Table.
Clinical, laboratory and radiological findings of short transverse myelitis in aquaporin-4-IgG-positive patients and a population-based cohort of aquaporin-4-IgG-negative patients
| AQP4-IgG (+) (n=25) | AQP4-IgG (-) (n=27) | p value | |
|---|---|---|---|
| Demographics | |||
| Female sex | 18 (72%) | 21 (78%) | 0.63 |
| Median age (range) at myelitis onset | 50 yrs (29-70) | 42 yrs (18-67) | 0.04 |
| Non-Caucasian ethnicitya | 8 of 23 (35%) | 0 (0%) | <0.01 |
| Clinical Features | |||
| Numbness | 24 (96%) | 26 (96%) | 1.0 |
| Weakness | 12 (48%) | 7 (26%) | 0.10 |
| Bowel/bladder | 6 (24%) | 6 (22%) | 0.88 |
| Lhermittes | 4 (16%) | 2 (7%) | 0.41 |
| Tonic spasms | 6 (24%) | 1 (4%) | 0.046 |
| Concomitant nausea and vomiting | 2 (8%) | 0 (0%) | 0.23 |
| Need for gait aid at maximal severity | 4 (16%) | 1 (4%) | 0.18 |
| Personal Hx of autoimmunityb | 10 (40%) | 2 (7%) | <0.01 |
| Family Hx autoimmunity (1st degree relative) | 9 (36%) | 5 (19%) | 0.21 |
| Family Hx MS (1st degree relative) | 0 (0%) | 3 (11%) | 0.24 |
| Laboratory abnormalities | |||
| Antinuclear antibody | 9 of 20 (45%) | 4 of 22 (18%) | 0.1 |
| SSA/double stranded DNA antibodies | 5 of 13 (38%) | 2 of 13 (15%) | 0.38 |
| Cerebrospinal fluidc | |||
| Elevated white cell count (>5/μL)d | 7 of 11 (64%) | 10 of 21 (48%) | 0.47 |
| Elevated protein (>45 mg/dL) | 4 of 9 (44%) | 11 of 21 (52%) | 1.0 |
| Oligoclonal bands (>3) | 1 of 11 (9%) | 11 of 21 (52%) | 0.02 |
| Spine MRIe | |||
| Median interval (range), Sx onset to MRI | 15.5 days (2-90) | 24 days (3-90) | 0.81 |
| Single lesion | 18 (72%) | 19 (70%) | 0.9 |
| Median (range) T2 length, vertebral segments | 1 (0.5-2.5) | 1 (0.5-2.5) | 0.09 |
| Spinal cord swellingf | 4 of 15 (27%) | 15 (56%) | 0.11 |
| Gadolinium-enhancing lesion (1 or more) | 14 (56%) | 20 (74%) | 0.17 |
| Central location on axial imagesg | 16 of 29 (55%)f | 12 of 53 (23%)f | <0.01 |
| T1 hypointense foci | 4 of 14 (29%) | 0 of 24 (0%) | 0.01 |
| Subsequent myelitis longitudinally extensiveh | 12 of 13 (92%) | 0 of 8 (0%) | <0.01 |
| Brain lesions meeting MS criteriai | 4 (16%) | 13 of 26 (50%) | 0.02 |
Abbreviations: AQP4-IgG, aquaporin-4-IgG; Hx, history; DNA, deoxyribonucleic acid; MRI, magnetic resonance imaging; MS, multiple sclerosis; NMOSD, neuromyelitis optica spectrum disorder; Sx, symptom; TM, transverse myelitis; yrs, years;
Of those with ethnicity details available. Non-Caucasian ethnicities: Asian, 4; Hispanic, 2; African-American, 1; and Native-American-Indian, 1.
Personal history of autoimmunity (some multiple): thyroid autoimmunity, 4; systemic lupus erythematosus, 2; psoriasis, 2; AChR-IgG-positive myasthenia gravis, 1; rheumatoid arthritis, 1; rheumatic fever, 1; Sjogren syndrome, 1; Autoimmune thrombocytopenia, 1; and Addison disease, 1.
Of those with lumbar puncture performed at time of initial myelitis
Lymphocyte predominant in all four AQP4-IgG (+) patients with details available and in all 10 AQP4-IgG (-) patients.
In 14 patients the MRI’s were not available for re-review and the details were taken from the radiology report by the radiologist or neurologist’s report of the features of the lesion.
- AQP4-IgG (+): cervical, 6 (cervico-medullary junction, 1); thoracic, 17; both, 2.
- AQP4-IgG (-): cervical spine, 13; thoracic spine, 9; both 5;
Of those with details available
Of those with multiple lesions, axial appearance of each counted separately
longitudinally extensive refers to those with T2 signal extending ≥3 vertebral segments
brain lesions by Polman 2011 revised criteria7