Clinical characteristics of 22 individual patients with MOG-IgG
Gender | Age at onset | Ethnicity | MOG-IgG1 CBA score | AQP4-IgG CBA score | Negative conversion of MOG-IgG at last follow-up | Onset phenotype | Phenotypes at last follow-up | Clinical diagnosis at last follow-up | No of attack | Current EDSS | OCB status | Other autoimmune serology | VEP/OCT | Brain MRI lesion localization | Follow-up duration (months) | Disease duration (months) | Current treatment | |
1 | F | 41 | K | 1 | K0 | No | O | O | Idiopathic AQP4-IgG negative ON | 6 | 3 | N | Antinuclear Ab(+), homogenous pattern | VEP: DPL | None | 49 | 49 | RTX |
2 | F | 8 | K | 3 | 0 | No | O(b)+B | O(b)+B | Seronegative NMOSD | 11 | 2 | N/A | Negative | OCT: RNFLT | SC, DGM | 194 | 195 | RTX |
3 | F | 46 | K | 3.5 | 0 | No | B | B | Seronegative NMOSD | 1 | 3 | N/A | Anti-SS-A Ab(+) | VEP: normal | PV | 34 | 50 | AZA |
4 | F | 30 | K | 3 | 0 | No | O | O+M (l)+B | Seronegative NMOSD | 4 | 1 | N | Antinuclear Ab(+), speckled pattern | OCT: RNFLT | JC, SC | 65 | 68 | AZA |
5 | F | 29 | K | 3 | 0 | No | O | O+B | Seronegative NMOSD | 4 | 2 | N | Negative | VEP: DPL, OCT: RNFLT | Bs | 30 | 53 | MMF |
6 | M | 50 | K | 1 | 0 | No | B | B+M | Other demyelinating syndrome | 2 | 2 | N | Negative | VEP: normal | SC | 32 | 34 | AZA |
7 | F | 35 | K | 3 | 0 | Yes | M | M+B | RRMS | 5 | 3 | P | Negative | VEP: normal | PV, JC, Bs | 106 | 107 | IFNβ |
8 | F | 24 | K | 2 | 0 | Yes | B | B+O (b)+M | Other demyelinating syndrome | 4 | 6 | P | Antinuclear Ab(+), speckled pattern | VEP: DPL | PV, SC | 200 | 201 | MMF |
9 | M | 42 | K | 1 | 0 | Yes | M(l) | M(l) | Idiopathic AQP4-IgG negative M | 5 | 6.5 | P | Negative | VEP: normal | None | 135 | 136 | AZA |
10 | M | 33 | K | 1 | 0 | Yes | O(b)+M | O(b)+M+B | Seronegative NMOSD | 3 | 1.5 | N | Negative | VEP: DPL, OCT: RNFLT | Bs | 94 | 95 | RTX |
11 | F | 29 | K | 2 | 0 | NA | O +M(l) | O +M(l) | Seronegative NMOSD | 1 | 0 | N | Negative | N/A | None | 7 | 126 | None |
12 | F | 4 | K | 2 | 0 | NA | O | O+B | Seronegative NMOSD | 16 | 3 | N | Negative | N/A | PV, Bs, DGM | 114 | 240 | AZA |
13 | F | 34 | K | 4 | 0 | NA | O(b) | O(b) | Idiopathic AQP4-IgG negative ON | 2 | 1 | N | Negative | VEP: DPL | None | 41 | 119 | None |
14 | F | 27 | K | 3 | 0 | NA | O | O | Idiopathic AQP4-IgG negative ON | 3 | 1 | P | Negative | VEP: DPL | None | 61 | 153 | None |
15 | F | 10 | K | 2 | 0 | NA | B | B | Other demyelinating syndrome | 2 | 0 | N/A | Negative | N/A | Bs, DGM | 23 | 128 | NA |
16 | M | 32 | K | 2 | 3.5 | NA | M(l) | M(l) +O | Seropositive NMOSD | 4 | 7 | N/A | Anti-SS-A Ab(+), antinuclear Ab(+), speckled pattern | VEP: DPL | None | 85 | 136 | AZA |
17 | M | 30 | K | 1 | 0 | NA | M(l) | M(l) | Idiopathic AQP4-IgG negative M | 1 | 0 | N/A | Negative | VEP: normal | None | 113 | 160 | None |
18 | F | 28 | K | 1 | 0.5 | NA | B | B+M(l) | Seronegative NMOSD | 2 | 5.5 | N | Anti-ds DNA Ab(+) | VEP: normal | PV, Bs | 71 | 105 | AZA |
19 | M | 30 | K | 2 | 0 | NA | M | M | Idiopathic AQP4-IgG negative M | 1 | 2.5 | P | Negative | VEP: normal | None | 44 | 68 | NA |
20 | F | 7 | K | 3 | 0 | NA | O | O+B | Seronegative NMOSD | 2 | 0 | N | Negative | VEP: DPL, OCT: RNFLT | SC | 28 | 28 | MMF |
21 | M | 31 | K | 3 | 0 | NA | M(l) | M(l) | Idiopathic AQP4-IgG negative M | 1 | 2.5 | N | Negative | VEP: normal | None | 23 | 24 | MMF |
22 | M | 21 | K | 3.5 | 0 | NA | O | O(b) | Idiopathic AQP4-IgG negative ON | 4 | 1 | N | Negative | VEP: DPL | None | 88 | 88 | MMF |
A score of less than 1 is negative, a score of 1–1.5 is considered borderline/low positive and above 1.5 is positive.
Ab, antibody; AQP4-IgG, aquaporin-4 immunoglobulin G; AZA, azathioprine; b, simultaneous bilateral; Bs, brainstem; CBA, cell-based assay; DGM, deep grey matter; DPL, delayed P100 latency; ds, double stranded; EDSS, Expanded Disability Status Scale; F, female; IFNβ; interferon-beta; JC, juxtacortical white matter; K, Korean; l, longitudinally extensive transverse myelitis; M, myelitis; M (gender), male; MMF, mycophenolate mofetil; MOG, myelin oligodendrocyte glycoprotein; N, negative; N/A, not available; NMOSD, neuromyelitis optica spectrum disorder; O/ON: optic neuritis; OCB, oligoclonal band; OCT, optical coherence tomography; P, positive; PV, periventricular white matter; RNFLT, retinal nerve fibre layer thinning; RRMS, relapsing remitting multiple sclerosis; RTX, rituximab; SC, subcortical white matter; VEP, visual evoked potential.