Table 5.
Clinical, CSF, and demographic characteristics of NMOSD patients with available paired CSF and serum samples
| Patient number | 29 | 38 | 44 | 45 | 46 | 47 |
|---|---|---|---|---|---|---|
| Sex | Female | Female | Female | Female | Female | Male |
| Age (y) | 73.7 | 52.4 | 41.4 | 50.1 | 28.3 | 68.9 |
| Age at onset (y) | 73.7 | 52.2 | 26.4 | 49.6 | 25.6 | 68.9 |
| Disease duration (y) | 0.00 | 0.21 | 14.90 | 0.50 | 2.72 | 0.01 |
| Diagnosis | NMO | ON | NMO | NMO | NMO | NMO |
| Acute relapse | yes | yes | yes | yes | yes | no |
| Number of relapses | 1 | 1 | 5 | 2 | 6 | 1 |
| EDSS | 2.0 | 2.0 | 6.5 | 2.5 | 6.0 | 5.5 |
| Treatment | NoTh | Pred | RTX | AZA | RTX | PLEX |
| CSF cells/μl | 9 | 4 | 5 | 1 | 4 | 73 |
| Albumin quotient | 6.59 | 3.97 | 6.20 | 8.92 | 2.32 | 18.12 |
| IgG index | 0.64 | 0.55 | 0.48 | 0.58 | 1.20 | 0.58 |
| CSF OCB | Negative | Negative | Negative | Negative | Positive | Negative |
| AQP4-IgG CSFa | 6.19 | 1.23 | 4.18 | 2.50 | 5.04 | 4.15 |
| AQP4-IgG seruma | 11.59 | 3.38 | 15.02 | 4.47 | 9.38 | 7.98 |
| AQP4-IgG quotient | 0.53 | 0.36 | 0.28 | 0.56 | 0.54 | 0.52 |
| AQP4-IgG pattern CSF | A | A | A | A | A | A |
| AQP4-IgG pattern serum | B | B | A | B | B | B |
AQP4-M23, AQP4-M1 and extracellular loop A, C, and E mutations were expressed in HEK-293A cells and assayed to categorize pattern A and pattern B serum samples as indicated in Fig. 3
AZA azathioprine, NoTh no therapy, PLEX plasma exchange, Pred corticosteroids, RTX rituximab, y years, albumin quotient CSF albumin/serum albumin, IgG index albumin quotient/IgG quotient, OCB oligoclonal bands, AQP4-IgG quotient CSF/serum AQP4-IgG
aFACS AQP4-IgG binding ratio for AQP4-M23