Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, USA.
Eur J Neurol. 2023 Aug;30(8):2498-2505. doi: 10.1111/ene.15825. Epub 2023 May 21.
Stiff person syndrome (SPS) spectrum disorders (SPSSD) cause spasms and rigidity throughout different body regions and can be associated with apnea and acute respiratory failure. There are limited data on the prevalence and predictors of respiratory symptoms with spasms (RSwS) in SPSSD. We sought to characterize the spirometry patterns and the frequency and predictors of RSwS in a large SPSSD cohort.
Participants were recruited from the Johns Hopkins SPS Center between 1997 and 2021, as part of an ongoing, longitudinal observational study. Medical records were reviewed to assess demographics and clinical characteristics. Data were analyzed using descriptive statistics and multivariable logistic regression models.
One-hundred ninety-nine participants (mean age = 53.4 ± 13.6 years, median time to diagnosis = 36 [IQR 66] months, 74.9% women, 69.8% White, 62.8% classic SPS phenotype) were included in final analyses; 35.2% of participants reported RSwS, of whom 24.3% underwent spirometry as part of routine clinical care. Obstructive (23.5%) and restrictive (23.5%) patterns were most commonly observed in those with SPSSD. An increasing number of body regions involved predicted the presence of RSwS (odds ratio [OR] = 1.95, 95% confidence interval [CI] = 1.50-2.53); those with ≥5 body regions involved (vs. ≤4) had higher odds (OR = 6.19, 95% CI = 2.81-13.62) of experiencing RSwS in adjusted models. Two patients died from SPSSD-associated respiratory compromise.
RSwS are common in SPSSD and may be predicted by an increasing number of body regions involved by SPSSD. Close clinical monitoring and having a low threshold to obtain spirometry should be considered in people with SPSSD.
僵人综合征（SPS）谱障碍（SPSSD）引起不同身体区域的痉挛和僵硬，并可能伴有呼吸暂停和急性呼吸衰竭。SPSSD 中与痉挛相关的呼吸症状（RSwS）的患病率和预测因素的数据有限。我们旨在描述大型 SPSSD 队列中的肺功能模式以及 RSwS 的频率和预测因素。
参与者于 1997 年至 2021 年期间从约翰霍普金斯 SPS 中心招募，作为一项正在进行的纵向观察研究的一部分。对病历进行回顾以评估人口统计学和临床特征。使用描述性统计和多变量逻辑回归模型进行数据分析。
最终分析纳入了 199 名参与者（平均年龄 53.4±13.6 岁，中位诊断时间 36 [IQR 66] 个月，74.9%为女性，69.8%为白人，62.8%为经典 SPS 表型）；35.2%的参与者报告有 RSwS，其中 24.3%在常规临床护理中进行了肺功能检查。在 SPSSD 患者中，最常见的是阻塞性（23.5%）和限制性（23.5%）模式。涉及的身体区域数量增加预测了 RSwS 的存在（比值比 [OR] = 1.95，95%置信区间 [CI] = 1.50-2.53）；与 ≤4 个身体区域受累（OR = 6.19，95% CI = 2.81-13.62）相比，≥5 个身体区域受累的患者发生 RSwS 的可能性更高。有 2 例患者死于 SPSSD 相关的呼吸衰竭。
RSwS 在 SPSSD 中很常见，可能与 SPSSD 受累的身体区域数量增加有关。在 SPSSD 患者中，应密切临床监测并降低获得肺功能检查的门槛。
