Tomczykiewicz Kazimierz, Pastuszak Zanna, Staszewski Jacek, Stepień Adam
Pol Merkur Lekarski. 2014 Jan;36(211):28-30.
Stiff person syndrome (SPS) is the rare disease and cause great inefficient. It is characterized by progressive stiffness muscles of trunk and the limbs on which the cramps of muscles overlap. In the electrophysiological investigation of the patients the involuntary discharge of motor unit potentials find and also simultaneous contraction agonistic and antagonistic muscles. SPS is presented with insulin-dependent diabetes mellitus often or is the symptom of the paraneoplastic syndrome. It is commonly associated with high anti-glutamic acid decarboxylaze (GAD) antibody titters in the serum of the blood of patients. Establishing the diagnosis can cause difficulties. We observed the patient in the last period about the atypical course the disease. The diagnosis was confirmed occurrences of the high titters of antibodies anti-GAD, the discharge of motor unit potential in paraspinal muscles in the rest and good response the treatment with diazepamem.
僵人综合征(SPS)是一种罕见疾病,会导致严重功能障碍。其特征为躯干和四肢肌肉进行性僵硬,伴有肌肉痉挛。在对患者进行电生理检查时,可发现运动单位电位的不自主放电,同时拮抗肌和协同肌会同时收缩。SPS常与胰岛素依赖型糖尿病相关,或是副肿瘤综合征的症状。患者血液血清中通常会出现高滴度的抗谷氨酸脱羧酶(GAD)抗体。确诊可能存在困难。我们观察了该患者疾病的非典型病程。高滴度抗GAD抗体的出现、静息时椎旁肌运动单位电位的放电以及地西泮治疗的良好反应均证实了诊断。