Department of Neurology, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.
Department of Neurology, Maoming People's Hospital, 101 Weimin Road, Maonan District, Maoming, Guangdong, China.
BMC Neurol. 2021 Jan 25;21(1):35. doi: 10.1186/s12883-021-02059-1.
Early stage neuromyelitis optica spectrum disorders (NMOSD) with non-opticospinal manifestations as initial symptoms are easily misdiagnosed; however, data on the full symptom profile are limited. Moreover, the clinical characteristics and long-term outcomes of these patients remain unknown. We sought to analyze the clinical characteristics, imaging features, and long-term outcomes of NMOSD with non-opticospinal manifestations as initial symptoms.
We retrospectively included relevant patients from our center. Clinical, demographic, magnetic resonance imaging, treatment, and outcome data were compared according to the non-opticospinal vs. opticospinal initial symptoms.
We identified 43 (9.13 %) patients with non-opticospinal initial symptoms among 471 patients with NMOSD. Of these, 88.37 % developed optic neuritis/myelitis during an average follow-up period of 6.33 years. All the non-opticospinal symptoms were brain/brainstem symptoms. Most of the symptoms and associated brain lesions were reversible. These patients had a younger onset age (P < 0.001), lower serum aquaporin-4 (AQP4) antibody titers (P = 0.030), and a lower Expanded Disability Status Scale (EDSS) score at onset (P < 0.001) and follow-up (P = 0.041) than NMOSD patients with opticospinal initial symptoms. In addition, EDSS scores reached 3.0 (indicating moderate disability) later than in patients with opticospinal initial symptoms (P = 0.028).
Patients with NMOSD with non-opticospinal initial symptoms have a younger onset age, lower serum AQP4 antibody titers, and better clinical outcomes.
以非视神经脊髓炎谱疾病（NMOSD）为初始症状的早期 NMOSD 缺乏视神经脊髓炎表现，易误诊；然而，此类患者的完整症状谱数据有限。此外，这些患者的临床特征和长期结局仍不清楚。我们旨在分析以非视神经脊髓炎谱疾病为初始症状的 NMOSD 的临床特征、影像学特征和长期结局。
我们回顾性纳入了来自本中心的相关患者。根据非视神经脊髓炎谱 vs. 视神经脊髓炎谱初始症状，比较了临床、人口统计学、磁共振成像、治疗和结局数据。
在 471 例 NMOSD 患者中，我们发现了 43 例（9.13%）以非视神经脊髓炎谱疾病为初始症状的患者。在平均 6.33 年的随访中，88.37%的患者发展为视神经炎/脊髓炎。所有非视神经脊髓炎谱疾病的症状均为脑/脑干部位症状。大多数症状和相关脑病变是可逆的。这些患者的发病年龄更轻（P < 0.001），血清水通道蛋白 4（AQP4）抗体滴度更低（P = 0.030），且发病时（P < 0.001）和随访时（P = 0.041）的扩展残疾状况量表（EDSS）评分更低。此外，EDSS 评分达到 3.0（表示中度残疾）的时间晚于视神经脊髓炎谱疾病患者（P = 0.028）。
以非视神经脊髓炎谱疾病为初始症状的 NMOSD 患者发病年龄更轻，血清 AQP4 抗体滴度更低，临床结局更好。
