Rosin L, DeCamilli P, Butler M, Solimena M, Schmitt H P, Morgenthaler N, Meinck H M
Department of Neurology, University of Heidelberg, Germany.
Neurology. 1998 Jan;50(1):94-8. doi: 10.1212/wnl.50.1.94.
We report a patient who developed stiff-man syndrome, including disabling shoulder subluxation and wrist ankylosis, in association with breast cancer. Immunologic investigations disclosed autoimmunity directed against not only glutamic acid decarboxylase but also amphiphysin, a 128-kd protein located in the presynaptic compartment of neurons. The patient improved after surgery and corticosteroid treatment and has been stable for nearly 4 years on only anti-estrogenics. The triad of stiff-man syndrome, breast cancer, and autoantibodies against amphiphysin identifies a new autoimmune paraneoplastic syndrome of the CNS.
我们报告了一名患有僵人综合征的患者，该综合征包括导致功能障碍的肩关节半脱位和腕关节强直，且与乳腺癌相关。免疫学检查发现，其自身免疫不仅针对谷氨酸脱羧酶，还针对 amphiphysin，一种位于神经元突触前区的128-kd蛋白质。患者在手术和皮质类固醇治疗后病情改善，仅使用抗雌激素药物治疗近4年病情一直稳定。僵人综合征、乳腺癌和抗 amphiphysin 自身抗体三联征确定了一种新的中枢神经系统自身免疫性副肿瘤综合征。
