Clinical characteristics of patients with MOGAD included in the study
| n (%) | All patients 102 (100) | Monophasic 58 (56.9) | Relapsing 44 (43.1) | Adults 58 (56.9) | Paediatric 44 (43.1) | P value (mono vs relapsing) | P value (adults vs paediatric) |
| Sex, male, n (%) | 43 (42.2) | 25 (58.1) | 18 (41.9) | 23 (39.7) | 20 (45.5) | 0.84 | 0.67 |
| Race, white n (%) | 97 (95.1) | 55 (94.8) | 42 (95.5) | 57 (98.3) | 40 (90.9) | 0.66 | 0.21 |
| Age at onset (years), median (IQR, range) | 17 (6–33) | 16 (5–33, 1–69) | 19 (8–31, 2–72) | 38 (31–50, 19–72) | 6 (3–12, 1–16) | 0.15* | <0.001 |
| Follow-up (months), median (IQR, range) | 29 (15–52, 3-232) | 27 (15–46, 3-151) | 36.5 (17–93, 3-232) | 29 (31–50, 3–208) | 28 (15–53, 5–232) | 0.16 | 0.55 |
| Relapsing course, n (%) | 44 (43.1) | – | – | 28 (48.3) | 16 (36.4) | – | 0.31 |
| MOG-IgG seroconversion to negative, n (%) | 41 (40.2) | 25 (43.1) | 16 (36.4) | 22 (37.9) | 19 (43.2) | 0.55 | 0.68 |
| Clinical phenotype at onset, n (%) | |||||||
| ON | 46 (45.1) | 28 (48.3) | 18 (40.9) | 29 (50.0) | 17 (38.6) | 0.58 | <0.001 |
| TM | 23 (22.5) | 10 (17.2) | 13 (29.5) | 20 (34.5) | 3 (6.8) | ||
| ON+TM | 5 (4.9) | 4 (6.9) | 1 (2.3) | 35.2) | 2 (4.5) | ||
| ADEM/ADEM-like | 26 (25.5) | 15 (25.9) | 11 (25.0) | 4 (6.9) | 22 (50.0) | ||
| Other† | 2 (2.0) | 1 (1.7) | 1 (2.3) | 2 (3.4) | 0 (0.0) | ||
| EDSS score at nadir ≥3 n/tot (%) | 30/91 (32.9) | 29/52 (55.8) | 20/39 (51.3) | 25/52 (48.1) | 24/39 (61.5) | 0.53 | 0.01 |
| Outcome after the onset event, n (%) | |||||||
| Complete recovery | 58 (56.9) | 29 (50) | 29 (65.9) | 30 (51.7) | 28 (63.6) | 0.01 | <0.001 |
| Partial recovery | 30 (29.4) | 16 (27.6) | 14 (31.8) | 25 (43.1) | 5 (11.4) | ||
| No recovery | 14 (13.7) | 13 (22.4) | 1 (2.3) | 3 (5.2) | 11 (25.0) | ||
| Patients treated with onset attack treatment, n (%) | 98 (96.1) | 55 (94.8) | 43 (97.7) | 55 (94.8) | 43 (97.7) | 0.63 | 0.63 |
| Oral steroids >1 month‡ | 91 (89.2) | 52 (89.7) | 39 (88.6) | 49 (84.5) | 42 (95.5) | 1.0 | 0.11 |
| Intravenous steroids | 59 (57.8) | 39 (67.2) | 20 (45.5) | 23 (39.7) | 36 (81.1) | 0.04 | <0.001 |
| Intravenous immunoglobulin | 12 (11.8) | 7 (12.1) | 5 (11.4) | 4 (6.9) | 8 (18.2) | 1.0 | 0.12 |
| Plasma exchange | 3 (2.9) | 1 (1.7) | 2 (4.5) | 3 (5.2) | 0 (0.0) | 0.58 | 0.26 |
| Patients treated with second line treatment, n (%) | 38 (37.3) | 16 (27.6) | 22 (50.0) | 30 (51.7) | 8 (18.2) | 0.02 | 0.001 |
| Azathioprine | 22 (21.6) | 11 (19.0) | 11 (25.0) | 20 (34.5) | 2 (4.5) | 0.48 | <0.001 |
| Rituximab | 12 (11.8) | 3 (5.2) | 9 (20.5) | 7 (12.1) | 5 (11.4) | 0.28 | 1.00 |
| Others | 7 (6.9) | 3 (5.2) | 4 (9.1) | 0.46 | |||
| EDSS score ≥3 at last follow-up, n/tot (%) | 7/95 (7.4) | 3/55 (5.5) | 4/40 (10.0) | 5/53 (9.4) | 2/42 (4.8) | 0.64 | 0.67 |
| Final diagnosis, n (%) | |||||||
| Monolateral ON | 20 (19.6) | 12 (20.7) | 8 (18.2) | 14 (24.1) | 6 (13.6) | 0.06 | <0.001 |
| Bilateral ON | 18 (17.6) | 13 (22.4) | 5 (11.4) | 10 (17.2) | 8 (18.2) | ||
| TM (short) | 7 (6.9) | 5 (8.6) | 2 (4.5) | 7 (12.1) | 0 (0.0) | ||
| LETM | 4 (3.9) | 3 (5.2) | 1 (2.3) | 4 (6.9) | 0 (0.0) | ||
| ADEM/ADEM-like | 28 (27.5) | 18 (31.0) | 10 (22.7) | 6 (10.3) | 22 (50.0) | ||
| NMOSD | 16 (15.7) | 4 (6.9) | 12 (27.3) | 11 (19.0) | 5 (11.4) | ||
| Others§ | 9 (8.8) | 3 (5.2) | 6 (13.6) | 6 (10.3) | 3 (6.8) | ||
*Mann-Whitney test.
†Other neurological presentations at onset in two patients included one with brainstem and cervical lesions and one with a pseudotumoral brain lesion and seizures.
‡Including gradual tapering of the dose of oral steroids.
§Other final diagnosis in nine patients included four patients with a combination of brain and spinal cord lesions, one patient with a bilateral ON and a single corpus callosum lesion, one with an ON and a cerebellar lesion, one with combined central and peripheral demyelination.
ADEM, acute disseminated encephalomyelitis; EDSS, Expanded Disability Status Sale; LETM, longitudinally extensive transverse myelitis; MOG-IgG, IgG antibodies against myelin oligodendrocyte glycoprotein; NMOSD, neuromyelitis optica spectrum disorder; ON, optic neuritis; TM, transverse myelitis.