Tang Li-Ya, Yu Sheng-Yuan, Huang Yong-Hua
Department of neurology, PLA Army General Hospital, Beijing, People's Republic of China.
J Musculoskelet Neuronal Interact. 2018 Sep 1;18(3):389-392.
We reported a Stiff person syndrome (SPS) patient with elevated autoantibodies against cardiolipin and β2 glycoprotein 1 but without glutamic acid decarboxylase (GAD) antibodies. A 40-year male was admitted due to limited mouth opening for 1 week. His blood routine, biochemical, infectious diseases, tumor markers, radiographic examinations were all normal. At day 3 (D3) after admission, he developed paroxysmal systemic muscle rigidity. At D6, the on-duty physician occasionally gave oral clonazepam, which effectively relieved the symptom. At D13, the titers of cardiolipin and β2 glycoprotein 1 autoantibodies elevated but the remaining autoantibodies were all in normal ranges. After clonazepam treatment for 1 week, the symptoms were basically relieved, and the titers of these two antibodies returned to normal range with the relief of symptoms. During the 3 years of follow-up, the symptoms did not present again, and the titers of both antibodies were stable in the normal ranges. He had no tumor and other immune system diseases. In summary, we reported a SPS case with elevated cardiolipin and β2 glycoprotein 1 autoantibodies. The patient was highly responsive to clonazepam therapy, and had favorable outcome in the 3 years follow-up. Our report is helpful for better understand the heterogeneous feature of SPS.
我们报告了一名僵人综合征（SPS）患者，其抗心磷脂和β2糖蛋白1自身抗体升高，但无谷氨酸脱羧酶（GAD）抗体。一名40岁男性因张口受限1周入院。他的血常规、生化、传染病、肿瘤标志物及影像学检查均正常。入院后第3天（D3），他出现阵发性全身肌肉僵硬。在D6时，值班医生偶然给予口服氯硝西泮，症状得到有效缓解。在D13时，心磷脂和β2糖蛋白1自身抗体滴度升高，但其余自身抗体均在正常范围内。氯硝西泮治疗1周后，症状基本缓解，随着症状缓解这两种抗体的滴度恢复到正常范围。在3年的随访期间，症状未再次出现，两种抗体的滴度均稳定在正常范围内。他没有肿瘤及其他免疫系统疾病。总之，我们报告了一例心磷脂和β2糖蛋白1自身抗体升高的SPS病例。该患者对氯硝西泮治疗反应良好，在3年随访中预后良好。我们的报告有助于更好地理解SPS的异质性特征。
