Table 1 Relation of thymoma and outcome in patients with anti-AMPAR encephalitis
Case | sex/ age | Tumor | Time from symptoms of AE to tumor diagnosis | Other autoimmune or antibodies | Treatment | Outcome | Reference |
|---|---|---|---|---|---|---|---|
1 | F/44 | Thymic carcinoma | Concurrent with first episode of encephalitis | ANA, dsDNA, cardiolipin antibodies | Tumor removal. At presentation and relapse: IVIg, corticosteroids. Chronic treatment with azathioprine. | First episode: returned to baseline. Subsequent relapsing: memory deficit. Residual short-term memory deficit after 3rd relapse. | Lai et al. 2009 |
2 | M/38 | Malignant thymoma | Concurrent with relapse of encephalitis | GAD antibodies | Tumor removal, radiation therapy, corticosteroids, plasma exchange, IVIg | First episode: returned to baseline. Mild residual memory deficit after relapse; steroid dependant muscle spasms and rigidity. | Lai et al. 2009 |
3 | F/44 | Thymoma | Concurrent with first episode of encephalitis | CV2/CRMP5 antibodies | N/A | Unexpected death due to cardiorespiratory arrest. | Lai et al. 2009 |
4 | F/60 | Malignant thymoma | Six years before first episode of encephalitis with residual thymoma without evidence of relapse. | None | Radiotherapy and chemotherapy six years before AE without thymectomy. At presentation: corticosteroids | Complete recovery | Graus et al. 2010 |
5 | F/47 | Thymoma (WHO type B1) | Concurrent with first episode of encephalitis | anti-AchR and titin antibodies | Corticosteroid at first episode. Corticosteroid, azathioprine and tumor removal when relapse. | First episode: short term memory deficit, severe anomic aphasia, executive dysfunction.Mild anomic aphasia and bulbar symptoms when relapse. | Li et al. 2015 |
6 | 62/M | Malignant thymoma | Concurrent with first episode of encephalitis | None | Tumor removal, corticosteroid, IVIg | Full treatment response | Höftberger et al. 2015 |
7 | 23/M | Thymoma | Concurrent with first episode of encephalitis | None | Tumor removal, IVIg, corticosteroid, rituximab | Partial treatment response | Höftberger et al. 2015 |
8 | 53/F | Malignant thymoma | Concurrent with first episode of encephalitis | CRMP5 | Tumor removal, chemotherapy, radiotherapy, corticosteroid, IVIg | No treatment response | Höftberger et al. 2015 |
9 | 71/M | Thymic carcinoid | Concurrent with first episode of encephalitis | NMDAR | Tumor removal, corticosteroid, plasma exchange | Full treatment response | Höftberger et al. 2015 |
10 | 21/M | Thymic carcinoid | Concurrent with first episode of encephalitis | None | Tumor removal, chemotherapy, radiotherapy, corticosteroid, IVIg | Mild deficits in working memory persisted at 18 months after disease onset. | Joubert et al. 2015 |
11 | F/34 | Thymoma (WHO type B3) | Concurrent with first episode of encephalitis; Recurrence with clinical relapse | None | Corticosteroid and tumor removal at first episode. Corticosteroid and resection of the recurrent tumor after relapse. | First episode: depressive symptoms. Memory deficits after relapse. | Omi et al. 2018 |
12 | F/50 | Thymoma (WHO type B2) | 2 years before first episode of encephalitis | anti-AchR antibodies | Tumor removal 2 years before AE. Corticosteroid and azathioprine. | First episode: returned to baseline. | Huang et al. |