Table 2.
Comparison of demographic and clinical characteristics between RLO-NMOSD and VLO-NMOSD.
| RLO-NMOSD (n=119) | VLO-NMOSD (n=15) | p Value | |
|---|---|---|---|
| Demographic | |||
| Female, n (%) | 106 (89.1) | 10 (66.7) | 0.032 |
| Age onset, year, Mean ± SD | 57.6 ± 6.0 | 74.5 ± 3.3 | 0.000 |
| Disease duration, month, Mean ± SD | 36.1 ± 31.4 | 19.7 ± 9.4 | 0.047 |
| Symptoms | |||
| Symptoms at onset, n (%) | |||
| ON | 30 (25.2) | 1 (6.7) | 0.190 |
| TM | 65 (54.6) | 13 (86.7) | 0.024 |
| Brainstem/cerebral | 10 (8.4) | 0 (0.0) | 0.602 |
| Combined a | 14 (11.8) | 1 (6.7) | >0.999 |
| Symptoms involvement, n (%) b | |||
| ON | 49 (41.2) | 3 (20.0) | 0.161 |
| TM | 104 (87.4) | 14 (93.3) | >0.999 |
| Brainstem+Cerebral | 45 (37.8) | 4 (26.7) | 0.571 |
| ON+TM a | 45 (37.8) | 3 (20.0) | 0.255 |
| Relapses | |||
| Recurrent, n (%) | 90 (75.6) | 10 (66.7) | 0.530 |
| Total number of relapses, Mean ± SD | 2.6 ± 1.5 | 2.1 ± 1.0 | 0.159 |
| Time to first relapse, month, Mean ± SD | 10.7 ± 10.4 | 8.2 ± 5.0 | 0.452 |
| ARR, Mean ± SD | 1.3± 0.9 | 1.5 ± 1.0 | 0.295 |
| ARR-1, Mean ± SD | 0.6 ± 0.5 | 0.7± 0.6 | 0.431 |
| Disability | |||
| EDSS at last follow up, median (IQR) | 3 (2.0-6.5) | 6 (3.5-8.0) | 0.026 |
| EDSS>6 at last follow up, n (%) | 32 (26.9) | 8 (53.3) | 0.068 |
| EDSS>8 at last follow up, n (%) | 13 (10.9) | 4 (26.7) | 0.100 |
| Visual acuity<0.1, n (%) c | 19 (16.0) | 2 (13.3) | >0.999 |
| Death, n (%) | 3 (2.5) | 1 (6.7) | 0.382 |
| Treatment | |||
| Acute phase treatment, n (%) | |||
| Glucocorticoid | 114 (96.0) | 13 (86.7) | 0.177 |
| PE or IVIG | 49 (41.2) | 10 (66.7) | 0.096 |
| Chronic treatment of IST, n (%) | 95 (79.8) | 7 (46.7) | 0.009 |
| Mycophenolate Mofetil | 81 (68.1) | 7 (46.7) | 0.147 |
| Azathioprine | 8 (6.7) | 0 (0.0) | 0.597 |
| Tacrolimus | 3 (2.5) | 0 (0.0) | >0.999 |
| Rituximab | 3 (2.5) | 0 (0.0) | >0.999 |
| Lab | |||
| AQP4-IgG positive, n (%) | 100 (84.0) | 13 (86.7) | >0.999 |
| Autoimmune antibodies, n (%) | 44 (37.0) | 8 (53.3) | 0.265 |
| Autoimmune diseases comorbidity, n (%) | 22 (18.5) | 1 (6.7) | 0.467 |
| Presence of OCB in CSF, n (%) | 5 (6.9) | 1 (6.7) | 0.517 |
| OCB unavailable, n (%) | 46 (38.7) | 4 (26.7) | 0.413 |
| Pleocytosis, n (%) | 23 (19.3) | 3 (20.0) | 0.736 |
| Increased CSF protein level, n (%) | 32 (26.9) | 7 (46.7) | 0.135 |
| MRI | |||
| Length of Spinal lesions d | |||
| Detailed data available, n (%) e | 98 (82.4) | 14 (93.3) | 0.464 |
| First attack, median (IQR) | 5.75 (3.0-8.25) | 9.0 (7.0-9.0) | 0.015 |
| At last follow up, median (IQR) | 6.0 (4.0-10.0) | 10.5 (7.5-14.25) | 0.007 |
| Brain lesions, n (%) | |||
| NMOSD typical lesion | 47 (39.5) | 3 (20.0) | 0.167 |
| Area postrema | 8 (6.7) | 1 (6.7) | >0.999 |
| Brainstem/cerebellum | 26 (21.9) | 1 (6.7) | 0.303 |
| Adjacent to 3rd ventricle | 14 (11.8) | 1 (6.7) | >0.999 |
| Surrounding lateral ventricles | 16 (13.5) | 3 (20.0) | 0.447 |
| Pyramidal tracts involvement | 7 (5.9) | 0 (0.0) | >0.999 |
| Extensive hemispheric lesions | 4 (3.4) | 0 (0.0) | >0.999 |
NMOSD, neuromyelitis optica spectrum disorder; RLO, very late onset; VLO, relative late onset; SD, stand deviation; IQR, inter-quartile range; ON, optic neuritis; TM, transverse myelitis; ARR, annualized relapse rate; ARR-1, ARR excluding the first attack; EDSS, Expanded Disability Status Scale; PE, plasma exchange; IVIG, intravenous immunoglobin; IST, immunosuppressive therapy; AQP4-IgG, aquaporin-4 immunoglobin G; CSF, cerebrospinal fluid; OCB, oligoclonal band.
Combined: defined as a combination of two or more core clinical characteristics.
Proportions of symptoms involved during the entire disease duration.
For the comparison of visual acuity, only those patients who had at least 1 optic neuritis attack were considered.
Length of the spinal cord lesions were measured in terms of the number of vertebral segments.
For the comparison of length of spinal cord lesions, only those patients who had at least 1 transverse myelitis attack were considered.
p values <0.05 were bolded.