Essalmi L, Meaux-Ruault N, Hafsaoui C, Gil H, Curlier E, Dupond J-L
Service de médecine interne et immunologie clinique, hôpital Jean-Minjoz, CHU de Besançon, 2, boulevard Fleming, 25030 Besançon cedex, France.
Rev Med Interne. 2007 Sep;28(9):627-30. doi: 10.1016/j.revmed.2007.05.010. Epub 2007 Jun 8.
Stiff person syndrome is a rare autoimmune neurologic disorder characterized by axial muscular rigidity and intermittent painful spasms. Three distinct forms are described: auto-immune, paraneoplastic and idiopathic.
We report the case of a 51-year-old man with an history of Stiff Person Syndrome with typical clinical, electrophysiological and immunological findings. Anti-glutamic acid decarboxylase antibodies were present, as well as anti-amphiphysin antibodies, commonly reported in the paraneoplastic syndrome. CT scan revealed a thymoma. Surgical resection was followed by reduction of the neurologic symptoms, without lowering the auto antibodies titer.
The association between Stiff Person Syndrome and thymoma is exceptional. Thymectomy is an effective treatment and may act without change of the autoantibodies titer. Patients with Stiff Person Syndrome should be systematically tested for thymoma.
僵人综合征是一种罕见的自身免疫性神经疾病，其特征为躯干肌肉僵硬和间歇性疼痛性痉挛。该病有三种不同类型：自身免疫性、副肿瘤性和特发性。
我们报告了一例51岁男性僵人综合征患者，其具有典型的临床、电生理和免疫学表现。患者存在抗谷氨酸脱羧酶抗体，以及副肿瘤综合征中常见的抗 amphiphysin 抗体。CT扫描显示有胸腺瘤。手术切除后神经症状有所减轻，但自身抗体滴度未降低。
僵人综合征与胸腺瘤的关联较为罕见。胸腺切除术是一种有效的治疗方法，且可能在自身抗体滴度不变的情况下发挥作用。僵人综合征患者应系统检查是否患有胸腺瘤。
