From the Department of Neurology, Thomas Jefferson University, Philadelphia, PA; Neuroimmunology Unit, National and Kapodistrian University of Athens Medical School, Athens, Greece.
Neurol Neuroimmunol Neuroinflamm. 2024 Mar;11(2):e200192. doi: 10.1212/NXI.0000000000200192. Epub 2023 Dec 26.
To describe a patient with mild GAD-positive stiff-leg syndrome (SLS) who developed severely disabling stiff-person syndrome (SPS) 1 week after mild COVID-19 and discuss the impact of viral implications.
Video-documented serial clinical observations at baseline, after acute COVID-19, and after IVIG treatments.
A 39-year-old man with left-SLS was stable during a 2-year follow-up with low-dose antispasmodics, working fully and functioning normally, even able to run. One week after mild COVID-19, he started to experience generalized SPS symptomatology that steadily worsened the following 2-3 weeks, becoming unable to walk, requiring a walker, with significant thoracolumbar and bilateral leg stiffness and spasms. GAD ab were very high. After 3 monthly IVIg infusions he showed improvements, but his gait remains significantly stiff. All clinical changes, from baseline to post-Covid, and then post- IVIg have been video-documented.
This is the first, clearly documented, severe GAD-positive SPS after COVID-19. Although viral or postviral causation can be incidental, the temporal connection with acute COVID-19, the severe disease worsening after symptom-onset, and the subsequent steady improvement after IVIg, suggest viral-triggered autoimmunity. Because COVID-19 reportedly can trigger or worsen GAD-associated diabetes type 1 through proinflammatory mediators, and SPS has been reportedly triggered by West Nile Virus, possibly through molecular mimicry, this case of acutely converting GAD-SLS to GAD-SPS suggest the need to explore viral etiologies in patients with GAD-SPS experiencing acute, long-lasting episodic exacerbations of stiffness and spasms.
描述一位患有轻度广泛性焦虑症阳性僵硬腿综合征（SLS）的患者，在轻度 COVID-19 后 1 周发展为严重致残性僵人综合征（SPS），并讨论病毒影响。
记录基线、急性 COVID-19 后和 IVIG 治疗后的视频记录连续临床观察。
一位 39 岁男性，左侧 SLS 在低剂量抗痉挛药物治疗下 2 年随访期间稳定，工作完全正常，甚至能够跑步。在轻度 COVID-19 后 1 周，他开始出现全身性 SPS 症状，症状在接下来的 2-3 周内逐渐恶化，导致无法行走，需要助行器，出现严重的胸腰椎和双侧腿部僵硬和痉挛。GAD ab 非常高。在接受 3 个月 IVIg 输注后，他有所改善，但步态仍明显僵硬。所有临床变化，从基线到 COVID-19 后，再到 IVIg 后，都进行了视频记录。
这是首例 COVID-19 后明确记录的严重 GAD 阳性 SPS。虽然病毒或 postviral 病因可能是偶然的，但与急性 COVID-19 的时间关联、症状出现后疾病的严重恶化以及随后 IVIg 后的稳定改善，提示病毒触发的自身免疫。由于 COVID-19 据报道可以通过促炎介质触发或加重 GAD 相关的 1 型糖尿病，并且 SPS 据报道可以通过分子模拟由西尼罗河病毒触发，这种急性将 GAD-SLS 转化为 GAD-SPS 的病例表明需要探索在急性、持久的僵硬和痉挛发作期间经历 GAD-SPS 的患者中的病毒病因。
