Table 1.
Demographic and clinical characteristics of AQP4-IgG-positive Japanese and German NMOSD patients included in this study.
| AQP4-IgG-positive NMOSD | German (Berlin) (n = 38) | Japanese (Chiba) (n = 54) | P |
|---|---|---|---|
| Age at last follow-up, years: mean ± SD | 50.61 ± 14.00 | 55.30 ± 13.13 | 0.104 |
| Sex: n female/male (female %) | 35/3 (92.1%) | 48/6 (88.9%) | 0.877 |
| Age at disease onset, years: mean ± SD | 42.50 ± 15.32 | 41.96 ± 14.96 | 0.867 |
| Early/late disease onset: n (%) | 0.858 | ||
| <30 years | 7 (18.4%) | 12 (22.2%) | |
| 30–50 years | 13 (34.2%) | 16 (29.6%) | |
| >50 years | 18 (47.4%) | 26 (48.1%) | |
| Disease duration at last follow-up, years: mean ± SD | 8.11 ± 6.90 | 13.33 ± 11.08 | 0.018 |
| EDSS at last follow-up: median [IQR] | 4 [2.12, 5.25] | 4.00 [2.00, 5.75] | 0.784 |
| Visual FSS: median [IQR] | 1.00 [0.00, 3.00] | 1.00 [1.00, 2.00] | 0.807 |
| Brainstem FSS: median [IQR] | 0.00 [0.00, 1.00] | 0.00 [0.00, 0.00] | <0.001 |
| Pyramidal FSS: median [IQR] | 1.00 [0.00, 3.00] | 0.00 [0.00, 0.00] | <0.001 |
| Cerebellar FSS: median [IQR] | 1.00 [0.00, 2.00] | 2.00 [0.00, 3.00] | 0.155 |
| Sensory FSS: median [IQR] | 2.00 [1.00, 3.00] | 0.50 [0.00, 2.00] | 0.001 |
| Bowel and bladder FSS: median [IQR] | 1.00 [0.00, 2.00] | 2.00 [0.00, 5.00] | 0.037 |
| Cerebral FSS: median [IQR] | 0.50 [0.00, 1.00] | 0.00 [0.00, 0.00] | <0.001 |
| Ambulation FSS: median [IQR] | 4.50 [0.00, 4.88] | 1.00 [0.00, 5.25] | 0.669 |
| Severe disability at last follow-up: n (%) | 8 (21.1%) | 14 (25.9%) | 0.771 |
| EDSS increase per attack: median [IQR] | 0.95 [0.67, 1.56] | 0.63 [0.48, 1.24] | 0.059 |
| CSF-specific oligoclonal bands: n (%) | 6/25(19.4%) | 8/27 (22.9%) | 0.964 |
| Autoimmune comorbidities: n (%) | 15 (39.5%) | 10 (18.5%) | 0.047 |
| – Sjoergen syndrome: n (%) | 3 (7.9%) | 6 (11.1%) | 0.877 |
| – Hashimoto disease: n (%) | 5 (13.2%) | 3 (5.6%) | 0.369 |
| – Rheumatoid arthritis: n (%) | 1 (2.6%) | 1 (1.9%) | >0.999 |
| – Myasthenia gravis: n (%) | 3 (7.9%) | 1 (1.9%) | 0.379 |
| – Systemic lupus erythematosus: n (%) | 6 (15.8%) | 0 (0.0%) | 0.010 |
| – Raynaud’s syndrome: n (%) | 1 (2.6%) | 0 (0.0%) | 0.859 |
| – Mixed connective tissue disease: n (%) | 2 (5.3%) | 0 (0.0%) | 0.328 |
| – Secondary antiphospholipid syndrome: n (%) | 1 (2.6%) | 0 (0.0%) | 0.859 |
AQP4-IgG: Aquaporin 4-immunoglobulin G; EDSS: expanded disability status scale; FSS: functional system score; IQR: interquartile range; n = number; NMOSD: neuromyelitis optica spectrum disorders, SD: standard deviation.
Note that these group comparisons were performed using t-test for current age, age at disease onset, and disease duration, Chi-square-test for categorial variables, severe disability at last follow-up (defined as an EDSS ≥6), and Wilcoxon-Mann-Whitney test for EDSS and functional system scores and EDSS increase per attack. Significant p-values are indicated in bold.