Skip to main content
Advertisement
  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Diversity, Equity, & Inclusion (DEI)
    • Neurology: Clinical Practice Accelerator
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • COVID-19
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Neurology: Neuroimmunology & Neuroinflammation COVID-19 Article Hub
    • Null Hypothesis
    • Patient Pages
    • Topics A-Z
    • Translations
    • UDDA Revision Series
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit Manuscript
    • Author Center

Advanced Search

Main menu

  • Neurology.org
  • Journals
    • Neurology
    • Clinical Practice
    • Education
    • Genetics
    • Neuroimmunology & Neuroinflammation
  • Online Sections
    • Neurology Video Journal Club
    • Diversity, Equity, & Inclusion (DEI)
    • Neurology: Clinical Practice Accelerator
    • Practice Buzz
    • Practice Current
    • Residents & Fellows
    • Without Borders
  • Collections
    • COVID-19
    • Disputes & Debates
    • Health Disparities
    • Infographics
    • Neurology: Neuroimmunology & Neuroinflammation COVID-19 Article Hub
    • Null Hypothesis
    • Patient Pages
    • Topics A-Z
    • Translations
    • UDDA Revision Series
  • Podcast
  • CME
  • About
    • About the Journals
    • Contact Us
    • Editorial Board
  • Authors
    • Submit 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

November 2017; 4 (6) ArticleOpen Access

Cryptogenic NORSE

Its distinctive clinical features and response to immunotherapy

Takahiro Iizuka, Naomi Kanazawa, Juntaro Kaneko, Naomi Tominaga, Yutaka Nonoda, Atsuko Hara, Yuya Onozawa, Hiroki Asari, Takashi Hata, Junya Kaneko, Kenji Yoshida, Yoshihiro Sugiura, Yoshikazu Ugawa, Masashi Watanabe, Hitomi Tomita, Arifumi Kosakai, Atsushi Kaneko, Daisuke Ishima, Eiji Kitamura, Kazutoshi Nishiyama
First published September 25, 2017, DOI: https://doi.org/10.1212/NXI.0000000000000396
Takahiro Iizuka
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Naomi Kanazawa
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
BS
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Juntaro Kaneko
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Naomi Tominaga
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Yutaka Nonoda
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Atsuko Hara
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Yuya Onozawa
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
PhD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Hiroki Asari
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Takashi Hata
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Junya Kaneko
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Kenji Yoshida
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Yoshihiro Sugiura
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD, PhD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Yoshikazu Ugawa
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD, PhD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Masashi Watanabe
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Hitomi Tomita
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Arifumi Kosakai
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Atsushi Kaneko
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Daisuke Ishima
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Eiji Kitamura
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Kazutoshi Nishiyama
From the Department of Neurology (T.I., N.K., J.K., N.T., A.Kaneko, D.I., E.K., K.N.), Department of Pediatrics (Y.N.), and Department of Pathology (A.H.), Kitasato University School of Medicine; Department of Clinical Laboratory (Y.O.), Kitasato University Hospital, Sagamihara, Japan; Department of Neurology (H.A., T.H.), Shizuoka City Shimizu Hospital, Shizuoka, Japan; Department of Emergency and Critical Care Medicine (J.K.), Nippon Medical School Tama Nagayama Hospital, Tama, Japan; Department of Neurology (K.Y., Y.S., Y.U.), School of Medicine, Fukushima Medical Hospital and Fukushima Global Medical Science Center (Y.U.), Advanced Clinical Research Center, Fukushima Medical University, Fukushima, Japan; Department of Neurology (M.W., H.T.), Ehime Prefectural Central Hospital, Matsuyama, Japan; and Department of Neurology (A.Kosakai), Keiyu Hospital, Yokohama, Japan.
MD, PhD
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Full PDF
Citation
Cryptogenic NORSE
Its distinctive clinical features and response to immunotherapy
Takahiro Iizuka, Naomi Kanazawa, Juntaro Kaneko, Naomi Tominaga, Yutaka Nonoda, Atsuko Hara, Yuya Onozawa, Hiroki Asari, Takashi Hata, Junya Kaneko, Kenji Yoshida, Yoshihiro Sugiura, Yoshikazu Ugawa, Masashi Watanabe, Hitomi Tomita, Arifumi Kosakai, Atsushi Kaneko, Daisuke Ishima, Eiji Kitamura, Kazutoshi Nishiyama
Neurol Neuroimmunol Neuroinflamm Nov 2017, 4 (6) e396; DOI: 10.1212/NXI.0000000000000396

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
2828

Share

  • Article
  • Figures & Data
  • Info & Disclosures
Loading

Abstract

Objective: To report the distinctive clinical features of cryptogenic new-onset refractory status epilepticus (C-NORSE) and the C-NORSE score based on initial clinical assessments.

