Eggiman Evan, Kerr William, Spivey Brandon, Lish Luke, Gregg Nathan, Leggett Jonathan
Campbell University's School of Medicine, Leon Levine Hall of Medical Sciences, 4350 US Hwy 421 S, Lillington, NC, 27546, USA.
Conway Medical Center, 300 Singleton Ridge Rd, Conway, SC, 29526, USA.
BMC Neurol. 2025 Apr 9;25(1):149. doi: 10.1186/s12883-025-04157-w.
Stiff Person Syndrome (SPS) is a rare autoimmune neurological disorder characterized by progressive muscle rigidity and painful spasms. Standard treatments often yield variable responses, particularly in severe, refractory cases. This case report highlights the novel use of ketamine as an effective therapeutic agent for managing acute SPS exacerbations, underscoring its potential as a second-line treatment for patients unresponsive to conventional therapies.
A 22-year-old male with SPS, diagnosed via anti-glycine receptor antibodies, presented with an acute exacerbation of symptoms, including severe stiffness triggered by sensory stimuli. Initial management with high-dose benzodiazepines, baclofen, and intravenous methocarbamol failed to provide adequate relief. The patient was subsequently treated with intravenous ketamine, resulting in rapid and significant symptom resolution. Despite initial improvement, the patient experienced multiple recurrent flares requiring repeated ketamine administration. Over time, ketamine proved consistently effective in resolving acute symptoms when standard treatments were insufficient. The patient's management was complicated by anxiety, hypoxia, venous thromboembolism, and other comorbidities, highlighting the need for a multidisciplinary approach.
This case illustrates the potential utility of ketamine in managing acute and refractory SPS symptoms, providing rapid symptom resolution and reducing disease burden during severe flares. Ketamine's mechanism of action, including NMDA receptor antagonism and enhancement of GABAergic signaling, makes it a promising adjunct in SPS treatment protocols. This report emphasizes the importance of individualized, multidisciplinary care and the need for further research to establish ketamine's role in the long-term management of SPS.
僵人综合征（SPS）是一种罕见的自身免疫性神经疾病，其特征为进行性肌肉僵硬和疼痛性痉挛。标准治疗的反应往往各不相同，尤其是在严重的难治性病例中。本病例报告强调了氯胺酮作为治疗急性SPS加重的有效治疗药物的新用途，突出了其作为对传统疗法无反应患者的二线治疗的潜力。
一名22岁的男性SPS患者，通过抗甘氨酸受体抗体确诊，出现症状急性加重，包括由感觉刺激引发的严重僵硬。最初使用高剂量苯二氮䓬类药物、巴氯芬和静脉注射美索巴莫治疗未能提供充分缓解。该患者随后接受静脉注射氯胺酮治疗，症状迅速且显著缓解。尽管最初有所改善，但患者经历了多次复发发作，需要重复使用氯胺酮。随着时间的推移，当标准治疗不足时，氯胺酮在解决急性症状方面始终有效。患者的治疗因焦虑、缺氧、静脉血栓栓塞和其他合并症而复杂化，凸显了多学科方法的必要性。
本病例说明了氯胺酮在管理急性和难治性SPS症状方面的潜在效用，可迅速缓解症状并减轻严重发作期间的疾病负担。氯胺酮的作用机制，包括N-甲基-D-天冬氨酸（NMDA）受体拮抗和增强γ-氨基丁酸（GABA）能信号传导，使其成为SPS治疗方案中有前景的辅助药物。本报告强调了个体化多学科护理的重要性以及进一步研究以确定氯胺酮在SPS长期管理中的作用的必要性。
