Claudia Chien1, Michael Scheel2, Tanja Schmitz-Hübsch1, Nadja Borisow3, Klemens Ruprecht4, Judith Bellmann-Strobl5, Friedemann Paul6, Alexander U Brandt7. 1. NeuroCure Clinical Research Center, Charité - Universitätsmedizin Berlin, Berlin, Germany. 2. NeuroCure Clinical Research Center, Charité - Universitätsmedizin Berlin, Berlin, Germany/Department of Neuroradiology, Charité - Universitätsmedizin Berlin, Berlin, Germany. 3. NeuroCure Clinical Research Center, Charité - Universitätsmedizin Berlin, Berlin, Germany/Department of Neurology, Charité - Universitätsmedizin Berlin, Berlin, Germany. 4. Department of Neurology, Charité - Universitätsmedizin Berlin, Berlin, Germany. 5. 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. 6. NeuroCure Clinical Research Center, Charité - Universitätsmedizin Berlin, Berlin, Germany/ Department of Neurology, Charité - Universitätsmedizin Berlin, Berlin, Germany/ Experimental and Clinical Research Center, Max Delbrück Center for Molecular Medicine and Charité -Universitätsmedizin Berlin, Berlin, Germany. 7. NeuroCure Clinical Research Center, Charité - Universitätsmedizin Berlin, Berlin, Germany/Department of Neurology, University of California, Irvine, CA, USA.
Abstract
BACKGROUND: Spinal cord (SC) affection is a hallmark symptom of neuromyelitis optica spectrum disorders (NMOSD). Patients with aquaporin-4 (AQP4-IgG+) or myelin oligodendrocyte glycoprotein (MOG-IgG+) antibody seropositivity show this overlapping clinical phenotype. OBJECTIVE: Quantitative comparison of SC lesions and atrophy in AQP4-IgG+ and MOG-IgG+ NMOSD. METHODS: AQP4-IgG+ (n = 38), MOG-IgG+ (n = 15) NMOSD patients and healthy controls (HC, n = 24) were analysed for SC lesion (prevalence, length, location), atrophy as mean upper cervical cord area (MUCCA), Expanded Disability Status Scale (EDSS), timed 25-foot walk speed (T25FWS) and 9-hole peg test (9HPT) measures. RESULTS: In total, 92% (35/38) of AQP4-IgG+ and 53% (8/15) of MOG-IgG+ patients had myelitis attacks (χ2 = 6.47, p = 0.011). 65.8%/26.7% of AQP4-/MOG-IgG+ patients had chronic SC lesions (χ2 = 5.16, p = 0.023), with similar proportions in cervical, upper thoracic and lower thoracic cord, and no length differences. MUCCA was decreased in AQP4-IgG+ (t = -2.27, p = 0.028), but not MOG-IgG+ patients (t = 0.58, p = 0.57) compared to HC. MUCCA associated with myelitis attacks (rho = -0.33, p = 0.016), EDSS (rho = -0.31, p = 0.030), pyramidal functional score (rho = -0.42, p = 0.003), T25FWS (r = 0.43, p = 0.010) and 9HPT Z-score (r = 0.32, p = 0.037), regardless of antibody status. CONCLUSION: AQP4-IgG+ patients had more myelitis attacks, SC lesions and SC atrophy was more pronounced than in MOG-IgG+ patients. MUCCA is associated with clinical myelitis attacks and disability in all NMOSD patients.
BACKGROUND: Spinal cord (SC) affection is a hallmark symptom of neuromyelitis optica spectrum disorders (NMOSD). Patients with aquaporin-4 (AQP4-IgG+) or myelin oligodendrocyte glycoprotein (MOG-IgG+) antibody seropositivity show this overlapping clinical phenotype. OBJECTIVE: Quantitative comparison of SC lesions and atrophy in AQP4-IgG+ and MOG-IgG+ NMOSD. METHODS:AQP4-IgG+ (n = 38), MOG-IgG+ (n = 15) NMOSD patients and healthy controls (HC, n = 24) were analysed for SC lesion (prevalence, length, location), atrophy as mean upper cervical cord area (MUCCA), Expanded Disability Status Scale (EDSS), timed 25-foot walk speed (T25FWS) and 9-hole peg test (9HPT) measures. RESULTS: In total, 92% (35/38) of AQP4-IgG+ and 53% (8/15) of MOG-IgG+ patients had myelitis attacks (χ2 = 6.47, p = 0.011). 65.8%/26.7% of AQP4-/MOG-IgG+ patients had chronic SC lesions (χ2 = 5.16, p = 0.023), with similar proportions in cervical, upper thoracic and lower thoracic cord, and no length differences. MUCCA was decreased in AQP4-IgG+ (t = -2.27, p = 0.028), but not MOG-IgG+ patients (t = 0.58, p = 0.57) compared to HC. MUCCA associated with myelitis attacks (rho = -0.33, p = 0.016), EDSS (rho = -0.31, p = 0.030), pyramidal functional score (rho = -0.42, p = 0.003), T25FWS (r = 0.43, p = 0.010) and 9HPT Z-score (r = 0.32, p = 0.037), regardless of antibody status. CONCLUSION:AQP4-IgG+ patients had more myelitis attacks, SC lesions and SC atrophy was more pronounced than in MOG-IgG+ patients. MUCCA is associated with clinical myelitis attacks and disability in all NMOSD patients.
Authors: Frederike Cosima Oertel; Svenja Specovius; Hanna G Zimmermann; Claudia Chien; Seyedamirhosein Motamedi; Charlotte Bereuter; Lawrence Cook; Marco Aurélio Lana Peixoto; Mariana Andrade Fontanelle; Ho Jin Kim; Jae-Won Hyun; Jacqueline Palace; Adriana Roca-Fernandez; Maria Isabel Leite; Srilakshmi Sharma; Fereshteh Ashtari; Rahele Kafieh; Alireza Dehghani; Mohsen Pourazizi; Lekha Pandit; Anitha D'Cunha; Orhan Aktas; Marius Ringelstein; Philipp Albrecht; Eugene May; Caryl Tongco; Letizia Leocani; Marco Pisa; Marta Radaelli; Elena H Martinez-Lapiscina; Hadas Stiebel-Kalish; Sasitorn Siritho; Jérome de Seze; Thomas Senger; Joachim Havla; Romain Marignier; Alvaro Cobo-Calvo; Denis Bichuetti; Ivan Maynart Tavares; Nasrin Asgari; Kerstin Soelberg; Ayse Altintas; Rengin Yildirim; Uygur Tanriverdi; Anu Jacob; Saif Huda; Zoe Rimler; Allyson Reid; Yang Mao-Draayer; Ibis Soto de Castillo; Axel Petzold; Ari J Green; Michael R Yeaman; Terry Smith; Alexander U Brandt; Friedemann Paul Journal: Neurol Neuroimmunol Neuroinflamm Date: 2021-09-15