Jonsson Dagur Ingi, Sveinsson Olafur, Moeini Nina, Pivac Emir, Wirdefeldt Karin, Brundin Lou, Iacobaeus Ellen
Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden.
Department of Neurophysiology, Karolinska University Hospital, Stockholm, Sweden.
Neurol Neuroimmunol Neuroinflamm. 2025 Nov;12(6):e200472. doi: 10.1212/NXI.0000000000200472. Epub 2025 Sep 9.
Myelitis is a relatively common clinical entity for neurologists, with diverse underlying causes. The aim of this study was to describe the incidence of myelitis, its causes, clinical presentation, and factors predicting functional outcomes and relapses.
Using the Swedish National Patient Registry, we identified all adult patients in Stockholm County between 2008 and 2018 using International Classification of Diseases, 10th Edition (ICD-10) codes likely to include myelitis. We collected medical records and classified patients using a modification of the 2002 Transverse Myelitis Consortium Group criteria. Long-term follow-up data were collected for patients not diagnosed with multiple sclerosis (MS) or neuromyelitis optica spectrum disorder as a result of the initial myelitis.
We identified 2,321 individuals, of whom 461 were patients with myelitis. The crude mean incidence of all-cause myelitis was 24.9 (95% CI 16.7-33.9) cases per million person-years, of which idiopathic myelitis had an incidence of 8.0 (95% CI 3.8-12.1) cases per million person-years. Partial myelitis was found in 80% of patients. Poor functional outcome was found in 11% of the cohort and correlated, in a multivariate logistic model, with age older than 50 years (OR 4.26, 95% CI 1.75-10.40), transverse spinal cord lesions (odds ratio [OR] 6.85, 95% CI 2.68-17.52), elevated CSF count of polymorphonuclear cells (OR 6.09, 95% CI 1.56-23.72), and elevated CSF/serum albumin ratio (OR 3.17, 95% CI 1.23-8.17). The median follow-up time was 5.4 years. Relapses occurred in 27% of patients with idiopathic myelitis and 72% of patients with unspecified demyelinating disease of the CNS. An increased relapse rate after idiopathic myelitis was found to be associated, in a multivariate model, with the presence of oligoclonal bands (incidence rate ratio [IRR] 4.47, 95% CI 1.70-11.73), transverse spinal cord lesions (IRR 2.81, 95% CI 1.11-7.12), and multifocal spinal cord lesions (IRR 2.82, 95% CI 1.03-7.69). Around half (48%) of all patients with myelitis received MS diagnosis during the study period.
This large population-wide study describes a relatively high incidence of myelitis and low risk of relapses after idiopathic myelitis. A complete diagnostic workup of myelitis, including MRI of the entire CNS and collection of CSF, is essential in evaluating underlying causes and prognosis.
脊髓炎是神经科医生相对常见的临床病症，其潜在病因多样。本研究旨在描述脊髓炎的发病率、病因、临床表现以及预测功能转归和复发的因素。
利用瑞典国家患者登记系统，我们通过国际疾病分类第10版（ICD - 10）编码识别了2008年至2018年间斯德哥尔摩郡所有可能包括脊髓炎的成年患者。我们收集了病历，并使用2002年横贯性脊髓炎协会组标准的修订版对患者进行分类。对因初始脊髓炎未被诊断为多发性硬化（MS）或视神经脊髓炎谱系障碍的患者收集长期随访数据。
我们识别出2321人，其中461例为脊髓炎患者。全因脊髓炎的粗年均发病率为每百万人口年24.9例（95%可信区间16.7 - 33.9），其中特发性脊髓炎的发病率为每百万人口年8.0例（95%可信区间3.8 - 12.1）。80%的患者为部分脊髓炎。11%的队列患者功能转归较差，在多因素逻辑回归模型中，与年龄大于50岁（比值比[OR] 4.26，95%可信区间1.75 - 10.40）、横贯性脊髓损伤（比值比[OR] 6.85，95%可信区间2.68 - 17.52）、脑脊液多形核细胞计数升高（OR 6.09，95%可信区间1.56 - 23.72）以及脑脊液/血清白蛋白比值升高（OR 3.17，95%可信区间1.23 - 8.17）相关。中位随访时间为5.4年。特发性脊髓炎患者中27%出现复发，中枢神经系统未特定脱髓鞘疾病患者中72%出现复发。在多因素模型中，发现特发性脊髓炎后复发率增加与寡克隆带的存在（发病率比[IRR] 4.47，95%可信区间1.70 - 11.73）、横贯性脊髓损伤（IRR 2.81，95%可信区间1.11 - 7.12）以及多灶性脊髓损伤（IRR 2.82，95%可信区间1.03 - 7.69）相关。在研究期间，所有脊髓炎患者中约一半（48%）被诊断为MS。
这项大规模的全人群研究描述了脊髓炎相对较高的发病率以及特发性脊髓炎后较低的复发风险。对脊髓炎进行全面的诊断检查，包括整个中枢神经系统的MRI检查和脑脊液采集，对于评估潜在病因和预后至关重要。
