Comparison of clinical features of four phenotypes with aquaporin-4 antibody-positive neuromyelitis optica patients
| All patients | Phenotype | p Value | ||||
| Neuromyelitis optica | Optic neuritis | Spinal type | Brain-dominant | |||
| No of patients | 583 | 199 | 21 | 38 | 31 | |
| Male:female (female%) | 47:502 (91.4%) | 13:182 (93.3%) | 3:19 (86.3%) | 5:33 (86.8%) | 5:26 (83.8%) | NS |
| Age at onset (median, year) | 44 (3–86) | 43 (6–85) | 39 (3–76) | 54 (24–79) | 35 (19–51) | * |
| Disease duration (median, year) | 4.5 (1–46) | 7 (1–46) | 6 (1–24) | 5 (1–18) | 2 (1–5) | * |
| Annual relapse rate (median) | 1.0 (0.1–9.0) | 1.0 (0.1–4.2) | 0.8 (0.3–4.0) | 1.0 (0.2–9.0) | 1.0 (0.5–3.0) | NS |
| Extended Disability Status Scale score (median) | 6 (0–10) | 6.5 (1–10) | 6 (2.5–8.5) | 7 (1–9) | 0 (0–1) | * |
| Visual outcome | ||||||
| Blindness | 65/401 (16.2%) | 33/176 (18.7%) | 1/8 (12.5%) | 0/38 (0.0%) | 9/28 (32.1%) | 0.0053† |
| Severe impairment | 115/401 (28.6%) | 76/176 (43.1%) | 6/8 (75.0%) | 0/38 (0.0%) | 8/28 (28.5%) | 0.00048† |
| First attack | ||||||
| Optic nerve | 245/500 (49.0%) | 117/199 (58.7%) | 21/21 (100.0%) | 0/37 (0.0%) | 14/31 (45.1%) | <0.0001† |
| Spinal cord | 232500 (46.4%) | 89/199 (44.7%) | 0/21 (0.0%) | 38/38 (100%) | 9/31 (29.0%) | <0.0001† |
| Brainstem | 46/500 (9.2%) | 16/199 (8.0%) | 0/21 (0.0%) | 0/37 (0.0%) | 3/31 (9.6%) | 0.041† |
| Cerebrum | 43/500 (8.6%) | 16/199 (8.0%) | 0/21 (0.0%) | 0/37 (0.0%) | 7/31 (22.5%) | 0.0020† |
| MRI lesions | ||||||
| Spinal cord | 386/452 (85.3%) | 199/199 (100%) | 1/10 (10.0%) | 38/38 (100%) | 15/27 (55.5%) | <0.0001† |
| Longitudinally extensive transverse myelitis | 329/452 (72.7%) | 199/199 (100%) | 0/10 (0.0%) | 38/38 (100%) | 0/27 (0.0%) | <0.0001† |
| Cerebrum | 227/444 (51.1%) | 93/199 (46.7%) | 1/10 (10.0%) | 16/37 (43.2%) | 31/31 (100%) | <0.0001† |
| Optic nerve | 205/491 (41.7%) | 83/199 (41.7%) | 17/21 (80.9%) | 0/37 (0.0%) | 17/30 (56.6%) | <0.0001† |
| Brainstem | 129/444 (29.0%) | 56/199 (28.1%) | 1/10 (10.0%) | 1/37 (2.7%) | 13/30 (43.3%) | 0.00069† |
| Systemic disease | ||||||
| Sjögren syndrome | 42/212 (19.8%) | 12/72 (16.6%) | 3/7 (42.8%) | 6/21 (28.5%) | 1/10 (10.0%) | NS |
| Thyroid diseases | 29/212 (13.6%) | 4/28 (14.2%) | 0/7 (0.0%) | 2/21 (10.5%) | 4/10 (40.0%) | NS |
| Autoantibody | ||||||
| Sjögren syndrome A antibody or Sjögren syndrome B antibody | 90/ 260 (34.6%) | 25/102 (24.5%) | 4/11 (36.3%) | 8/21 (38.0%) | 5/14 (35.7%) | NS |
| Antinuclear autoantibody | 136/249 (54.6%) | 43/102 (42.1%) | 7/11 (63.6) | 14/21 (33.3%) | 10/14 (71.4%) | 0.041† |
| Cerebrospinal fluid | ||||||
| Oligoclonal band | 33/274 (12.0%) | 14/108 (12.9%) | 0/6 (0.0%) | 4/24 (16.6%) | 0/14 (0.0%) | NS |
| Myelin basic protein | 141/245 (57.5%) | 44/82 (53.6%) | 1/2 (50.0%) | 19/22 (86.3%) | 8/16 (50.0%) | 0.036† |
↵* Multiple comparisons between four phenotypes were made using the Tukey honestly significant difference test, and each statistical value is shown in figure 1.
↵† Statistically significant by χ2 test.
Optic neuritis type, isolated recurrent optic neuritis more than three times; spinal type, isolated recurrent myelitis more than three times.