Wirrom-Jorrie Sally, Karaca Anara, Canessa Carlos
General Internal Medicine, King's College Hospital NHS Foundation Trust, London, GBR.
Cureus. 2025 Jul 7;17(7):e87410. doi: 10.7759/cureus.87410. eCollection 2025 Jul.
Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon autoimmune neurological condition that is rarely associated with vomiting as the only initial symptom. We report a 67-year-old Caribbean female who was admitted to the general medicine ward with intractable vomiting for about two weeks. Her condition two weeks later progressed to brainstem syndrome, progressive dysphagia, and slurred speech. A month from admission, she developed quadriparesis, symptoms initially suggestive of a stroke. The underlying neurological diagnosis remained unclear for some time. Her initial symptoms were attributed to viral gastroenteritis, later with gastroscopy to esophagitis and subsequently to a possible stroke, which brain CT and MRI ruled out. However, a brain MRI with contrast revealed longitudinal and extensive hyperintense T2 changes in the brainstem, including the area postrema (lower medulla) and the cervical region. Ultimately, after more than a month of her admission, she was diagnosed with NMOSD, confirmed by the presence of aquaporin-4 antibodies in the cerebrospinal fluid via lumbar puncture. She received high-dose corticosteroid therapy and plasma exchange as acute treatment, followed by maintenance with mycophenolate. This led to improvements in her speech and swallowing; however, the improvement in her muscle weakness was not that prominent. After a long hospital admission, a patient was transferred to a neurorehabilitation centre to support recovery and enhance muscle strength, along with immunosuppressive treatment. During her extended hospital stay, the patient required nasogastric feeding, and her course was complicated by recurrent chest and urinary tract infections. This case highlights the atypical presentation and diagnostic challenges of NMOSD. We hope this report will help clinicians consider NMOSD earlier in similar clinical scenarios.
视神经脊髓炎谱系障碍(NMOSD)是一种罕见的自身免疫性神经系统疾病,很少以呕吐作为唯一的初始症状。我们报告一名67岁的加勒比女性,因顽固性呕吐入院,在普通内科病房治疗了约两周。两周后,她的病情发展为脑干综合征、进行性吞咽困难和言语不清。入院一个月后,她出现四肢瘫痪,最初症状提示中风。一段时间内,潜在的神经学诊断仍不明确。她最初的症状被归因于病毒性肠胃炎,后来经胃镜检查诊断为食管炎,随后又怀疑是中风,但脑部CT和MRI排除了中风。然而,增强脑部MRI显示脑干出现纵向和广泛的T2高信号改变,包括最后区(延髓下部)和颈部区域。最终,入院一个多月后,她被诊断为NMOSD,腰椎穿刺脑脊液中检测到水通道蛋白-4抗体得以确诊。她接受了大剂量皮质类固醇治疗和血浆置换作为急性治疗,随后用霉酚酸酯维持治疗。这使她的言语和吞咽功能有所改善;然而,肌肉无力的改善并不显著。经过长时间住院后,患者被转至神经康复中心,在进行免疫抑制治疗的同时,支持康复并增强肌肉力量。在她延长的住院期间,患者需要鼻饲,病程中并发反复的胸部和尿路感染。本病例突出了NMOSD的非典型表现和诊断挑战。我们希望本报告能帮助临床医生在类似临床情况下更早地考虑到NMOSD。