Table 1.
Clinical and laboratory characteristics of Japanese patients with MS and NMOSD.
| MS (n = 528) | NMOSD (n = 165) | p | |
|---|---|---|---|
| Female | 368/528 (69.7) | 143/165 (86.7) | < 0.001 |
| Age at registration, y | 41 [34–49] | 53 [44–63] | < 0.001 |
| Course (RR/SP/PP) | 438/70/19 (83.1/13.3/3.6) | – | – |
| Age at onset, y | 30 [23–38] | 45 [33–54] | < 0.001 |
| Duration of disease, y | 9.1 [4.6–15.1] | 7.0 [3.4–12.8] | 0.004 |
| Number of relapses | 2 [1–4] | 2 [1–5] | 0.257 |
| Annualized relapse rate | 0.26 [0.13–0.55] | 0.41 [0.21–0.75] | 0.001 |
| Patients with family history of MS or NMOSD | 9/517 (1.7) | 5/161 (3.1) | 0.339 |
| Lesion sites | |||
| Optic nerve (optic neuritis) | 191/519 (36.8) | 98/164 (59.8) | < 0.001 |
| Cerebrum | 345/521 (66.2) | 39/163 (23.9) | < 0.001 |
| Cerebellum | 109/517 (21.1) | 14/163 (8.6) | < 0.001 |
| Brain stem | 254/517 (49.1) | 63/163 (38.7) | 0.020 |
| Spinal cord | 396/520 (76.2) | 138/164 (84.2) | 0.031 |
| CSF IgG abnormality | 309/440 (70.2) | 55/137 (40.2) | < 0.001 |
| Presence of OCBs | 262/411 (63.7) | 20/118 (17.0) | < 0.001 |
| High IgG index (> 0.658) | 212/374 (56.7) | 46/122 (37.7) | < 0.001 |
| Anti-AQP4 Ab positivity | 4/454 (0.9) | 155/163 (95.1) | < 0.001 |
| EDSS score | 2.0 [1.0–4.0] | 3.5 [2.0–5.5] | < 0.001 |
| PI | 0.22 [0.07–0.49] | 0.42 [0.23–0.94] | < 0.001 |
| MSSS | 2.65 [0.67–5.78] | – | – |
| FS score* | |||
| Visual functions | 0 [0–1] | 0 [0–5] | < 0.001 |
| Brainstem functions | 0 [0–2] | 0 [0–1] | 0.015 |
| Pyramidal functions | 1 [0–3] | 2 [0–3] | 0.001 |
| Cerebellar functions | 0 [0–2] | 0 [0–1] | < 0.001 |
| Sensory functions | 0 [0–2] | 1 [0–4] | < 0.001 |
| Bowel and bladder functions | 0 [0–1] | 0 [0–2] | 0.010 |
| Cerebral functions | 0 [0–1] | 0 [0–0] | 0.050 |
| Episodes | |||
| Bilateral optic neuritis | 27/516 (5.2) | 29/160 (18.1) | < 0.001 |
| Transverse myelitis | 41/513 (8.0) | 64/163 (39.3) | < 0.001 |
| MRI | |||
| Huge brain lesion > 3 cm | 8/522 (1.5) | 7/165 (4.2) | 0.060 |
| LESCL | 10/523 (1.9) | 93/162 (57.4) | < 0.001 |
| Benign course | |||
| EDSS ≤ 2 and disease duration ≥ 10 y | 101/234 (43.2) | 9/50 (18.0) | 0.001 |
| EDSS ≤ 3 and disease duration ≥ 10 y | 127/234 (54.3) | 13/50 (26.0) | < 0.001 |
| AID/autoantibodies | 77/494 (15.6) | 64/155 (41.3) | < 0.001 |
| AID | 31/491 (6.3) | 23/151 (15.2) | 0.001 |
| Sjögren syndrome | 7/491 (1.4) | 16/151 (10.6) | < 0.001 |
| Thyroid diseases (Basedow, Hashimoto) | 15/491 (3.1) | 3/151 (2.0) | 0.586 |
| Autoantibodies | 66/484 (13.6) | 60/148 (40.5) | < 0.001 |
| ANA | 26/484 (5.4) | 29/148 (19.6) | < 0.001 |
| Anti-SS-A/SS-B Ab | 14/484 (2.9) | 31/148 (21.0) | < 0.001 |
| Anti-Tg/TPO/TSHR Ab | 26/484 (5.4) | 14/148 (9.5) | 0.083 |
| PSL/IS | 69/526 (13.1) | 162/165 (98.2) | < 0.001 |
| PSL | 57/509 (11.2) | 157/163 (96.3) | < 0.001 |
| IS | 24/516 (4.7) | 111/164 (67.7) | < 0.001 |
| DMD | 459/528 (86.9) | 27/165 (16.4) | < 0.001 |
| IFN-β | 361/528 (68.4) | 27/165 (16.4) | < 0.001 |
| Fingolimod | 217/525 (41.3) | 1/164 (0.6) | < 0.001 |
Bold values indicate significant differences.
Values are median [interquartile ranges] or count (%).
Ab, antibody, AID autoimmune disease, ANA, anti-nuclear antibody, AQP4 aquaporin 4, CSF cerebrospinal fluid, EDSS, expanded disability status scale, FS functional system, IFN interferon, IS immunosuppressants, LESCL longitudinally extensive spinal cord lesion, MS multiple sclerosis, MSSS multiple sclerosis severity score, NMOSD neuromyelitis optica spectrum disorders, OCBs oligoclonal IgG bands, PI progression index, PP primary progressive, PSL prednisolone, RR, relapsing–remitting, SP secondary progressive, Tg thyroglobulin, TPO thyroid peroxidase, TSHR thyroid stimulating hormone receptor, y years.
*In the section of functional system scores, values are median [10–90th percentile].