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AQP4-autoimmunity in Japan and Germany - a comparative retrospective study in neuromyelitis optica spectrum disorders
Author(s): ,
A.U. Brandt
Affiliations:
NeuroCure Clinical Research Center, Charité – Universitätsmedizin Berlin, Berlin, Germany; Department of Neurology, University of California Irvine, Irvine, CA, United States
,
M. Mori
Affiliations:
Department of Neurology, Chiba University, Chiba, Japan
,
H.G. Zimmermann
Affiliations:
NeuroCure Clinical Research Center, Charité – Universitätsmedizin Berlin, Berlin, Germany
,
N. Borisow
Affiliations:
NeuroCure Clinical Research Center, Charité – Universitätsmedizin Berlin, Berlin, Germany
,
F. Schmidt
Affiliations:
NeuroCure Clinical Research Center, Charité – Universitätsmedizin Berlin, Berlin, Germany
,
A. Uzawa
Affiliations:
Department of Neurology, Chiba University, Chiba, Japan
,
H. Masuda
Affiliations:
Department of Neurology, Chiba University, Chiba, Japan
,
R. Ohtani
Affiliations:
Department of Neurology, Chiba University, Chiba, Japan
,
K. Sugimoto
Affiliations:
Department of Neurology, Chiba University, Chiba, Japan
,
J. Liu
Affiliations:
Department of Neurology, Chiba University, Chiba, Japan
,
K. Ruprecht
Affiliations:
Department of Neurology, Charité – Universitätsmedizin Berlin
,
J. Bellmann-Strobl
Affiliations:
NeuroCure Clinical Research Center, Charité – Universitätsmedizin Berlin, Berlin, Germany; Experimental and Clinical Research Center, Max Delbrück Center for Molecular Medicine and Charité – Universitätsmedizin Berlin, Berlin, Germany
,
S. Kuwabara
Affiliations:
Department of Neurology, Chiba University, Chiba, Japan
F. Paul
Affiliations:
NeuroCure Clinical Research Center, Charité – Universitätsmedizin Berlin, Berlin, Germany; Experimental and Clinical Research Center, Max Delbrück Center for Molecular Medicine and Charité – Universitätsmedizin Berlin, Berlin, Germany
ECTRIMS Online Library. Brandt A. Oct 12, 2018; 228823; P981
Alexander U. Brandt
Alexander U. Brandt
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Abstract: P981

Type: Poster Sessions

Abstract Category: Clinical aspects of MS - MS Variants

Introduction: Neuromyelitis optica spectrums disorders (NMOSD) are autoimmune inflammatory conditions of the central nervous system, which share an overlapping clinical phenotype with optic neuritis and myelitis and may also show brain and brainstem involvement. Disease presentation and prognosis differ substantially between different regions in the world. Contrasting patients in different regions potentially allows investigating disease-relevant factors derived from environment but also genetic background in NMOSD.
Objectives: To investigate differences in disease presentation, course and treatment of NMOSD between Germany and Japan.
Aims: To recognize modulators of NMOSD in order to better understand disease mechanisms and aid in future treatment development
Methods: Retrospective, two center study involving one German center and one Japanese center, both large referral centers for NMOSD. Data was collected from ongoing prospective observational studies in both centers. Patients were included, if they fulfilled the 2015 IPND criteria for NMOSD and tested seropositive for aquaporin-4 IgG.
Results: A total of 38 patients were included from Berlin (35 female), 54 from Chiba (48 female). Mean disease duration was 8±7 years in Berlin and 13±11 years in Chiba. Patients in Chiba had a very similar age at disease onset (42±15 years) as patients in Berlin (42.5±15.3years, W=1061.5, p=0.7812926). Histogram analysis revealed three peaks of onset, which were present in both the Japanese and German cohorts: 1) around 20 years of age, 2) around 40 years of age, and 3) around 60 years of age. Patients in Chiba presented more frequently (21 from 54 patients) with brain attacks (area postrema, brainstem or cerebral syndrome) than patients from Berlin (4 from 36 patients, p=0.008). Treatment in Chiba relied mostly on corticosteroids alone or in combination with azathioprine, whereas Berlin patients were treated most often with rituximab or azathioprine.
Conclusions: Aquaporin-4-IgG seropositive NMOSD patients show different clinical disease phenotypes in Germany and Japan and are treated differently. Underlying factors investigating the cause of different disease characteristics should be investigated further in a prospective study and might allow insight into genetic or environmental disease modulators.
Disclosure: Alexander U. Brandt is cofounder and shareholder of Motognosis and Nocturne. He is named as inventor on several patent applications regarding MS serum biomarkers, OCT image analysis and perceptive visual computing.
Masahiro Mori: nothing to disclose.
Hanna G. Zimmermann received a research grant from Novartis and speaking fees from Teva.
Nadja Borisow: nothing to disclose.
Felix Schmidt: nothing to disclose.
Akiyuki Uzawa: nothing to disclose.
Hiroki Masuda: nothing to disclose.
Ryohei Ohtani: nothing to disclose.
Kazuo Sugimoto: nothing to disclose.
Jia Liu reports: nothing to disclose.
Klemens Ruprecht was supported by the German Ministry of Education and Research (BMBF/KKNMS, Competence Network Multiple Sclerosis) and has received research support from Novartis and Merck Serono as well as speaking fees and travel grants from Guthy Jackson Charitable Foundation, Bayer Healthcare, Biogen Idec, Merck Serono, sanofi-aventis/Genzyme, Teva Pharmaceuticals, Roche and Novartis.
Judith Bellmann-Strobl has received travel grants and speaking fees from Bayer Healthcare, Biogen Idec, Merck Serono, sanofi-aventis/Genzyme, Teva Pharmaceuticals, and Novartis.
Satoshi Kuwabara serves as academic editor for JNNP.
Friedemann Paul serves on the scientific advisory board for Novartis; received speaker honoraria and travel funding from Bayer, Novartis, Biogen Idec, Teva, Sanofi-Aventis/Genzyme, Merck Serono, Alexion, Chugai, MedImmune, and Shire; is an academic editor for PLoS ONE; is an associate editor for Neurology® Neuroimmunology & Neuroinflammation; consulted for SanofiGenzyme, Biogen Idec, MedImmune, Shire, and Alexion; and received research support from Bayer, Novartis, Biogen Idec, Teva, Sanofi-Aventis/Genzyme, Alexion, Merck Serono, German Research Council, Werth Stiftung of the City of Cologne, German Ministry of Education and Research, Arthur Arnstein Stiftung Berlin, EU FP7 Framework Program, Arthur Arnstein Foundation Berlin, Guthy Jackson Charitable Foundation, and National Multiple Sclerosis of the USA.

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