Table 2.
Immunotherapy response in children and older patients with N-methyl-D-aspartate antibody encephalitis
| Patient demographics | Tumor and surgical management | ITx (%) | Response to first-line ITx | Second-line ITx | Response to second-line ITx | Relapse rate (%) | ITx | |||
|---|---|---|---|---|---|---|---|---|---|---|
| Study | Female | Median (range) age (years) | Type | Surgery (%) | Combination CS/IVIg/PLEX | |||||
| Florance et al. [11] | 32 (81) | 14 (23 months–18 years) | Ovarian teratoma (31 %) | 100 | 97 | 77 % improved: full recovery 29 % Substantial improvement 45 % Limited improvement 26 % |
23 % rituximab (n = 2), cyclophosphamide (n = 1) both (n = 4) |
60 % improved; 40 % slow but progressive improvement | 25 (all NP) | NA |
| Titulaer et al. [8] | 177 (74) | NA | Mostly/all ovarian teratomas (details NA) | 100 (n = 35) | 95 (n = 168) | 49 % responded; 98 % of these reached mRS 0–2 at 24 months | 56 of total (32 %) Rituximab (n = 42) Cyclophosphamide (n = 29) Other (n = 11) |
Overall at 24 months: 86 % mRS 0–2 (81 % of those receiving second-line ITx vs 65 % of those who failed first-line ITx but received no further treatment) | NA | NA |
| Armangue et al. [12] | 20 (70) | 13 (8 months–18 years) | Ovarian teratoma (n = 1), Follicular cyst (n = 1) |
100 (n = 2/2) | 20 | 60 % improved | Rituximab ± cyclophosphamide (n = 7) +MMF (n = 2) |
100 % improved; overall (firs ± second line): 60 % full recovery 25 % mild disability 10 % severe disability 5 % death (n = 1) |
0 | |
| Wright et al. [20] | 31 (74) | 8 (22 months–17 years) | Ovarian teratoma (n = 1) | 100 | 100 | Full recovery 50 % Partial recovery 30 % None 20 % Suggestion that regime with PLEX superior |
32 % of patients. Rituximab (n = 3) Cyclophosphamide (n = 3) Both (n = 3) MMF (n = 1) |
Full recovery 80 % Partial recovery 20 % Long-term outcome not related to use of second-line ITx; instead, good outcome more likely in early diagnosis |
23 % 6/7 prior first-line ITx |
First-line ITx in all + second-line ITx in 4/7 prevented further relapses |
| Titulaer et al. [13] | 31 (55) | 52 (45–84) | Ovarian teratoma (n = 1), Thymic cancer (n = 1), ovarian cancer (n = 1), breast cancer (n = 2), lung cancer (n = 2) | 71 | 91 | 45 % improved | 44 % of nonresponders to first-line ITx Cyclophosphamide and/or rituximab | Good response 6/7; Overall 60 % mRS 0–2 |
||
ITx = immunotherapy; IVIg = intravenous immunoglobulin; PLEX = plasma exchange; NP = nonparaneoplastic; NA = not available; mRS = modified Rankin Scale; MMF = mycophenolate mofetil