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Human Serum Anti-aquaporin-4 Immunoglobulin G Detection by Cell-based Assay
Published on: April 5, 2019
Autoantibody to MOG suggests two distinct clinical subtypes of NMOSD
Yaping Yan1, Yujing Li2, Ying Fu2
1Departments of Neurology and Immunology, Tianjin Neurological Institute, Tianjin Medical University General Hospital, Tianjin, 300052, China. yaping.yan@tmu.edu.cn.
Abstract:
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.
Insights
Neuromyelitis optica spectrum disorder (NMOSD) patients with both aquaporin-4 (AQP4) and myelin-oligodendrocyte glycoprotein (MOG) autoantibodies showed severe disease. Those with only MOG autoantibodies had an intermediate phenotype between NMOSD and multiple sclerosis (MS).
Area of Science:
- Neuroimmunology
- Autoimmune neurological disorders
- Neuromyelitis Optica Spectrum Disorder (NMOSD)
Background:
- Neuromyelitis optica spectrum disorder (NMOSD) is a severe autoimmune condition affecting the central nervous system.
- Autoantibodies against aquaporin-4 (AQP4) and myelin-oligodendrocyte glycoprotein (MOG) are key biomarkers in NMOSD.
- The clinical and radiological phenotypes associated with dual positivity for AQP4 and MOG autoantibodies remain incompletely understood.
Purpose of the Study:
- To characterize the clinical and radiological features of NMOSD patients with co-existing AQP4 and MOG autoantibodies.
- To compare the disease course and disability in NMOSD patients with dual autoantibody positivity versus MOG autoantibody positivity alone.
- To investigate the potential pathogenic role of MOG autoantibodies in NMOSD.
Main Methods:
- Retrospective analysis of 125 NMOSD patients.
- Serological testing for AQP4 and MOG autoantibodies.
- Clinical data collection including disease course, relapse rate, and disability.
- Magnetic Resonance Imaging (MRI) analysis of brain and spinal cord lesions.
- Ophthalmic examination for retinal nerve fiber layer thickness and optic nerve atrophy.
Main Results:
- 10% of NMOSD patients were double-positive for AQP4 and MOG autoantibodies; 11.2% were MOG-ab single positive.
- Double-positive patients exhibited a multiphase disease course, high relapse rates, and severe disability, with MS-like brain lesions and severe spinal cord edema.
- MOG-ab single-positive patients showed a predominantly monophasic course, milder disability, distinct MRI lesion patterns (spinal cord edema, periventricular brain lesions), and less optic nerve damage.
Conclusions:
- NMOSD patients with both AQP4 and MOG autoantibodies present with a severe phenotype combining features of classic NMOSD and relapsing-remitting MS.
- NMOSD patients with MOG autoantibodies only display an intermediate phenotype between NMOSD and MS.
- The presence of MOG autoantibodies may indicate pathogenicity and influence disease management strategies in NMOSD.
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