Journal
SCIENCE CHINA-LIFE SCIENCES
Volume 59, Issue 12, Pages 1270-1281Publisher
SCIENCE PRESS
DOI: 10.1007/s11427-015-4997-y
Keywords
MOG antibody; AQP4 antibody; neuromyelitis optica spectrum disorder; phenotype
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Funding
- Neuroimmunology team in Tianjin
- Neuroimmunology team in Barrow
- National Basic Research Program of China [2013CB96690]
- Natural Science Foundation of China [81100888, 81230028, 81371372]
- National Key Clinical Specialty Construction Program of China
- US National Institute of Health [R01AI083294]
- American Heart Association [14GRNT-18970031]
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We characterized a unique group of patients with neuromyelitis optica spectrum disorder (NMOSD) who carried autoantibodies of aquaporin-4 (AQP4) and myelin-oligodendrocyte glycoprotein (MOG). Among the 125 NMOSD patients, 10 (8.0%) were AQP4- and MOG-ab double positive, and 14 (11.2%) were MOG-ab single positive. The double-positive patients had a multiphase disease course with a high annual relapse rate (P=0.0431), and severe residual disability (P<0.0001). Of the double-positive patients, 70% had MS-like brain lesions, more severe edematous, multifocal regions on spinal magnetic resonance imaging (MRI), pronounced decreases of retinal nerve fiber layer thickness and atrophy of optic nerves. In contrast, patients with only MOG-ab had a higher ratio of monophasic disease course and mild residual disability. Spinal cord MRI illustrated multifocal cord lesions with mild edema, and brain MRIs showed more lesions around lateral ventricles. NMOSD patients carrying both autoantibodies to AQP4 and MOG existed and exhibited combined features of prototypic NMO and relapsing-remitting form of MS, whereas NMOSD with antibodies to MOG only exhibited an intermediate phenotype between NMOSD and MS. Our study suggests that antibodies against MOG might be pathogenic in NMOSD patients and that determination of anti-MOG antibodies maybe instructive for management of NMOSD patients.
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