NEUROMYELITIS OPTICA SPECTRUM DISORDER (NMOSD) DENGAN ANTIBODI AQP4 POSITIF

Rima Febry Lesmana, A. Triningrat, Made Paramita Wijayanti, I. Kusumadjaja, I. Indrayani, Gede Kambayana
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Abstract

Background: Neuromyelitis optica (NMO) is an in?ammatory demyelinating autoimmune disease of the central nervous system that most commonly affects the optic nerves and spinal cord. Seropositive antiAQP4 differentiates NMO from MS and the presence of manifestation in the postrema, brainsteam or diencephalic areas extend to NMO Spectrum Disorder (NMOSD). Case Description: A 18 years old male complain sudden vision loss on his left eye since 2 weeks ago. The examination show the visual acuity on the right eye was 6/6 and LPBP on the left eye. Positive RAPD on the left eye, funduscopy and the OCT RNFL within normal limits. Head MRI focus orbita with contrast show optic neuritis. Patient was diagnosed with left eye retrobulbar optic neuritis and ONTT therapy was given. The visual acuity improved to 1/60 then therapy change to oral steroid. Four months later, the patient suddenly got vision loss on the right eye accompanied by paraparesis. The visual acuity on the right eye was NLP and the left eye was 1/300, with mid-dilated papil. The results of the OCT RNFL show on the right eye edema papil and left eye atrophy papil. An MRI of the head focus orbital and whole spain was reexamined followed by VEP examination and an AntiAQP4 which showed an NMOSD. He was given ONTT then continued with immunosuppressants. The visual acuity of the right eye improved to 3/60 and the left eye remained 1/300. Discussion: This patient first presented with complain on the left eye with clinical and supporting symptoms suggesting an optic neuritis. The presence of a new attack on the right eye with paraparesis is a clinical feature of NMO supported by MRI results and seropositive AQP4 indicates an NMOSD. Conclusion: Establishment of diagnosis and administration of therapy quickly and precisely can reduce the severity and risk of recurrence which leads to greater disability and blindness. Key Words: Neuromyelitis Optica, Neuromyelitis Optica Spectrum Disorder, AQP4
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背景:视神经脊髓炎(NMO)是一种慢性疾病。炎性脱髓鞘性自身免疫性疾病的中枢神经系统,最常见的影响视神经和脊髓。血清抗aqp4阳性可将NMO与MS区分开来,且在脑后、脑蒸汽或间脑区出现表现可扩展为NMO谱系障碍(NMOSD)。病例描述:一名18岁男性,自2周前以来左眼突然失明。检查显示右眼视力为6/6,左眼LPBP。左眼RAPD阳性,眼底镜及OCT RNFL正常。头部MRI聚焦眼眶造影显示视神经炎。患者被诊断为左眼球后视神经炎,给予ONTT治疗。视力改善至1/60后改为口服类固醇治疗。4个月后,患者突然出现右眼视力减退,并伴有截瘫。右眼视力为NLP,左眼视力为1/300,乳头中等扩张。OCT RNFL结果显示右眼乳头水肿,左眼乳头萎缩。复查头部病灶、眼眶和整个西班牙的MRI,随后复查VEP检查和AntiAQP4检查,结果显示NMOSD。他被给予ONTT,然后继续使用免疫抑制剂。右眼视力提高到3/60,左眼视力保持1/300。讨论:该患者首先表现为左眼主诉,临床和支持症状提示视神经炎。右眼出现新的发作伴截瘫是NMO的临床特征,MRI结果支持,血清AQP4阳性提示NMOSD。结论:快速准确的诊断和治疗可以降低复发的严重程度和风险,从而减少致残和致盲。关键词:视神经脊髓炎,视神经脊髓炎频谱障碍,AQP4
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