Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders

Abstract

Objective: To evaluate clinical features among patients with neuromyelitis optica spectrum disorders (NMOSD) who have myelin oligodendrocyte glycoprotein (MOG) antibodies, aquaporin-4 (AQP4) antibodies, or seronegativity for both antibodies.

Methods: Sera from patients diagnosed with NMOSD in 1 of 3 centers (2 sites in Brazil and 1 site in Japan) were tested for MOG and AQP4 antibodies using cell-based assays with live transfected cells.

Results: Among the 215 patients with NMOSD, 7.4% (16/215) were positive for MOG antibodies and 64.7% (139/215) were positive for AQP4 antibodies. No patients were positive for both antibodies. Patients with MOG antibodies represented 21.1% (16/76) of the patients negative for AQP4 antibodies. Compared with patients with AQP4 antibodies or patients who were seronegative, patients with MOG antibodies were more frequently male, had a more restricted phenotype (optic nerve more than spinal cord), more frequently had bilateral simultaneous optic neuritis, more often had a single attack, had spinal cord lesions distributed in the lower portion of the spinal cord, and usually demonstrated better functional recovery after an attack.

Conclusions: Patients with NMOSD with MOG antibodies have distinct clinical features, fewer attacks, and better recovery than patients with AQP4 antibodies or patients seronegative for both antibodies.

Figure 1. Bilateral optic neuritis in a patient with MOG antibodies

Axial short TI inversion recovery (STIR) and T1-weighted with gadolinium MRI of the optic nerves from a 28-year-old man with bilateral simultaneous optic neuritis and myelin oligodendrocyte glycoprotein (MOG) antibodies (titer 1:4,096) shows bilateral STIR hyperintense lesions with contrast enhancement.

Figure 2. Recurrent longitudinally extensive transverse myelitis in a patient with MOG antibodies

Sagittal T2-weighted MRI of the spinal cord from a 6-year-old girl with recurrent longitudinally extensive transverse myelitis and myelin oligodendrocyte glycoprotein (MOG) antibodies (titer 1:1,024) shows 2 longitudinally extensive lesions. On T1-weighted sagittal sequence, the lesion in the thoracolumbar transition shows a ring contrast enhancement.