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Multicenter Study
. 2023 Jun;29(7):819-831.
doi: 10.1177/13524585231151212. Epub 2023 Feb 14.

Cognition in patients with neuromyelitis optica spectrum disorders: A prospective multicentre study of 217 patients (CogniNMO-Study)

Martin W Hümmert  1 , Carlotta Stern  1 , Friedemann Paul  2 , Ankelien Duchow  3 , Judith Bellmann-Strobl  3 , Ilya Ayzenberg  4 , Carolin Schwake  4 , Ingo Kleiter  5 , Kerstin Hellwig  4 , Sven Jarius  6 , Brigitte Wildemann  6 , Makbule Senel  7 , Achim Berthele  8 , Katrin Giglhuber  8 , Felix Luessi  9 , Matthias Grothe  10 , Luisa Klotz  11 , Rasmus Schülke  12 , Stefan Gingele  1 , Jürgen H Faiss  13 , Annette Walter  14 , Clemens Warnke  15 , Florian Then Bergh  16 , Orhan Aktas  17 , Marius Ringelstein  18 , Jan-Patrick Stellmann  19 , Vivien Häußler  20 , Joachim Havla  21 , Hannah Pellkofer  22 , Tania Kümpfel  22 , Bruno Kopp  1 , Corinna Trebst  1   23
Affiliations
Multicenter Study

Cognition in patients with neuromyelitis optica spectrum disorders: A prospective multicentre study of 217 patients (CogniNMO-Study)

Martin W Hümmert et al. Mult Scler. 2023 Jun.

Abstract

Background: There is limited and inconsistent information on the prevalence of cognitive impairment in neuromyelitis optica spectrum disorders (NMOSD).

Objective: To assess cognitive performance and changes over time in NMOSD.

Methods: This study included data from 217 aquaporin-4-IgG-seropositive (80%) and double-seronegative NMOSD patients. Cognitive functions measured by Symbol Digit Modalities Test (SDMT), Paced Auditory Serial-Addition Task (PASAT), and/or Multiple Sclerosis Inventory Cognition (MuSIC) were standardized against normative data (N = 157). Intraindividual cognitive performance at 1- and 2-year follow-up was analyzed. Cognitive test scores were correlated with demographic and clinical variables and assessed with a multiple linear regression model.

Results: NMOSD patients were impaired in SDMT (p = 0.007), MuSIC semantic fluency (p < 0.001), and MuSIC congruent speed (p < 0.001). No significant cognitive deterioration was found at follow-up. SDMT scores were related to motor and visual disability (pBon < 0.05). No differences were found between aquaporin-4-IgG-seropositive and double-seronegative NMOSD.

Conclusions: A subset of NMOSD patients shows impairment in visual processing speed and in semantic fluency regardless of serostatus, without noticeable changes during a 2-year observation period. Neuropsychological measurements should be adapted to physical and visual disabilities.

Keywords: Neuromyelitis optica spectrum disorders; cognition; cognitive neuropsychology; neuroimmunology.

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Conflict of interest statement

