Campbell Amy, Ogundipe Olayinka Ayodele
Medicine of the Elderly Department, Royal Infirmary of Edinburgh, Edinburgh, UK.
BMJ Case Rep. 2018 Sep 8;2018:bcr-2018-225601. doi: 10.1136/bcr-2018-225601.
This case describes an 81-year-old woman with a history of Sjögren's syndrome presenting with recurrent falls and poor balance. She subsequently developed new and rapidly evolving neurology including hyperaesthesia, spastic paraplegia and sphincteric dysfunction. Following serial clinical reviews and detailed investigations, MRI (brainstem and cervicothoracic spine) and a seropositive result for aquaporin 4 IgG, a diagnosis of neuromyelitis optica spectrum disorder (NMOSD) was made. This case describes the clinical course of this index patient with an unusual late age of onset. The report also includes a discussion on NMOSD. We review aspects of terminology, brief epidemiology, pathogenesis, notable autoimmune associations, variance in clinical presentation and current diagnostic criteria. We also review the importance of distinguishing NMOSD from multiple sclerosis in view of the significant implications for treatment and prognosis.
本病例描述了一位81岁患有干燥综合征的女性,她反复跌倒且平衡能力差。随后,她出现了新的且迅速发展的神经症状,包括感觉过敏、痉挛性截瘫和括约肌功能障碍。经过一系列临床检查和详细的调查,磁共振成像(脑干和颈胸段脊柱)以及水通道蛋白4免疫球蛋白G血清学阳性结果,最终诊断为视神经脊髓炎谱系障碍(NMOSD)。本病例描述了该首例患者不寻常的晚发临床病程。报告还包括对视神经脊髓炎谱系障碍的讨论。我们回顾了术语、简要流行病学、发病机制、显著的自身免疫关联、临床表现差异和当前诊断标准等方面。鉴于对治疗和预后的重大影响,我们还回顾了区分视神经脊髓炎谱系障碍与多发性硬化症的重要性。