Huang Juan, Meng Huan-Yu, Duan Xian, Li Wen-Wen, Xu Hong-Wei, Zhou Ya-Fang, Zhou Lin
Department of Geriatric Neurology, Xiangya Hospital, Central South University, Changsha, China.
National Clinical Research Center for Geriatric Disorders, Xiangya Hospital, Central South University, Changsha, China.
Front Neurol. 2020 May 8;11:284. doi: 10.3389/fneur.2020.00284. eCollection 2020.
Stiff limb syndrome (SLS) is a rare autoimmune-related central nervous system disorder, resulting in stiffness and spasms of limbs since onset with rare involvement of the truncal muscles. However, SLS patients will gain notable effects by appropriate therapy focusing on symptomatic treatment and immunotherapy. We reported on a 55-year-old female who showed typical painful spasms in both lower limbs and abduction of the right eyeball that partially responded to low-dose diazepam and had high-titer anti-glutamic acid decarboxylase (anti-GAD) antibody. Electromyography (EMG) only showed continuous motor unit activity (CMUA) in the anterior tibialis and right triceps. Eventually, our patient was diagnosed with SLS and treated with intravenous immunoglobulin (IVIG) and glucocorticoid combined simultaneously. She obtained notable effects. We also review and summarize the current literature on clinical characteristics, coexisting disease, treatment, and outcome of 40 patients with SLS. We hope that this report will provide a basis for further understanding of SLS and promote the formation of more advanced diagnosis and treatment processes.
僵肢综合征(SLS)是一种罕见的自身免疫相关的中枢神经系统疾病,自发病起即导致肢体僵硬和痉挛,躯干肌肉受累罕见。然而,通过以对症治疗和免疫治疗为主的适当治疗,SLS患者会取得显著疗效。我们报告了一名55岁女性,她双下肢出现典型的疼痛性痉挛,右眼球外展,对低剂量地西泮部分有效,且抗谷氨酸脱羧酶(抗GAD)抗体滴度高。肌电图(EMG)仅显示胫前肌和右三头肌有持续运动单位活动(CMUA)。最终,我们的患者被诊断为SLS,并同时接受静脉注射免疫球蛋白(IVIG)和糖皮质激素治疗。她取得了显著疗效。我们还回顾并总结了40例SLS患者的临床特征、并存疾病、治疗及预后的当前文献。我们希望本报告将为进一步了解SLS提供依据,并促进更先进的诊断和治疗流程的形成。