Table 1.
Epidemiological and clinical features according to diagnosis at last follow-up
| Total population n = 125 |
Relapsing ON n = 61 |
NMOSD-like phenotype n = 41 |
bRelapsing TM n = 11 |
cMADEM/brainstem S n = 5 |
dMS-like/optico-spinal phenotype n = 7 |
|
|---|---|---|---|---|---|---|
| Females, n (%) | 69 (55.2) | 33 (54.1) | 24 (58.5) | 5 (45.5) | 3 (60) | 4 (57.1) |
| Age at onset, years, median (range) | 34.1 (18.0–67.1) | 36.0 (18.0–67.1) | 34.6 (18.0–62.5) | 33.7 (18.0–42.1) | 45.7 (31.3–60.7) | 22.7 (19.4–53.7) |
| Caucasian, n (%) | 120 (96) | 58 (95.1) | 40 (97.6) | 11 (100) | 5 (100) | 6 (85.7) |
| Follow-up, years, median (range) | 4.5 (0.2–47) | 1.4 (0.4–47) | 5.7 (0.2–47) | 10.9 (2.1–21.2) | 2.5 (0.56–4.0) | 5.5 (0.2–19.3) |
| Phenotype at onset, n (%) | ||||||
| ON | 82 (65.6) | 61 (100) | 16 (39) | 0 | 0 | 5 (71.4) |
| Myelitis | 25 (20) | 0 | 12 (29.3) | 11 (100) | 0 | 2 (28.6) |
| ON and myelitis | 9 (7.2) | 0 | 9 (22) | 0 | 0 | 0 |
| Encephalopathic/brainstem S. | 9 (7.2) | 0 | 4 (9.7) | 0 | 5 (100) | 0 |
| EDSS at onset, median (range) | 3 (0–9) | 2.5 (0–4) | 3.25 (0–7.5) | 3 (1–6) | 4.5 (3.5–9) | 3.5 (3–8) |
| EDSS 0–2.5 | 47 (38.2) | 30 (50) | 22 (55) | 4 (36.6) | 3 (60) | 5 (71.4) |
| EDSS 3–5.5 | 64 (52) | 30 (50) | 32 (55) | 4 (36.6) | 3 (60) | 5 (71.3) |
| EDSS ≥ 6.0 | 12 (9.8) | 0 | 6 (15) | 2 (18.2) | 2 (40) | 2 (28.6) |
| aARR mean (SD) | 0.79 (0.91) | 0.80 (0.76) | 0.64 (0.76) | 0.46 (0.41) | 1.13 (1.06) | 1.78 (2.19) |
| Acute treatment MTP/PLEX/IVIG), n (%) | 120 (96) | 61 (100) | 39 (95.1) | 10 (90.9) | 3 (60) | 7 (100) |
| Paraclinical features, n (%) | ||||||
| CSF OCB | 10/107 (9.4) | 1/49 (2.04) | 4/36 (11.1) | 3/11 (27.3) | 0/5 (0) | 2/6 (33.3) |
| CSF pleiocytosis | 41/98 (41.8) | 7/46 (15.2) | 21/31 (67.7) | 6/10 (60) | 4/5 (80) | 3/6 (50) |
|
Abnormal brain MRI, at onset |
28/74 (37.8) | 7/38 (18.4) | 11/23 (47.8) | 3/6 (50) | 4/4 (100) | 3/3 (100) |
| EDSS at the last follow-up, median (range) | 2 (0–7) | 1.0 (0–4) | 2 (0–7) | 2 (0–4) | 2.5 (1–3.5) | 3.5 (0–6.5) |
| EDSS 0–2.5 | 86 (69.9) | 48 (80) | 26 (65) | 7 (63.6) | 3 (60) | 2 (28.6) |
| EDSS 3–5.5 | 31 (25.2) | 12 (20) | 11 (27.5) | 4 (36.6) | 2 (40) | 2 (28.6) |
| EDSS ≥ 6.0 | 6 (4.9) | 0 | 3 (7.5) | 0 | 0 | 3 (42.9) |
| VA at the last follow-up | ||||||
| VA ≥ 0.7 | 61/100 (61) | 36/61 (59) | 18/31 (58.1) | – | 3/3 (100) | 4/5 (80) |
| VA > 0.2–0.6 | 21/100 (21) | 13/61 (21.3) | 8/31 (25.8) | – | 0 | 0 |
| VA ≤ 0.2 | 18/100 (18) | 12/61 (19.7) | 5/31 (16.1) | – | 0 | 1/5 (20) |
aFor ARR (SD), index event was excluded
bFive patients had an extensive transverse myelitis
cThree patients had multiphasic-ADEM with further ON relapses (ADEM-ON)
dOptico-spinal phenotypes in 4, multiple sclerosis-like phenotype in 3 patients
ON optic neuritis, NMOSD neuromyelitis optica spectrum disorder, TM transverse myelitis, ADEM-ON acute disseminated encephalomyelitis-optic neuritis, Brainstem S brainstem syndrome, MS multiple sclerosis, Optico-spinal optico-spinal phenotype, EDSS Expanded Disability Status Scale, ARR annualised relapse ratio, SD standard deviation, MTP methylprednisolone, PLEX plasma exchange, IVIG intravenous immunoglobulins, CSF cerebrospinal fluid, OCB oligoclonal bands, MRI magnetic resonance imaging, VA visual acuity