Maureen A Mealy1, Sarah E Mossburg2, Su-Hyun Kim3, Silvia Messina4, Nadja Borisow5, Reydmar Lopez-Gonzalez6, Juan Pablo Ospina7, Michael Scheel5, Anusha K Yeshokumar8, Amine Awad4, M Isabel Leite4, JorgeA Jimenez Arango6, Friedemann Paul5, Jacqueline Palace4, Ho Jin Kim3, Michael Levy7. 1. Johns Hopkins University, School of Medicine, Department of Neurology, Baltimore, MD, USA; Johns Hopkins University School of Nursing, Baltimore, MD, USA. Electronic address: mmealy1@jhmi.edu. 2. Johns Hopkins University School of Nursing, Baltimore, MD, USA. 3. Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, South Korea. 4. Nuffield Department of Clinical Neurosciences, West Wing, John Radcliffe Hospital, University of Oxford, Oxford, UK. 5. Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, NeuroCure Clinical Research Center and Department of Neurology, Berlin, Germany; Experimental and Clinical Research Center, Max Delbrueck Center for Molecular Medicine, Berlin, Germany. 6. University of Antioquia, Department of Neurology, Neuroclinica, Medellín, CO, Colombia. 7. Johns Hopkins University, School of Medicine, Department of Neurology, Baltimore, MD, USA. 8. Johns Hopkins University, School of Medicine, Department of Neurology, Baltimore, MD, USA; Icahn School of Medicine at Mount Sinai, Department of Neurology, New York, NY.
Abstract
BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease of the central nervous system (CNS) that preferentially targets the spinal cord and optic nerves. Increasing disability is accrued with each inflammatory attack. Disability has been shown to be an independent predictor of poor quality of life in those with NMOSD. Factors associated with increasing disability need further systematic investigation. METHODS: We performed a multi-center retrospective chart analysis of aquaporin-4 (AQP4) seropositive NMOSD patients with a history of myelitis seen at five large referral centers for patients with NMOSD worldwide for whom thorough records including relapse history and corresponding imaging were available. Potential contributors to long-term disability were extracted including demographics, radiographic findings, and clinical characteristics. Multivariable regression modeling was conducted to determine correlates of disability in patients with NMOSD, as measured by the Expanded Disability Status Scale (EDSS). RESULTS: One hundred eighty-two AQP4 seropositive patients (88% female) were included in this analysis. Multiple regression modeling revealed that older age at disease onset, delay in diagnosis/preventive treatment, length of longest acute myelitis lesion and presence of symptomatic brain/brainstem lesions were associated with increased disability when holding other variables constant. CONCLUSION: While age at onset is a factor that cannot be controlled in NMOSD, we can reduce the delay in diagnosis/preventive treatment and reduce future relapses in the brain/brainstem and spinal cord. Delay in diagnosis/preventive treatment and imaging variables that contributed to increased disability support the need for improved measures for early, accurate diagnosis and management of NMOSD, and aggressive treatment of acute relapses.
BACKGROUND:Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease of the central nervous system (CNS) that preferentially targets the spinal cord and optic nerves. Increasing disability is accrued with each inflammatory attack. Disability has been shown to be an independent predictor of poor quality of life in those with NMOSD. Factors associated with increasing disability need further systematic investigation. METHODS: We performed a multi-center retrospective chart analysis of aquaporin-4 (AQP4) seropositive NMOSDpatients with a history of myelitis seen at five large referral centers for patients with NMOSD worldwide for whom thorough records including relapse history and corresponding imaging were available. Potential contributors to long-term disability were extracted including demographics, radiographic findings, and clinical characteristics. Multivariable regression modeling was conducted to determine correlates of disability in patients with NMOSD, as measured by the Expanded Disability Status Scale (EDSS). RESULTS: One hundred eighty-two AQP4 seropositive patients (88% female) were included in this analysis. Multiple regression modeling revealed that older age at disease onset, delay in diagnosis/preventive treatment, length of longest acute myelitis lesion and presence of symptomatic brain/brainstem lesions were associated with increased disability when holding other variables constant. CONCLUSION: While age at onset is a factor that cannot be controlled in NMOSD, we can reduce the delay in diagnosis/preventive treatment and reduce future relapses in the brain/brainstem and spinal cord. Delay in diagnosis/preventive treatment and imaging variables that contributed to increased disability support the need for improved measures for early, accurate diagnosis and management of NMOSD, and aggressive treatment of acute relapses.
Authors: Yara Dadalti Fragoso; Heloisa Helena Ruocco; Ronaldo Maciel Dias; Hideraldo Cabeça; Ricardo Gonçalves; Nise A de Carvalho Sousa; Caroline Vieira Spessotto; Carlos Bernardo Tauil; Soniza Vieira Alves-Leon; Sidney Gomes; Marcus Vinicius M Gonçalves; Suzana C Nunes Machado; Andrea Anacleto; Eber Castro Correa; Maria Lucia V Pimentel; Gutemberg Augusto C Santos Journal: Neurol Ther Date: 2019-07-02
Authors: Romain Marignier; Jeffrey L Bennett; Ho Jin Kim; Brian G Weinshenker; Sean J Pittock; Dean Wingerchuk; Kazuko Fujihara; Friedemann Paul; Gary R Cutter; Ari J Green; Orhan Aktas; Hans-Peter Hartung; Fred D Lublin; Ian M Williams; Jorn Drappa; Dewei She; Daniel Cimbora; William Rees; Michael Smith; John N Ratchford; Eliezer Katz; Bruce A C Cree Journal: Neurol Neuroimmunol Neuroinflamm Date: 2021-03-26
Authors: C Restrepo-Aristizábal; L M Giraldo; Y M Giraldo; A M Pino-Pérez; F Álvarez-Gómez; C A Franco; J V Tobón; J L Ascencio; M I Zuluaga Journal: Heliyon Date: 2021-04-17