Table 1.
Patient demographics and immunotherapy response in the 4 largest studies of N-methyl-D-aspartate receptor encephalitis
| Description | Tumour and surgical management | Patients treated with ITx | Response to first-line ITx | Second-line ITx used (n, %) | Response to second-line ITx | Relapse rate | Relapse treatment and response | ||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Study | Total patients (n) | Age (years), median (range) | Women | n | Type (n) | Surgically resected (%) | n (%) | Percentage tumor surgery | Combination of: IVMP/poCS (75–100 % of those treated) ± IVIg (34–67 %) ± PLEX (~33 %) | ||||
| Dalmau et al. [7] | 100 | 23 (5–76) | 91 | 58 | Ovarian teratoma (53) | 88; earlier tumor resection associated with better outcome; 6 had no ITx |
92 (92) | Nonresponders to first-line ITx: 17 (18 %) Overall results (including second-line ITX): full recovery (mRS 0) 47 % (31 % if ITx only) Mild stable deficits (mRS 1–2) 28 % 18 % severe deficits |
20 patients (22 %): rituximab (10) cyclophosphamide (9) AZA (1) Alone or combination |
76 % responded to cyclophos-phamide and/or rituximab | 15 % (1–3 each) 14/15 no tumor or late tumor detection, 0/15 on ITx at relapse |
mRS 0–2 in 10/15 with S ± first-line ITx ± rituximab | |
| Mediastinal teratoma (1) | |||||||||||||
| Testicular teratoma (1) | |||||||||||||
| SCLC (1) | |||||||||||||
| Sex chord stromal tumor (1); neuroendocrine tumor (1) | |||||||||||||
| Irani et al. [6] | 44 | 22 (2–49) | 31 (70) | 9 | Ovarian teratoma (8) | 35 (80) | Outcome NP = P; better with shorter time to oophorectomy |
NA Improvement only if receiving ITx within 40 days of onset; trend to better outcome if CS + other ITx |
Cyclophosphamide (9 %) rituximab (5 %), AZA (2 %), MMF (2 %), alone or combination |
NA | 10 % (23 % of NP); 2–4 each, no or limited ITx at relapse | NA | |
| Hodgkin’s lymphoma (1) | |||||||||||||
| Titulaer et al. [8] | 501 | 21 (8 months–85 years) | 81 % | 220/577 38 % | Ovarian teratoma (94 %) | 96 % teratomas resected | 462 (92) |
2 % S alone | 53 % response (including S in P group) 96 % mRS 0–2 within 24 months |
27 % total (57 % of nonresponders to first-line ITx) Rituximab (20 %) Cyclophosphamide (16 %) AZA/MMF/tacrolimus/MTX (6 %) |
67 % mRS 0–2 51 % of nonresponders to first-line ITx evolved to mRS 0–2 without second-line ITx |
12 %; NP > > P ITx < < no ITx |
NA; ↓ reduced rate if start 2nd line ITx |
| Extraovarian teratoma (2 %) | |||||||||||||
| 4 %: breast/lung/testicular/ovarian/thymic/pan-creatic carcinoma | |||||||||||||
| Viaccoz et al. [19] | 71 | 25 (18–75) | 58 (81) | 25 (35 %) | Ovarian teratoma (23) Breast (1) Schwannnoma (1 (male)) |
N/A | N/A (12/13 male pts) |
Not reported, but 5/12 (42 %) treated male patients and 51–60 % female patients received only first-line ITx | Rituximab (42 % of total patients) Cyclophosphamide (10 % of total) AZA/MMF (28 % of total) |
Not clearly reported 59 % mRS 0 at 12 months |
15.5 % | Not reported | |
Data are n (%) unless otherwise indicated
ITx = immunotherapy; IVMP = intravenous methylprednisolone; IVIg = intravenous immunoglobulin; PLEX = plasma exchange; SCLC = small cell lung carcinoma; NP = nonparaneoplastic; P = paraneoplastic; S = surgery; mRS = modified Rankin Scale; NA = not available; CS = corticosteroids; AZA = azathioprine; MMF = mycophenolate mofetil; MTX = methotrexate