The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: MWH reports no disclosures relevant to the manuscript. CSt reports no disclosures relevant to the manuscript. FP receives honoraria for lecturing, and travel expenses for attending meetings from Guthy Jackson Foundation, Sanofi Genzyme, Novartis, Alexion, Viela Bio, Roche, UCB, Mitsubishi Tanabe, and Celgene. His research is funded by the German Ministry for Education and Research (BMBF), Deutsche Forschungsgemeinschaft (DFG), Einstein Foundation, Guthy Jackson Charitable Foundation, EU FP7 Framework Program, Biogen, Genzyme, Merck Serono, Novartis, Bayer, Teva, Alexion, Roche, Parexel, Viela Bio, and Almirall. FP serves on advisory boards and steering committees for Novartis and Viela Bio and is Associate Editor of Neurology, Neuroimmunology & Neuroinflammation and Academic Editor for PLoS ONE. AD reports no disclosures relevant to the manuscript. JBS has received travel grants and speaking honoraria from Bayer Healthcare, Biogen Idec, Merck Serono, Sanofi Genzyme, Teva Pharmaceuticals, Roche, and Novartis all unrelated to this work. IA received personal fees from Roche, Alexion, and Merck and received research support from Diamed, none related to this manuscript. CSc reports no disclosures relevant to the manuscript. IK has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Alexion, Biogen, Celgene, Hexal, Horizon, Merck, and Roche/Chugai. KH received consultant and speaker honoraria from Bayer, Biogen, Merck, Novartis, Sanofi Genzyme, Roche, and Teva. SJ reports no disclosures relevant to the manuscript. BW received grants from the German Ministry of Education and Research, Deutsche Forschungsgemeinschaft, Dietmar Hopp Foundation, and Klaus Tschira Foundation, grants and personal fees from Merck, Novartis, and personal fees from Roche; none related to this work. MS has received consulting and/or speaker honoraria from Alexion, Bayer, Biogen, Merck, Roche, and Sanofi Genzyme. She has received research funding from the Hertha-Nathorff-Program. None of this interfered with the current report. AB received speaker and consulting honoraria from Alexion, Biogen, Bayer Healthcare, Celgene, Merck, Novartis Pharma, and Roche; all outside the submitted work. KG reports no disclosures relevant to the manuscript. FL received consultancy fees from Roche and support with travel cost from Teva Pharma. MG received honoraria and travel reimbursements for attending meetings from Biogen, Celgene, Merck Serono, Novartis, Roche, Sanofi-Genzyme, and Teva and research grants from the German Ministry for Education and Research (BMBF), Merck Serono, and Novartis. None of this interfered with the current report. LK received compensation for serving on Scientific Advisory Boards for Alexion, Biogen, Celgene GmbH, Genzyme, Horizon, Janssen, Merck Serono, Novartis, and Roche. She received speaker honoraria and travel support from Bayer, Biogen, Celgene GmbH, Genzyme, Grifols, Merck Serono, Novartis, Roche, Santhera, and Teva. She receives research support from the German Research Foundation, the IZKF Münster, IMF Münster, Biogen, Immunic AG, Novartis, and Merck Serono. RS reports no disclosures relevant to the manuscript. SG has received speaker honoraria from Alnylam, not related to this manuscript. JHF reports no disclosures relevant to the manuscript. AW received speaker honoraria and meeting expenses from Novartis, Bayer, Biogen, Sanofi Genzyme, Teva, Roche, and Merck. CW has received institutional honoraria and/or grant support from Novartis, Sanofi-Genzyme, Alexion, Janssen, Merck, Biogen, and Roche. FTB has received honoraria for speaking and advisory board consultation from Alexion, Roche, and Horizon Therapeutics; none of these had an impact on this manuscript. OA has received personal fees from Alexion, Bayer Healthcare, Biogen, Celgene, Merck Serono, MedImmune, Novartis, Roche, Teva, and Zambon, outside of the submitted work. MR received speaker honoraria from Novartis, Bayer Vital GmbH, Roche, Alexion, and Ipsen and travel reimbursement from Bayer Schering, Biogen Idec, Merz, Genzyme, Teva, Roche, and Merck, none related to this study. JPS reports no disclosures relevant to the manuscript. VH reports no disclosures relevant to the manuscript. JH reports personal fees, research grants and non-financial support from Merck, Novartis, Roche, Santhera, Biogen, Alexion, Celgene, Janssen; and non-financial support of the Guthy-Jackson Charitable Foundation, all outside the submitted work. J.H. is (partially) funded by the German Federal Ministry of Education and Research [Grant Numbers 01ZZ1603[A-D] and 01ZZ1804[A-H] (DIFUTURE)]. HP received honoraria for lectures from Bayer Health Care, Biogen Idec, and Teva Pharma and travel reimbursement from Novartis. TK has received speaker honoraria and/or personal fees for advisory boards from Bayer Healthcare, Teva Pharma, Merck, Novartis Pharma, Sanofi-Aventis/Genzyme, Roche Pharma, Alexion, and Biogen as well as grant support from Novartis and Chugai Pharma in the past. None of this interfered with the current report. BK reports no disclosures relevant to the manuscript. CT has received honoraria for consultation and expert testimony from Alexion Pharma Germany GmbH, Biogen Idec/GmbH, Chugai Pharma Germany GmbH, MERCK, Novartis Pharma GmbH and Roche Pharma GmbH. None of this interfered with the current report.

Figures

Figure 1.
Figure 1.
CogniNMO-Study—Selection procedure and cohort characterization stratified by serostatus. Note: Age and disease duration are shown as median with range in years, female sex is shown as percentage. Patient subgroups were defined based on serostatus. EDSS values of 30 patients were missing. This did not affect the representativeness of the group composition. AQP4-IgG + = aquaporin-4 immunoglobulin G antibody-positive patients; Double seronegative = AQP4- and MOG-antibody negative NMOSD patients; EDSS = Expanded Disability Status Scale (score is shown as median with range); IPND = International Panel for Neuromyelitis Optica (NMO) Diagnosis; MOG(AD) = myelin oligodendrocyte glycoprotein antibody (associated disease); NMOSD = neuromyelitis optica spectrum disorders.
Figure 2.
Figure 2.
Mean z scores of cognitive tests at baseline. Note: Patients ⩾ 60 years were excluded. CI = confidence interval; PASAT = Paced Auditory Serial-Addition Task; SDMT = Symbol Digit Modalities Test.
See this image and copyright information in PMC

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