Table 2.
Clinical and MRI features of patients with paraneoplastic and non-paraneoplastic NMOSD with AQP4 antibodies.
| Paraneoplastic (n = 17) | Non-paraneoplastic (n = 151) | p value | |
|---|---|---|---|
| Age in years, median (range) | 55 (17–87) | 40 (10–77) | 0.006 |
| Male/female | 5/12 | 10/141 | 0.009 |
| Autoimmune diseases | 1 | 39 | 0.172 |
| Presenting syndrome | |||
| Optic neuritis | 2 | 64 | |
| LETM | 7 | 60 | 0.001 |
| NMO | 0 | 16 | |
| Brainstem | 7a | 10 | |
| Other symptoms | 1b | 1c | |
| Spine MRI | |||
| No. of vertebral segments; median (range)d | 7 (3–20) | 5 (1–23) | 0.068 |
| CSF findings | |||
| WBC; abnormal (>10 cell/mm3) | 5/15 | 30/127 | 0.769 |
| Proteins; abnormal (>45 mg/dL) | 9/15 | 37/127 | 0.404 |
| Oligoclonal bands | 0/12 | 23/126 | 0.233 |
| Clinical outcome | |||
| Improved | 10 | 36 | 0.204 |
| Stable | 2 | 44 | |
| Worse | 5 | 71 | |
| Death | 5 | 8 | 0.005 |
LETM: longitudinal extensive transverse myelitis; NMO: neuromyelitis optica; WBC: white blood cell; MRI: magnetic resonance imaging; NMOSD: neuromyelitis optica spectrum disorder; AQP4: aquaporin-4.
a
Four patients rapidly presented an additional LETM.
b
Encephalitis rapidly followed by LETM.
c
Myelitis without LETM criteria.
d
Data collected from 12 paraneoplastic and 87 non-paraneoplastic NMOSD patients who developed myelitis.