Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Orphanet J Rare Dis. 2024 Sep 27;19(1):356. doi: 10.1186/s13023-024-03357-w.
Stiff person syndrome spectrum disorders (SPSD) are rare, disabling disorders of the nervous system that are associated with risk factors for Coronavirus disease 2019 (COVID-19). However, limited data exist on the overall impact of COVID-19 on SPSD.
Patients with SPSD and COVID-19 who are followed at Johns Hopkins SPS Center were included. Demographics and SPSD characteristics along with COVID-19-specific data were recorded.
Thirty-five cases of SPSD with COVID-19 cases were reported during the study time period. Mean age of the cohort was 56 (SD ± 10) and most were female (66.7%). Eighty percent of the COVID-19 cases were confirmed with testing, and the rest were highly suggestive of COVID-19. COVID-19 comorbidities among patients were hypertension (n = 6), diabetes (n = 6), obesity (n = 5), and cardiovascular disease (n = 4). The majority of participants were on immune therapies and/or benzodiazepines. Out of the cases reported, only 2 required hospitalization, both of whom had diabetes, and one was on immunosuppressive therapy. The majority of cases were post-full-vaccination cases. Fever was the most common COVID-19-associated symptom. Transient neurological symptoms were also reported.
Risk factors for developing severe COVID-19 in SPSD appear to be the same as historical data in the general population. Importantly, COVID-19 did not appear to be associated with worsening SPSD post-COVID-19. Vaccination may have played a role in preventing severe cases of COVID-19.
僵人综合征谱障碍(SPSD)是一种罕见的、使人丧失能力的神经系统疾病,与 2019 年冠状病毒病(COVID-19)的危险因素有关。然而,关于 COVID-19 对 SPSD 的总体影响的数据有限。
纳入在约翰霍普金斯 SPS 中心接受治疗的 SPSD 合并 COVID-19 的患者。记录患者的人口统计学和 SPSD 特征以及 COVID-19 特异性数据。
在研究期间报告了 35 例 SPSD 合并 COVID-19 病例。队列的平均年龄为 56(标准差±10),大多数为女性(66.7%)。80%的 COVID-19 病例通过检测确诊,其余病例高度提示 COVID-19。患者的 COVID-19 合并症包括高血压(n=6)、糖尿病(n=6)、肥胖(n=5)和心血管疾病(n=4)。大多数参与者正在接受免疫治疗和/或苯二氮䓬类药物治疗。报告的病例中,只有 2 例需要住院治疗,这 2 例都患有糖尿病,其中 1 例正在接受免疫抑制治疗。大多数病例是完全接种疫苗后的病例。发烧是 COVID-19 相关症状中最常见的症状。也有报告称出现短暂的神经系统症状。
SPSD 发生严重 COVID-19 的危险因素似乎与一般人群的历史数据相同。重要的是,COVID-19 似乎与 COVID-19 后 SPSD 恶化无关。接种疫苗可能在预防 COVID-19 重症方面发挥了作用。