Bhatti Adnan Bashir, Gazali Zarine Anwar
Department of Medicine, Capital Development Authority Hospital, Islamabad, Pakistan.
Research Fellow, MITR Hospital, Kharghar, Navi Mumbai, Maharashtra, India.
Cureus. 2015 Dec 22;7(12):e427. doi: 10.7759/cureus.427.
Stiff Person Syndrome (SPS) is one of the rarest autoimmune neurological disorders, which is mostly reported in women. It is characterised by fluctuating muscle rigidity and spasms. There are many variants of SPS, these include the classical SPS, Stiff Leg Syndrome (SLS), paraneoplastic variant, gait ataxia, dysarthria, and abnormal eye movements. Studies have shown that the paraneoplastic variant of SPS is more common in patients with breast cancer who harbour amphiphysin antibodies, followed by colon cancer, lung cancer, Hodgkin's disease, and malignant thymoma. Currently, the treatment for SPS revolves around improving the quality of life by reducing the symptoms as far as possible with the use of GABAergic agonists, such as diazepam or other benzodiazepines, steroids, plasmapheresis, and intravenous immunoglobulin (IVIG). There have been random clinical trials with Rituximab, but nothing concrete has been suggested. A treatment approach with standard drugs and cognitive behavioral therapy (CBT) seems to be promising.
僵人综合征（SPS）是最罕见的自身免疫性神经疾病之一，多见于女性。其特征为肌肉僵硬和痉挛程度波动。SPS有多种变体，包括经典型SPS、僵腿综合征（SLS）、副肿瘤变体、步态共济失调、构音障碍和异常眼动。研究表明，SPS的副肿瘤变体在携带抗 amphiphysin 抗体的乳腺癌患者中更为常见，其次是结肠癌、肺癌、霍奇金病和恶性胸腺瘤。目前，SPS的治疗主要围绕通过使用γ-氨基丁酸能激动剂（如地西泮或其他苯二氮䓬类药物）、类固醇、血浆置换和静脉注射免疫球蛋白（IVIG）尽可能减轻症状来提高生活质量。已有关于利妥昔单抗的随机临床试验，但尚未得出确切结论。采用标准药物和认知行为疗法（CBT）的治疗方法似乎很有前景。
