We report a 20-year-old man with intractable
myasthenia gravis successfully treated with total
lymphoid irradiation (TLI). An encapsulated
thymoma in the anterior mediastinum was resected as extended
thymectomy by video-assisted thoracoscopic surgery at 2 months after the onset of ptosis and
muscle weakness. Following treatments, such as ambenonium hydrochloride, an immunosuppression therapy (
prednisolone and azatioprine), 5 courses of immunoadsorption
therapy, and a high dose of
cyclophosphamide and
methylprednisolone, did not make persistent improvement of myasthenic symptoms.
Ageusia occurred twice prior to myasthenic crises and subsided with other myasthenic symptoms
after treatments.
Steroid psychosis and secondary
Cushing's syndrome made us to reduce the dose of
prednisolone. Post-operative residual, recurrent, or metastatic thymus was not unveiled, then we added the low dose fractionated irradiation (1.5 Gy x 12 = 18 Gy) to the mediastinum. Three months after the irradiation, however, a crisis occurred and the titer of anti-
acetylcholine receptor antibody increased up to 100 nmol/l. Therefore, we performed TLI (Mantle; 1.5 Gy x 9 = 13.5 Gy, paraaortic and inverted-Y; 1.5 Gy x 14 = 21 Gy), which brought about persistent improvement of myasthenic symptoms with decrease in the titer of anti-
acetylcholine receptor antibody.