Cognition in patients with neuromyelitis optica spectrum disorders: A prospective multicentre study of 217 patients (CogniNMO-Study)
Martin W. Hümmert, Carlotta Stern, Friedemann Paul, Ankelien Duchow, Judith Bellmann–Strobl, Ilya Ayzenberg, Carolin Schwake, Ingo Kleiter, Kerstin Hellwig, Sven Jarius, Brigitte Wildemann, Makbule Şenel, Achim Berthele, Katrin Giglhuber, Felix Luessi, Matthias Grothe, Luisa Klotz, Rasmus Schülke, Stefan Gingele, Jürgen Faiss, Annette O. Walter, Clemens Warnke, Florian Then Bergh, Orhan Aktaş, Marius Ringelstein, Jan‐Patrick Stellmann, Vivien Häußler, Joachim Havla, Hannah Pellkofer, Tania Kümpfel, Bruno Kopp, Corinna Trebst
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 ( p Bon < 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.