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视神经脊髓炎(NMO)中慢性炎性髓鞘变性的非典型表现

An Atypical Presentation of Chronic Inflammatory Myelin Degeneration in Neuromyelitis Optica (NMO).

作者信息

Allu Sai Vishnu Vardhan, Parikh Harsh R, Schmidt Patrik, Alonso Gabriel, Khanal Sneha, Fortuzi Ked, Khaja Misbahuddin

机构信息

Internal Medicine, BronxCare Health System, Bronx, USA.

Internal Medicine and Pulmonary Critical Care, Icahn School of Medicine at Mount Sinai/BronxCare Health System, Bronx, USA.

出版信息

Cureus. 2023 Jul 13;15(7):e41803. doi: 10.7759/cureus.41803. eCollection 2023 Jul.

DOI:10.7759/cureus.41803
PMID:37575757
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10422679/
Abstract

Neuromyelitis optica (NMO) is an autoimmune disorder characterized by aquaporin-4 (AQP4) IgG autoantibodies. These autoantibodies induce chronic neuroinflammatory damage to the spinal cord and optic nerve. NMO clinically manifests as relapsing and overlapping neurodegenerative episodes of optic neuritis (ON) and transverse myelitis (TM). Contrasting from other autoimmune neurodegenerative disorders, NMO has a poor prognostic profile often involving permanent neurological disability. We present a case of a 65-year-old male who presented with a progressive weakening in his left upper and lower extremities with reduced sensation and was found to have an acute flare of NMO. We explore the broad symptomatology involved in the disorder along with relevant crucial imaging findings pointing toward the diagnosis of NMO. Finally, we discuss treatment modalities in the context of our patient's clinical course and prognostic factors. Early intervention and suppression of relapse in this neuroinflammatory neurodegenerative disorder can help decrease the duration of acute flares and improve long-term outcomes for patients affected by NMO.

摘要

视神经脊髓炎(NMO)是一种以水通道蛋白4(AQP4)IgG自身抗体为特征的自身免疫性疾病。这些自身抗体可对视神经和脊髓造成慢性神经炎性损伤。NMO的临床表现为视神经炎(ON)和横贯性脊髓炎(TM)反复发作且相互重叠的神经退行性发作。与其他自身免疫性神经退行性疾病不同,NMO的预后较差,常导致永久性神经功能残疾。我们报告一例65岁男性患者,该患者出现左上肢和下肢进行性无力伴感觉减退,被诊断为NMO急性发作。我们探讨了该疾病所涉及的广泛症状以及有助于诊断NMO的相关关键影像学表现。最后,我们结合患者的临床病程和预后因素讨论了治疗方式。在这种神经炎性神经退行性疾病中,早期干预和抑制复发有助于缩短急性发作的持续时间,并改善NMO患者的长期预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/778d/10422679/f2084e312a37/cureus-0015-00000041803-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/778d/10422679/f2084e312a37/cureus-0015-00000041803-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/778d/10422679/f2084e312a37/cureus-0015-00000041803-i01.jpg

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