Table 2 Review previous case series associated with thymoma and Isaacs’ syndrome
Age | 33 [9] | 42 [10] | 46 [11] | 46 [12] | 48 [13] | 53[14] | 65 [15] | 65 [16] | 68 [10] |
|---|---|---|---|---|---|---|---|---|---|
Sex | Male | Male | Male | Male | Male | Male | Male | Male | Male |
Thymoma | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Yes |
Stage (AJCC1) | Stage IV | Stage IV | Stage IV | Stage IV | Stage IV | Stage IV | Stage IIIB | Stage IV | No data |
Isaacs’ syndrome | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Yes |
Myasthenia gravis | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Not mentioned | No |
Other paraneoplastic syndromes | – | – | Pure red cell aplasia | – | Pure red cell aplasia | – | – | – | – |
AchR2 antibody | Positive | Positive | Not mentioned | Positive | Positive | Not test | Yes | No test | Yes |
VGKC3/VGKC-complex antibody | Negative | No test | CASPR2a | VGKC | VGKC | LGI1b and CASPR2 | No test | No test | No test |
Other antibodies | – | – | – | – | Antistriatal antibodies | Antistriatal antibody | – | – | – |
Thymoma treatment | Thymectomy Chemotherapy Radiotherapy Cytoreductive surgery, HITOC4 with cisplatin | Thymectomy Splenectomy | Thymectomy Chemotherapy Radiotherapy | Thymectomy Chemotherapy Radiotherapy | Thymectomy Chemotherapy Radiotherapy | Thymectomy Thoracotomy Pleurectomy Chemotherapy Photodynamic therapy | Thymectomy | Thymectomy Radiotherapy | Subtotal thymectomy Radiotherapy |
Isaacs’ syndrome treatment Poor response | Phenytoin Baclofen, Tacrolimus Plasma exchange | – | Prednisolone (40 mg/day), Phenytoin, Baclofen, DFPP5 | Phenytoin (300 mg/day) Carbamazepine (600 g/day) | – | Gabapentin High-dose methylprednisolone IVIG6 | IVIG (also for MG crisis) with partial improvement | – | – |
Good response | Carbamazepine | Periodic plasmapheresis Immunosuppressant | Rituximab (600 mg) | Azathioprine Steroids Plasma exchanges (also for MG crisis) | – | – | Carbamazepine | – | Plasma exchange |
Follow-up | Symptoms relieved after thymoma treatment. No symptoms and no tumor recurrence in the next 1.5 years. | The patient died due to metastatic disease a few months later. | Moderate decrease of CASPR2 antibody and free of myokymia two months after initiation of rituximab. | During steroid and cytotoxic therapy, anti-VGKC antibodies fell. VGKC antibodies increased after the withdrawal of cytotoxic treatment. The patient died from cardiopulmonary arrest. | The patient died 5 months later due to acute liver failure. | Symptoms improved after chemotherapy. | Symptoms still existed but were under control by carbamazepine. | No symptoms exist after radiotherapy. | The patient died 8 months after the operation. The autopsy was declined. |