Therapeutic Apheresis for Intravenous Methylprednisolone-Refractory Neuromyelitis Optica Spectrum Disorder: Clinical and Radiological Outcomes in a Single-Center Case Series.

IF 1.2
Wataru Horiguchi, Daisuke Katagiri, Takeyuki Watadani, Shunichi Matsuda, Hiroshi Kaneko, Noritoshi Arai
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Abstract

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing autoimmune disease of the central nervous system. High-dose intravenous methylprednisolone (IVMP) is the standard first-line therapy for acute attacks, although some patients remain refractory. Therapeutic apheresis is often used in such cases, although its efficacy in milder presentations or spinal cord-dominant forms remains less established. Few studies have quantitatively assessed the treatment effects using imaging, particularly magnetic resonance imaging (MRI)-based lesion measurements, leaving a gap in the objective evaluation of therapeutic outcomes.

Objective: We aimed to evaluate the clinical and radiological outcomes of therapeutic apheresis in patients with NMOSD, including those with mild disabilities, following IVMP.

Methods: We retrospectively analyzed eight patients with NMOSD treated between 2016 and 2023, who underwent therapeutic apheresis after IVMP for acute relapse. The Expanded Disability Status Scale (EDSS) scores and spinal cord lesion areas on T2-weighted MRI were compared before and after apheresis.

Results: Seven of eight patients showed clinical improvement, with EDSS scores improving from 5.5 ± 1.6 to 4.0 ± 2.3 (p = 0.007). Spinal cord lesion areas decreased from 4.21 ± 3.37 to 1.12 ± 0.657 cm2 (p = 0.03). Apheresis was effective even in patients with lower baseline EDSS scores. Seven of the eight patients had nonoptic neuritis, supporting efficacy for spinal cord-dominant NMOSD.

Conclusions: Therapeutic apheresis following IVMP may benefit a broad range of patients with NMOSD, including those with mild symptoms and nonoptic presentations. Our findings underscore the importance of incorporating objective radiological assessments into future evaluations of treatment responses.

静脉注射甲基强的松龙难治性神经脊髓炎视谱障碍的治疗性采珠术:单中心病例系列的临床和放射学结果
背景:视神经脊髓炎谱系障碍(NMOSD)是一种复发性中枢神经系统自身免疫性疾病。大剂量静脉注射甲基强的松龙(IVMP)是急性发作的标准一线治疗,尽管一些患者仍然难治性。治疗性离心分离法常用于此类病例,尽管其对较轻症状或脊髓显性形式的疗效尚不确定。很少有研究使用影像学,特别是基于磁共振成像(MRI)的病变测量来定量评估治疗效果,这在客观评估治疗结果方面留下了空白。目的:我们旨在评估包括轻度残疾患者在内的NMOSD患者在IVMP后治疗性分离的临床和放射学结果。方法:回顾性分析2016年至2023年间8例因急性复发而接受静脉穿刺治疗的NMOSD患者。比较采血前后扩展残疾状态量表(EDSS)评分和t2加权MRI脊髓损伤面积。结果:8例患者中有7例临床改善,EDSS评分由5.5±1.6分提高到4.0±2.3分(p = 0.007)。脊髓损伤面积由4.21±3.37 cm2减少至1.12±0.657 cm2 (p = 0.03)。即使在基线EDSS评分较低的患者中,离心分离法也有效。8例患者中有7例患有非视神经炎,支持脊髓主导型NMOSD的疗效。结论:IVMP后的治疗性分离可能有益于广泛的NMOSD患者,包括那些症状轻微和无视觉表现的患者。我们的发现强调了将客观放射学评估纳入未来治疗反应评估的重要性。
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