Methods: A retrospective study was conducted for 136 patients with clinically suspected autoimmune encephalitis who underwent testing for autoantibodies to neuronal surface antigens between January 1, 2007, and August 31, 2016. Eleven patients with C-NORSE were identified. Their clinical features were compared with those of 32 patients with anti-NMDA receptor encephalitis (NMDARE).

Results: The clinical outcome of 11 patients (median age, 27 years; 7 [64%] women) with C-NORSE was evaluated after a median follow-up of 11 months (range, 6–111 months). Status epilepticus was frequently preceded by fever (10/11 [91%]). Brain MRIs showed symmetric T2/fluid-attenuated inversion recovery hyperintensities (8/11 [73%]) and brain atrophy (9/11 [82%]). Only 2 of the 10 treated patients responded to the first-line immunotherapy, and 4 of the 5 patients treated with IV cyclophosphamide responded to the therapy. The long-term outcome was poor in 8 patients (73%). Compared with 32 patients with NMDARE (median age, 27 years; 24 [75%] women), those with C-NORSE had more frequent prodromal fever, status epilepticus, ventilatory support, and symmetric brain MRI abnormalities, had less frequent involuntary movements, absent psychobehavioral symptoms, CSF oligoclonal bands, or tumor association, and had a worse outcome. The C-NORSE score was higher in patients with C-NORSE than those with NMDARE.

Conclusions: Patients with C-NORSE have a spectrum of clinical-immunological features different from those with NMDARE. The C-NORSE score may be useful for discrimination between them. Some patients could respond to immunotherapy.

GLOSSARY

AE=
autoimmune encephalitis;
AED=
antiepileptic drug;
AERRPS=
acute encephalitis with refractory repetitive partial seizures;
AMPAR=
α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor;
C-NORSE=
cryptogenic new-onset refractory status epilepticus;
DESC=
devastating epileptic encephalopathy in school-age children;
FIRES=
febrile infection-related epilepsy syndrome;
GABAaR=
γ-aminobutyric acid A receptor;
GABAbR=
γ-aminobutyric acid B receptor;
GCSE=
generalized convulsive status epilepticus;
HSV=
herpes simplex virus;
IL=
interleukin;
IVCPA=
IV cyclophosphamide;
IVIg=
IV immunoglobulin;
IVMP=
IV high-dose methylprednisolone;
LGI1=
leucine-rich glioma-inactivated 1;
mRS=
modified Rankin Scale;
NMDARE=
anti-NMDA receptor encephalitis;
NSA=
neuronal cell-surface antigen;
OCB=
oligoclonal band;
PLEX=
plasma exchange;
RSE=
refractory status epilepticus;
SE=
status epilepticus;
STESS=
Status Epilepticus Severity Score

Footnotes

  • Funding information and disclosures are provided at the end of the article. Go to Neurology.org/nn for full disclosure forms. The Article Processing Charge was funded by the authors.

  • Supplemental data at Neurology.org/nn

  • Received June 26, 2017.
  • Accepted in final form August 2, 2017.
  • Copyright © 2017 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

You must ensure that your Disclosures have been updated within the previous six months. Please go to our Submission Site to add or update your Disclosure information.

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. [email protected]
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
    • AUTHOR CONTRIBUTIONS
    • STUDY FUNDING
    • DISCLOSURE
    • ACKNOWLEDGMENT
    • Footnotes
    • REFERENCES
  • Figures & Data
  • Info & Disclosures
Advertisement

Hastening the Diagnosis of Amyotrophic Lateral Sclerosis

Dr. Brian Callaghan and Dr. Kellen Quigg

► Watch

Topics Discussed

  • Autoimmune diseases
  • Encephalitis
  • MRI
  • Paraneoplastic syndrome
  • Status epilepticus

Alert Me

  • Alert me when eletters are published

Recommended articles

  • Contemporary Issues
    New onset refractory status epilepticus research
    What is on the horizon?
    Teneille E. Gofton, Nicolas Gaspard, Sara E. Hocker et al.
    Neurology, March 20, 2019
  • Article
    Clinically based score predicting cryptogenic NORSE at the early stage of status epilepticus
    Atsuko Yanagida, Naomi Kanazawa, Juntaro Kaneko et al.
    Neurology: Neuroimmunology & Neuroinflammation, July 29, 2020
  • Editorial
    New-onset super-refractory status epilepticus (NOSRSE)
    What's in a name?
    Stephan Rüegg et al.
    Neurology, September 17, 2020
  • Article
    Decreased occipital lobe metabolism by FDG-PET/CT
    An anti–NMDA receptor encephalitis biomarker
    John C. Probasco, Lilja Solnes, Abhinav Nalluri et al.
    Neurology: Neuroimmunology & Neuroinflammation, November 15, 2017
Neurology - Neuroimmunology Neuroinflammation: 11 (1)

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
  • Continuum
  • Brain & Life
  • Neurology Today

Wolters Kluwer Logo

Neurology: Neuroimmunology & Neuroinflammation
Online ISSN: 2332-7812

© 2023 American Academy of Neurology

  • Privacy Policy
  • Feedback
  • Advertise