Table 1 –
Clinical and demographic features of 68 included patients
|
Median age, years
|
44 (range, 1–78) |
|
| |
|
Children (age <18 years)
|
11 (17%) |
|
| |
|
Female sex
|
35 (52%) |
|
| |
| Neurological syndrome misinterpreted as NMOSD | |
| Isolated myelopathy | 29 (43%) |
| Myelopathy + optic neuropathy | 26 (38%) |
| Isolated optic neuropathy | 5 (7%), bilateral in 3 (60%) |
| Isolated area postrema syndrome | 2 (3%) |
| Combinations | 6 (9%) |
|
| |
| Disease course | |
| Progressive | 36/67 (54%) |
| Monophasic | 17/67 (25%) |
| Relapsing | 14/67 (21%) |
|
| |
| MRI findings | |
| Spinal Cord MRI T2 abnormalities | 56/63 (89%) |
| Longitudinally extensive for ≥ 3 VBS | 45/56 (80%) |
| Enhancing spinal cord lesions | 38/55 (69%) |
| Brain T2 abnormalities | 36/59 (61%) |
| Optic nerve T2 abnormalities | 16/52 (31%) |
| Enhancing brain/optic nerve lesions | 15/32 (47%) |
|
| |
| CSF findings | |
| Pleocytosis (>5 WBC) | 20/46 (43%) |
| Median cell count | 40 (range, 6–1800) |
| Protein elevation (>45 mg/dl) | 25/42 (60%) |
| CSF-restricted oligoclonal bands (≥ 2) | 9/29 (31%) |
|
| |
| Treatment | |
|
| |
| Received immunotherapy | 58/66 (88%) |
| Improvement after immunotherapy | 25/56 (45%) |
| Median number of treatments per patient | 2 (range, 1–5) |
| Corticosteroids | 56/58 (97%) |
| Plasma exchange | 16/58 (28%) |
| Rituximab | 7/58 (12%) |
| Intravenous immunoglobulins | 5/58 (9%) |
| Azathioprine | 4/58 (7%) |
| Cyclophosphamide | 4/58 (7%) |
| Mycophenolate Mofetil | 4/58 (7%) |
| DMTs | 1/58 (2%) |
| Infliximab | 1/58 (2%) |
|
| |
|
Seronegative NMOSD criteria met
|
12 (18%) |
Abbreviations – CSF = cerebrospinal fluid; DMTs = disease modifying treatments for multiple sclerosis; MRI = magnetic resonance imaging; NMOSD = neuromyelitis optica spectrum disorder; VBS = vertebral body segments; WBC = white blood cells