Characteristics | Autopsy proven (n = 8) | Non-NMO-IgG tested (n = 38) | NMO-IgG tested (n = 50) |
Female | 7/8 | 37/38 | 44/50 |
Age at onset (years) | 28.3 | 30.3 | 32.6 |
Disease duration (years)* | 3.4 | 12.5 | 9.5 |
Annual attack rate** | 1.5 | 0.9 | 0.9 |
Clinical disease outside of the optic nerve or spinal cord† | 1/8 | 8/38 | 4/50 |
Brain MRI not meeting diagnostic criteria for MS‡ | 3/3 | 34/37 | 49/50 |
Contiguous spinal cord MRI lesion extending over ⩾3 vertebral segments. | 2/2 | 33/35 | 45/49 |
NMO-IgG seropositive status. | ND | ND | 16/50 |
CSF pleiocytosis (>50×106 white blood cells/l) | 2/6 | 4/35 | 2/44 |
Bilateral optic neuritis. | 8/8 | 29/38 | 38/50 |
Severe optic neuritis with fixed visual acuity more than 20/200 in at least one eye. | 6/8 | 28/38 | 32/50 |
Weakness in at least one limb; severe (MRC ⩽2), fixed and attack-related*** | 7/8 | 23/38 | 18/50 |
Non-NMO-IgG tested patients fulfil the 1999 NMO criteria.
NMO-IgG tested patients fulfil the 2006 NMO criteria.
†All symptoms were related to lesions contiguous to the optic nerves (hypothalamus) and/or spinal cord (ie, lower medulla).
‡Not fulfilling Paty criteria; cerebral MRI assessment: median time after onset of RNMO was 2.0 years.
*p = 0.005 autopsy proven vs non-NMO-IgG tested patients; p = 0.005 autopsy proven vs NMO-IgG tested patients.
**p = 0.02 autopsy proven vs non-NMO-IgG tested patients; p = 0.01 autopsy proven vs NMO-IgG tested patients.
***p = 0.01 autopsy proven vs NMO-IgG tested patients.
MRC, Medical Research Council; MS, multiple sclerosis; RNMO, relapsing neuromyelitis optica.