Ortiz Juan Fernando, Ghani Mohammad R, Morillo Cox Álvaro, Tambo Willians, Bashir Farah, Wirth Martín, Moya Gustavo
Neurology, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA.
Medicine, Universidad San Francisco de Quito, Quito, ECU.
Cureus. 2020 Dec 9;12(12):e11995. doi: 10.7759/cureus.11995.
Stiff-person syndrome (SPS) is a rare and disabling central nervous system disorder with no satisfactory treatment. Muscle rigidity, sporadic muscle spasms, and chronic muscle pain characterize SPS. SPS is strongly correlated with autoimmune diseases, and it is usual to find high titers of antibodies against acid decarboxylase (GAD65). Due to its highly disabling nature and complicated treatment, we aim to create a treatment protocol through a narrative review of currently available treatments that show efficacy. We expect to facilitate management based on treatment responses ranging from first-line medication to refractory medication. We conducted a medical subject heading (MeSH) strategy. We used the term SPS with the subheading treatment: "Stiff-Person Syndrome/Therapy" [MeSH]. An initial data gathering of 270 papers came out with the initial research. After using the inclusion criteria, we had 159 articles. We excluded 31 papers for being either systematic reviews, literature reviews, or meta-analysis. From the 128 remaining articles, we excluded another 104 papers because the extraction of the data was not possible or the study outcome did not meet our demands. There are two main treatments for SPS: GABAergic (gamma-aminobutyric acid) therapy and immunotherapy. For treatment, we suggest starting with benzodiazepines as first-line treatment. We recommend adding levetiracetam or pregabalin if symptoms persist. As second-line therapy, we recommend oral baclofen over rituximab and tacrolimus. We also suggest rituximab over tacrolimus. For patients with refractory treatment, we can use intrathecal baclofen, intravenous immunoglobulin (IVIG), or plasmapheresis. We conclude that intrathecal baclofen and IVIG are more effective than plasmapheresis in patients with refractory symptoms. Propofol may be used as a bridge - temporary therapy before initiating a permanent treatment.
僵人综合征（SPS）是一种罕见的、使人致残的中枢神经系统疾病，目前尚无令人满意的治疗方法。肌肉僵硬、散发性肌肉痉挛和慢性肌肉疼痛是SPS的特征。SPS与自身免疫性疾病密切相关，通常会发现高滴度的抗酸脱羧酶（GAD65）抗体。由于其高度致残的性质和复杂的治疗方法，我们旨在通过对目前显示出疗效的现有治疗方法进行叙述性综述来制定一种治疗方案。我们期望根据从一线药物到难治性药物的治疗反应来促进管理。我们采用了医学主题词（MeSH）策略。我们使用了“SPS”一词，并加上副标题“治疗”：“僵人综合征/治疗”[MeSH]。初步数据收集得到了270篇论文作为初步研究。在应用纳入标准后，我们得到了159篇文章。我们排除了31篇系统性综述、文献综述或荟萃分析类的论文。在剩下的128篇文章中，我们又排除了104篇，因为无法提取数据或研究结果不符合我们的要求。SPS主要有两种治疗方法：GABA能（γ-氨基丁酸）疗法和免疫疗法。对于治疗，我们建议首先使用苯二氮䓬类药物作为一线治疗。如果症状持续，我们建议加用左乙拉西坦或普瑞巴林。作为二线治疗，我们推荐口服巴氯芬，而不是利妥昔单抗和他克莫司。我们也建议优先使用利妥昔单抗而非他克莫司。对于难治性治疗的患者，我们可以使用鞘内注射巴氯芬、静脉注射免疫球蛋白（IVIG）或血浆置换。我们得出结论，对于难治性症状的患者，鞘内注射巴氯芬和IVIG比血浆置换更有效。丙泊酚可作为一种过渡——在开始永久性治疗前的临时治疗。
