Iancu Raluca, Pirvulescu Ruxandra, Anton Nicoleta, Iancu George, Istrate Sinziana, Romanitan Mihaela Oana, Geamanu Aida, Popa Cherecheanu Matei
Department of Ophthalmology, "Carol Davila" University of Medicine and Pharmacy, 050474 Bucharest, Romania.
Department of Ophthalmology, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania.
Diagnostics (Basel). 2024 Apr 23;14(9):863. doi: 10.3390/diagnostics14090863.
Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorder (NMOSD) are autoimmune-mediated central nervous system disorders distinguished by the presence of serum aquaporine-4 IgG antibody (AQP4-Ab). The clinical panel comprises severe optic neuritis (ON) and transverse myelitis, which can result in incomplete recovery and a high risk of recurrence.
This study aimed to evaluate the visual outcomes of three patients with severe acute ON in NMOSD that was non-responsive to intravenous methylprednisolone (IVMP), who received plasma exchange therapy (PLEX). We included three patients (P1, P2 and P3) with severe acute ON who had no improvement after IVMP treatment and were admitted to the ophthalmology department at the Emergency University Hospital Bucharest from January 2022 to September 2023. All three patients with ON were diagnosed in accordance with the criteria described by the Optic Neuritis Treatment Trial. All the subjects were experiencing their first attack.
The mean recruitment age was 35.3 ± 7.71. All patients were seropositive for the AQP4 antibody. All patients were tested for serum myelin oligodendrocyte glycoprotein (MOG) antibody but only one showed a positive test (P3). Lesions visible in orbital MRI indicated the involvement of retrobulbar, canalicular and/or intracranial segments. All three subjects had no response or incomplete remission after an IVMP protocol (5 days of 1000 mg intravenous methylprednisolone in sodium chloride 0.9%). The mean time from onset of optic neuritis to PLEX was 37.6 days. The PLEX treatment protocol comprised five cycles of plasma exchange treatment over 10 days, with a plasma exchange session every other day. An amount of 1 to 1.5 volumes of circulating plasma were dialyzed for 2-4 h. At 1 month after the completion of PLEX therapy, BCVA and VF parameters were improved in all three patients.
The treatment of ON remains subject to debate and is somewhat controversial. Plasma exchange must be considered as a rescue therapy when IVMP is insufficient for AQP4-ON patients. This study revealed that PLEX treatment effectively improves the visual outcomes of patients experiencing their first attack of severe acute isolated ON after high-dose IVMP treatment. This study suggests that PLEX may be associated with improved visual outcomes in NMOSD acute optic neuritis.
视神经脊髓炎（NMO）和视神经脊髓炎谱系障碍（NMOSD）是自身免疫介导的中枢神经系统疾病，其特征是血清水通道蛋白4 IgG抗体（AQP4-Ab）阳性。临床症状包括严重视神经炎（ON）和横贯性脊髓炎，可导致恢复不完全和复发风险高。
本研究旨在评估3例对静脉注射甲泼尼龙（IVMP）无反应的NMOSD严重急性ON患者接受血浆置换疗法（PLEX）后的视觉预后。我们纳入了3例（P1、P2和P3）严重急性ON患者，他们在IVMP治疗后无改善，并于2022年1月至2023年9月入住布加勒斯特紧急大学医院眼科。所有3例ON患者均根据视神经炎治疗试验描述的标准进行诊断。所有受试者均为首次发作。
平均入组年龄为35.3±7.71岁。所有患者AQP4抗体血清学阳性。所有患者均检测了血清髓鞘少突胶质细胞糖蛋白（MOG）抗体，但只有1例呈阳性（P3）。眼眶MRI可见的病变表明球后、小管和/或颅内段受累。所有3例受试者在接受IVMP方案（5天，每天静脉注射1000mg甲泼尼龙于0.9%氯化钠溶液中）后无反应或未完全缓解。从视神经炎发作到进行PLEX的平均时间为37.6天。PLEX治疗方案包括在10天内进行5个周期的血浆置换治疗，每隔一天进行一次血浆置换。每次置换1至1.5个循环血浆量，持续2至4小时。在PLEX治疗完成后1个月，所有3例患者的最佳矫正视力（BCVA）和视野（VF）参数均得到改善。
ON的治疗仍存在争议。当IVMP对AQP4-ON患者治疗不足时，必须考虑将血浆置换作为一种挽救疗法。本研究表明，PLEX治疗可有效改善高剂量IVMP治疗后首次发作的严重急性孤立性ON患者的视觉预后。本研究提示，PLEX可能与NMOSD急性视神经炎患者视觉预后改善有关。
