CAJ | 학술논문

目的：探讨临床表现酷似僵人综合征的神经系统副肿瘤综合征患者的诊断学特征。方法回顾性分析1例临床表现为僵人综合征的神经系统副肿瘤综合征患者的临床资料，并复习相关文献。结果患者临床表现为僵直、肌肉痉挛、癫痫，脑脊液检查示细胞数、蛋白正常；抗钾通道相关抗体、抗 N-甲基-D-天冬氨酸受体抗体、抗谷氨酸脱羧酶抗体、单胞病毒抗体正常，肌电图示连续的运动单位活动。颅脑强化磁共振示右蝶骨嵴区脑膜瘤，颈椎磁共振示 C3-4、C4-5、C5-6、C6-7椎间盘轻度后突出；胸部 CT 示纵隔内及双侧锁骨上下窝区多发肿大淋巴结，锁骨上肿大淋巴结病理结果显示转移性小细胞癌。结论临床表现似僵人综合征的患者，如找不到病因，应行全身检查尤其是肺部检查排除神经系统副肿瘤综合征。
목적：탐토림상표현혹사강인종합정적신경계통부종류종합정환자적진단학특정。방법회고성분석1례림상표현위강인종합정적신경계통부종류종합정환자적림상자료，병복습상관문헌。결과환자림상표현위강직、기육경련、전간，뇌척액검사시세포수、단백정상；항갑통도상관항체、항 N-갑기-D-천동안산수체항체、항곡안산탈최매항체、단포병독항체정상，기전도시련속적운동단위활동。로뇌강화자공진시우접골척구뇌막류，경추자공진시 C3-4、C4-5、C5-6、C6-7추간반경도후돌출；흉부 CT 시종격내급쌍측쇄골상하와구다발종대림파결，쇄골상종대림파결병리결과현시전이성소세포암。결론림상표현사강인종합정적환자，여조불도병인，응행전신검사우기시폐부검사배제신경계통부종류종합정。
Objective To explore the diagnostic features of paraneoplstic syndrome (PNS ) presented with stiff-person syndrome(SPS).Methods One patient with SPS caused by tumor was diagnosed as paraneoplastic neurological syndrome.The relevant literatures were reviewed.Results One patient was mainly presented with rigidity,muscular spasm and epilepsy;the cerebro-spinal fluid (CSF)protein content and CSF cell count were normal.The anti-potassium channel antibody,anti-N-methyl-D-aspartate receptor antibody,anti-glutamate decarboxylase antibody and anti-herpes simplex virus antibody were all negative.The electromyography revealed continuous motor unit activity.The sphenoid ridge meningoma was found on cerebral enhanced MR.Mild cervical disc herniation was presented on cervical spine MR.Chest CT showed multiple swollen lymph nodes in mediastinal and supradavicular and the pathologic result of the supraclavicular lymph node showed metastatic small cell cancer.Conclusions For patients presented as SMS without particular causes,the general examinations,especially the chest examinations are essential for diagnosis.