Department of Neurology, Tulane University School of Medicine, 1430 Tulane Avenue 8065, New Orleans, LA 70112, USA.
Department of Neurology, Tulane University School of Medicine, 1430 Tulane Avenue 8065, New Orleans, LA 70112, USA.
Neurol Clin. 2013 Feb;31(1):319-328. doi: 10.1016/j.ncl.2012.09.005.
Recognizing stiff person syndrome is clinically important. It is uncommon, characterized by body stiffness associated with painful muscle spasms, and varies in location and severity. It is subdivided into stiff trunk versus stiff limb presentation, and as a progressive encephalomyelitis. Stiff person-type syndrome also reflects a paraneoplastic picture. Most patients demonstrate exaggerated lumbar lordosis. Roughly 60% of patients have antiglutamic acid decarboxylase antibodies in the blood and the cerebrospinal fluid. The differential diagnosis includes many severe conditions. There are reports of response to muscle relaxants, immunosuppressants, intravenous gamma globulin, plasma exchange, a number of anticonvulsants, and botulinum toxin.
认识到僵人综合征具有重要的临床意义。它并不常见，其特征为伴有疼痛性肌肉痉挛的躯体僵硬，且其位置和严重程度存在差异。它分为僵硬躯干与僵硬肢体表现，以及进展性脑脊髓炎。僵人样综合征也反映出一种副肿瘤表现。大多数患者表现为腰椎过度前凸。大约 60%的患者血液和脑脊液中存在抗谷氨酸脱羧酶抗体。鉴别诊断包括许多严重疾病。有报道称肌肉松弛剂、免疫抑制剂、静脉注射丙种球蛋白、血浆置换、多种抗惊厥药和肉毒毒素治疗有效。
