Hagiwara H, Enomoto-Nakatani S, Sakai K, Ugawa Y, Kusunoki S, Kanazawa I
Department of Neurology, Division of Neuroscience, Graduate School of Medicine, University of Tokyo, Hongo 7-3-1, Tokyo, 113-8655, Bunkyo, Japan.
J Neurol Sci. 2001 Dec 15;193(1):59-62. doi: 10.1016/s0022-510x(01)00602-5.
We report a case of a 40-year-old female with continuous muscle stiffness and painful muscle spasms. The symptoms worsened over a two-week period after onset. Electrophysiological examinations revealed continuous muscle discharge, which was markedly reduced by intravenous administration of diazepam. High levels of anti-glutamic acid decarboxylase (GAD) antibodies were detected in both serum and cerebrospinal fluid, suggesting that the patient suffered from stiff-person syndrome. Steroid pulse therapy and immunoadsorption therapy alleviated the clinical symptoms and decreased the anti-GAD antibody titer. A chest CT revealed the presence of an invasive thymoma. Neither anti-acetylcholine receptor (AChR) antibodies nor symptoms of myasthenia gravis (MG) were observed. The patient underwent a thymectomy and postoperative radiotherapy. These treatments further alleviated the clinical symptoms. The present case is the first that associates stiff-person syndrome with invasive thymoma, and not accompanied by MG. The autoimmune mechanism, in this case, may be triggered by the invasive thymoma.
我们报告一例40岁女性患者，患有持续性肌肉僵硬和疼痛性肌肉痉挛。症状在发病后两周内加重。电生理检查显示持续性肌肉放电，静脉注射地西泮后明显减少。血清和脑脊液中均检测到高水平的抗谷氨酸脱羧酶（GAD）抗体，提示该患者患有僵人综合征。类固醇冲击疗法和免疫吸附疗法缓解了临床症状并降低了抗GAD抗体滴度。胸部CT显示存在侵袭性胸腺瘤。未观察到抗乙酰胆碱受体（AChR）抗体，也没有重症肌无力（MG）的症状。该患者接受了胸腺切除术和术后放疗。这些治疗进一步缓解了临床症状。本病例是首例将僵人综合征与侵袭性胸腺瘤相关联且不伴有MG的病例。在这种情况下，自身免疫机制可能由侵袭性胸腺瘤触发。
