Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
Department of Molecular Neurology and Therapeutics, Kumamoto University Hospital, Kumamoto, Japan.
Eur J Neurol. 2021 Aug;28(8):2574-2581. doi: 10.1111/ene.14901. Epub 2021 May 25.
Neuromyelitis optica spectrum disorder (NMOSD) often presents in the elderly with an insidious onset of symptoms and aggressive progression. There have been anecdotal cases of very late onset (VLO)-NMOSD, but case series reports are rare. The aim of this retrospective study was to clarify the clinical features of VLO-NMOSD.
According to the age at onset, we classified patients with NMOSD into three subgroups: ≤49 years, early onset NMOSD (EO-NMOSD); 50-69 years, late onset NMOSD (LO-NMOSD); and ≥70 years, VLO-NMOSD. We evaluated the clinical characteristics, magnetic resonance imaging (MRI) findings, laboratory data, and immunotherapies of the groups.
Overall, 12 men and 64 women with a median (interquartile range) age at onset and duration of disease of 42.0 (29.0-55.8) years and 70.0 (16.3-143.0) months, respectively, were included. Eight (11%) patients had VLO-NMOSD, 22 (29%) had LO-NMOSD, and 46 (61%) had EO-NMOSD. Patients with EO-NMOSD had a significantly longer interval between episodes as well as time between the first symptom and diagnosis of NMOSD than did those with VLO-NMOSD and LO-NMOSD (p = 0.046). Optic neuritis and nerve lesions on MRI were significantly less frequent in patients with VLO-NMOSD than in those with LO-NMOSD and EO-NMOSD (p = 0.002 and p = 0.028, respectively). In contrast, patients with VLO-NMOSD had higher nadir Expanded Disability Status Scale and Nurick scale scores and a significantly longer spinal lesion length than did those with LO-NMOSD and EO-NMOSD (p = 0.029, p = 0.049, and p = 0.032, respectively).
Patients with VLO-NMOSD tend to develop severe myelitis with long cord lesions but not optic neuritis.
视神经脊髓炎谱系疾病（NMOSD）常发生于老年人，起病隐匿，进展迅速。有极晚发（VLO）-NMOSD 的个案报道，但病例系列报告较为罕见。本回顾性研究旨在阐明 VLO-NMOSD 的临床特征。
根据发病年龄，我们将 NMOSD 患者分为三组：≤49 岁为早发 NMOSD（EO-NMOSD）；50-69 岁为晚发 NMOSD（LO-NMOSD）；≥70 岁为极晚发 NMOSD（VLO-NMOSD）。我们评估了各组的临床特征、磁共振成像（MRI）表现、实验室数据和免疫治疗情况。
共有 12 名男性和 64 名女性纳入研究，中位（四分位距）发病年龄和病程分别为 42.0（29.0-55.8）岁和 70.0（16.3-143.0）个月。8 名（11%）患者为 VLO-NMOSD，22 名（29%）为 LO-NMOSD，46 名（61%）为 EO-NMOSD。EO-NMOSD 患者的发作间隔和首发症状至 NMOSD 诊断时间均明显长于 VLO-NMOSD 和 LO-NMOSD 患者（p=0.046）。VLO-NMOSD 患者的视神经炎和 MRI 神经病变明显少于 LO-NMOSD 和 EO-NMOSD 患者（p=0.002 和 p=0.028）。相反，VLO-NMOSD 患者的最低扩展残疾状况量表和 Nurick 评分以及脊髓病变长度均明显长于 LO-NMOSD 和 EO-NMOSD 患者（p=0.029、p=0.049 和 p=0.032）。
VLO-NMOSD 患者易发生严重的长脊髓炎，但无视神经炎。
