Silva Paula Baleeiro Rodrigues, Apóstolos Pereira Samira Luisa, Aguiar Graziella, Terrim Sara, Filho Flávio Vieira Marques, Silva Guilherme Diogo, Neto Herval, Boaventura Mateus, Adoni Tarso, Sato Douglas Kazutoshi, Dellavance Alessandra, Marchiori Paulo Eurípedes, Callegaro Dagoberto
Neuroimmunology Group, Division of Neurology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), 255 Doutor Enéas de Carvalho Aguiar Avenue, São Paulo, 05403-000, Brazil.
Neuroimmunology Group, Division of Neurology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HC-FMUSP), 255 Doutor Enéas de Carvalho Aguiar Avenue, São Paulo, 05403-000, Brazil.
Mult Scler Relat Disord. 2025 Feb;94:106279. doi: 10.1016/j.msard.2025.106279. Epub 2025 Jan 20.
Neuromyelitis optica spectrum disorder (NMOSD) with positivity for aquaporin-4 antibody (AQP4-IgG) represents one of the mains etiologies of longitudinally extensive transverse myelitis (LETM). Advancements in early diagnosis and treatment have led to a decline in NMOSD-related mortality. However, long-term prognostic data for patients experiencing their first episode of LETM remain scarce, especially in Brazil. This study aims to evaluate the final diagnosis and long-term prognosis of patients with first episode of LETM and investigate factors associated with worse prognosis.
This is an observational retrospective study involving all consecutive patients diagnosed with longitudinally extensive myelopathy who were sequentially referred to the clinical neurology department of a brazilian tertiary hospital between January 2005 and December 2011. Only patients meeting the criteria for the first episode of idiopathic LETM were included. Data were retrieved from electronic medical records from October 2023 to January 2024.
39 patients met the inclusion criteria. After a median follow-up of 12 years, 51% patients remained with isolated monophasic seronegative LETM, 28% were diagnosed with NMOSD AQP4-IgG positive, 7.7% with NMOSD AQP4-IgG negative, 5% with MOGAD, 5% experienced recurrent seronegative LETM, and only 1 (2.6%) developed multiple sclerosis. The mortality rate was 10% at last follow-up, with a median time to death of 3 years. Deceased patients had a higher age at onset of LETM (OR 1.09, 95% CI 1.01-1.21). Among survivors, 17% had an Expanded Disability Status Scale (EDSS) ≥7 at last follow-up. Predictors of severe sequelae included higher EDSS at nadir (OR 5.29; 95% CI 1.38-39), pain as an initial myelitis symptom (OR 11.1; 95% CI 1.51-230) and spinal shock during the first myelitis (p < 0.001).
In this cohort, after a median 12-year follow-up, half of the patients remained as isolated monophasic seronegative LETM, mortality reached 10% and 83% of survivors were ambulatory. Predictors of poor prognosis included older age, presence of pain as an initial symptom and higher initial severity.
水通道蛋白4抗体（AQP4-IgG）阳性的视神经脊髓炎谱系障碍（NMOSD）是纵向广泛横贯性脊髓炎（LETM）的主要病因之一。早期诊断和治疗的进展已导致NMOSD相关死亡率下降。然而，首次发作LETM患者的长期预后数据仍然稀缺，尤其是在巴西。本研究旨在评估首次发作LETM患者的最终诊断和长期预后，并调查与预后较差相关的因素。
这是一项观察性回顾性研究，纳入了2005年1月至2011年12月期间连续转诊至巴西一家三级医院临床神经科的所有诊断为纵向广泛脊髓病的患者。仅纳入符合特发性LETM首次发作标准的患者。数据于2023年10月至2024年1月从电子病历中检索。
39例患者符合纳入标准。中位随访12年后，51%的患者仍为孤立性单相血清阴性LETM，28%被诊断为AQP4-IgG阳性的NMOSD，7.7%为AQP4-IgG阴性的NMOSD，5%为MOGAD，5%经历复发性血清阴性LETM，只有1例（2.6%）发展为多发性硬化症。最后一次随访时的死亡率为10%，中位死亡时间为3年。死亡患者LETM发病时年龄较大（OR 1.09，95%CI 1.01-1.21）。在幸存者中，17%在最后一次随访时扩展残疾状态量表（EDSS）≥7。严重后遗症的预测因素包括最低点时较高的EDSS（OR 5.29；95%CI 1.38-39）、疼痛作为最初的脊髓炎症状（OR 11.1；95%CI 1.51-230）以及首次脊髓炎期间的脊髓休克（p<0.001）。
在该队列中，中位随访12年后，一半的患者仍为孤立性单相血清阴性LETM，死亡率达到10%，83%的幸存者可独立行走。预后不良的预测因素包括年龄较大、疼痛作为初始症状以及初始严重程度较高。
