Téma: Original Works

Diagnosis and treatment of thymoma-associated paraneoplastic myasthenia gravis

Peter Špalek1, Milan Schnorrer2, Tibor Krajč3

In seropositive myasthenia gravis (MG), the pathogenic, high-affinity autoantibodies are directed against nicotinic acetylcholine receptors (AChR). In about 10-15 % of anti-AChR-seropositive cases, MG is a paraneoplastic phenomenon caused by thymomas. The paraneoplastic MG starts with abnormal, non-tolerogenic T-cells selection inside thymomas with specifity for AChR. Next step is the export of intolerant T-cells to the „peripheral“ extrathymomatous immmune system, (lymph nodes, normal thymus, spleen and bone marrow). Finally, at these sites the production of AChR autoantibodies occur. Two-thirds of thymomas are benign, one-third are invasive and may extend into mediastinal fat, pleura, pericardium, great vessels and lung. Hematogenous or lymph node metastases occur rarely. This article focuses on diagnosis and treatment of MG associated with thymoma. Paraneoplastic MG is typically generalized but otherwise indistinguishable from other cases of adult-onset MG. All patients with MG and thymoma have antibodies against AChR. Thymomas are highly suggestive in patients who present with seropositive generalised MG and with autoantibodies to titin and ryanodine receptor. All patients with newly diagnosed MGshould have chest CT for thymoma evaluation. Surgical resection of thymoma is indicated in all cases to prevent thymoma progression and to facilitate treatment of MG. Complete MG remission after thymomectomy is unlikely. Therefore, most patients with paraneoplastic MG require longterm immunosuppressive therapy. With benign thymoma, the prognosis of paraneoplastic MG is similar to cases of generalized MG without thymoma. Malignant thymomas require adjunctive radiation treatment. Chemotherapy may be used for metastatic thymoma.

Ročník 2010  Témy časopisu Neurology 2 / 2010

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