Neurology Service, Department of Neurosciences, Centro Hospitalar Universitário de Santo António, Porto, Portugal.
Neurology Service, Department of Neurosciences, Centro Hospitalar Universitário de Santo António, Porto, Portugal; Unit for Multidisciplinary Research in Biomedicine, Instituto de Ciências Biomédicas de Abel Salazar da Universidade do Porto, Porto, Portugal.
J Neurol Sci. 2024 Sep 15;464:123176. doi: 10.1016/j.jns.2024.123176. Epub 2024 Aug 12.
Neuromyelitis optica spectrum disorders (NMOSD) and MOG-associated disease (MOGAD) are an increasingly recognized group of demyelinating disorders of the central nervous system. Previous studies suggest that prognosis is predicted by older age at onset, number of relapses, the severity of the first attack and autoantibody status.
To study prognostic factors associated with disability progression and additional relapses in the 3-year follow-up of a national NMOSD/MOGAD cohort.
Out of 180 of the initial Portuguese cohort, data on 82 patients was available at the end of the follow-up period (2019-2022). Two patients died. Twenty (24.4%) patients had one or more attack in this period (25 attacks in total), mostly transverse myelitis (TM) (56.0%) or optic neuritis (32.0%). MOGAD was significantly associated with a monophasic disease course (p = 0.03), with milder attacks (p = 0.01), while AQP4 + NMOSD was associated with relapses (p = 0.03). The most common treatment modalities were azathioprine (38.8%) and rituximab (18.8%). AQP4 + NMOSD more frequently required chronic immunosuppressive treatment, particularly rituximab (p = 0.01). Eighteen (22.5%) had an EDSS ≥6 at the end of the follow-up. AQP4 + NMOSD (p < 0.01) and the occurrence of transverse myelitis (TM) during disease (p = 0.04) correlated with an EDSS≥6 at the end of the follow-up period. MOGAD was significantly associated with an EDSS<6 (p < 0.01), and MOG+ cases that reached an EDSS>6 were significantly older (64.0 ± 2.8 versus 31.0 ± 17.1, p = 0.017). A bivariate logistic regression model including the serostatus and TM attacks during disease history successfully predicted 72.2% of patients that progressed to an EDSS≥6.
This study highlights that myelitis predict increased disability (EDSS≥6) in NMOSD/MOGAG and AQP4 positivity is associated with increased disability.
视神经脊髓炎谱系疾病（NMOSD）和髓鞘少突胶质细胞糖蛋白相关疾病（MOGAD）是一组日益被认识的中枢神经系统脱髓鞘疾病。先前的研究表明，发病年龄较大、复发次数、首发发作的严重程度和自身抗体状态可预测预后。
研究与 NMOSD/MOGAD 队列 3 年随访期间残疾进展和额外复发相关的预后因素。
在最初的 180 例葡萄牙队列中，有 82 例患者在随访结束时（2019-2022 年）有数据。两名患者死亡。在此期间，20 名（24.4%）患者发生了一次或多次发作（共 25 次发作），主要是横断性脊髓炎（TM）（56.0%）或视神经炎（32.0%）。MOGAD 与单相病程显著相关（p=0.03），且发作程度较轻（p=0.01），而 AQP4+NMOSD 与复发相关（p=0.03）。最常见的治疗方法是硫唑嘌呤（38.8%）和利妥昔单抗（18.8%）。AQP4+NMOSD 更常需要慢性免疫抑制治疗，特别是利妥昔单抗（p=0.01）。18 名（22.5%）患者在随访结束时 EDSS≥6。AQP4+NMOSD（p<0.01）和疾病期间发生的横断性脊髓炎（TM）（p=0.04）与随访期末 EDSS≥6 相关。MOGAD 与 EDSS<6 显著相关（p<0.01），且达到 EDSS>6 的 MOG+病例年龄明显更大（64.0±2.8 与 31.0±17.1，p=0.017）。包括血清状态和疾病期间 TM 发作的二元逻辑回归模型成功预测了 72.2%进展为 EDSS≥6 的患者。
本研究强调，脊髓炎预测 NMOSD/MOGAD 中的残疾增加（EDSS≥6），AQP4 阳性与残疾增加相关。
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