095 ‘No End in Sight’, Management dilemma of refractory MOG antibody positive optic neuritis

Samuel Kwok, James JH Hughes, Dark LD Lisa
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Abstract

Background The patient is a 38-year-old lady who presented with impaired visual acuity in her right eye which was accompanied with pain on extra-ocular movements. Her symptoms initially resolved with high dose steroid therapy. This is on a background of eosinophilic asthma which is refractory to maximal inhaler therapy and IL-5 monoclonal antibody therapy. Methods/Results The patient had unremarkable blood results and inflammatory makers and a normal CSF study. She was subsequently found to be myelin oligodendrocyte glycoprotein (MOG) antibody positive but negative for (Aquaporin -4) AQP-4 antibodies. Radiological findings on MRI of the brain and spine was suggestive of multiple sclerosis (MS). Whilst receiving intravenous immunoglobulin (IVIG) as a bridging therapy to Multiple Sclerosis (MS) modifying therapy, the patient developed severe adverse reaction to IVIG with desquamation of her hands and legs. Furthermore, she did not achieve satisfactory disease control with Ocrelizumab. Has cushingoid features secondary to long term high dose steroid therapy. Conclusions To highlight the challenges and difficulty in managing a patient with refractory MOG antibody optic neuritis and its impact on the quality of life of the patient. To demonstrate the gap in available clinical evidence in the management of recurrent MOG antibody positive optic neuritis. To initiate an open discussion regarding the treatment of these challenging group of patients References Chen JJ, Tobin WO, Majed M, Jitprapaikulsan J, Fryer JP, Leavitt JA, Flanagan EP, McKeon A, Pittock SJ. Prevalence of myelin oligodendrocyte glycoprotein and aquaporin-4-IgG in patients in the optic neuritis treatment trial. JAMA ophthalmology 2018;136(4):419–422. Chen JJ, Flanagan EP, Bhatti MT, Jitprapaikulsan J, Dubey D, Lopez Chiriboga ASS, Fryer JP, Weinshenker BG, McKeon A, Tillema J-M, Lennon VA, Lucchinetti CF, Kunchok A. Steroid-sparing maintenance immunotherapy for MOG-IgG associated disorder. Neurology 2020;95(2):e111–e120.
[95]《看不见尽头》,难治性MOG抗体阳性视神经炎的治疗困境
患者是一名38岁的女性,她表现为右眼视力受损,并伴有眼外运动疼痛。她的症状最初通过大剂量类固醇治疗得到缓解。这是在嗜酸性粒细胞性哮喘的背景下,这是难治性的最大吸入器治疗和IL-5单克隆抗体治疗。方法/结果患者血检及炎性因子无明显变化,脑脊液检查正常。随后发现髓鞘少突胶质细胞糖蛋白(MOG)抗体阳性,但(水通道蛋白-4)AQP-4抗体阴性。脑部和脊柱的MRI表现提示多发性硬化症(MS)。在接受静脉注射免疫球蛋白(IVIG)作为多发性硬化症(MS)修饰治疗的桥接治疗时,患者出现了严重的免疫球蛋白不良反应,手部和腿部出现脱皮。此外,她使用Ocrelizumab并没有达到令人满意的疾病控制。具有库欣样特征继发于长期大剂量类固醇治疗。结论强调难治性MOG抗体视神经炎患者治疗的挑战和困难及其对患者生活质量的影响。目的探讨MOG抗体阳性视神经炎复发治疗的临床证据差异。为了对这些具有挑战性的患者群体的治疗展开公开讨论参考文献Chen JJ, Tobin WO, Majed M, Jitprapaikulsan J, Fryer JP, Leavitt JA, Flanagan EP, McKeon A, Pittock SJ。视神经炎治疗试验中髓鞘少突胶质细胞糖蛋白和水通道蛋白-4- igg的流行。中华眼科杂志,2018;36(4):419-422。Chen JJ, Flanagan EP, Bhatti MT, Jitprapaikulsan J, Dubey D, Lopez Chiriboga ASS, Fryer JP, Weinshenker BG, McKeon A, Tillema J m, Lennon VA, Lucchinetti CF, Kunchok A. MOG-IgG相关疾病的激素保留维持免疫治疗。神经学2020;95 (2):e111-e120。
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