:
    1. NONE

    Clinical Procedures or Imaging Studies:
    1. NONE

    Research Support, Commercial Entities:
    1. NONE

    Research Support, Government Entities:
    1. NONE

    Research Support, Academic Entities:
    1. NONE

    Research Support, Foundations and Societies:
    1. NONE

    Stock/Stock Options/Board of Directors Compensation:
    1. NONE

    License Fee Payments, Technology or Inventions:
    1. NONE

    Royalty Payments, Technology or Inventions:
    1. NONE

    Stock/Stock Options, Research Sponsor:
    1. NONE

    Stock/Stock Options, Medical Equipment & Materials:
    1. NONE

    Legal Proceedings:
    1. NONE

  21. Thomas Berger, MD
  22. Scientific Advisory Boards:
    1. NONE

    Gifts:
    1. NONE

    Funding for Travel or Speaker Honoraria:
    1. NONE

    Editorial Boards:
    1. NONE

    Patents:
    1. NONE

    Publishing Royalties:
    1. NONE

    Employment, Commercial Entity:
    1. NONE

    Consultancies:
    1. NONE

    Speakers' Bureaus:
    1. NONE

    Other Activities:
    1. NONE

    Clinical Procedures or Imaging Studies:
    1. NONE

    Research Support, Commercial Entities:
    1. NONE

    Research Support, Government Entities:
    1. NONE

    Research Support, Academic Entities:
    1. NONE

    Research Support, Foundations and Societies:
    1. NONE

    Stock/Stock Options/Board of Directors Compensation:
    1. NONE

    License Fee Payments, Technology or Inventions:
    1. NONE

    Royalty Payments, Technology or Inventions:
    1. NONE

    Stock/Stock Options, Research Sponsor:
    1. NONE

    Stock/Stock Options, Medical Equipment & Materials:
    1. NONE

    Legal Proceedings:
    1. NONE

  1. From the Clinical Department of Neurology (F.D.P., M.R., R.B., K.S., E.S., T.B.) and Department of Neuroradiology (P.R.), Medical University of Innsbruck; Institute of Neurology (R.H.) and Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Departments of Neurology and Multiple Sclerosis Therapeutics (D.S., K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan.
  1. Correspondence to Dr. Berger: thomas.berger{at}i-med.ac.at
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