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多发性硬化症和视神经脊髓炎谱系疾病的神经功能障碍进行性模式。

Progressive patterns of neurological disability in multiple sclerosis and neuromyelitis optica spectrum disorders.

机构信息

Department of Neurology, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.

Department of Education and Support for Regional Medicine, Tohoku University Hospital, Sendai, Japan.

出版信息

Sci Rep. 2020 Aug 17;10(1):13890. doi: 10.1038/s41598-020-70919-w.

Abstract

The progressive patterns of neurological disability in multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) and the significance of clinical relapses to the progressions of neurological disability in these diseases have not been fully elucidated. In this study, to elucidate the impact of relapses to the progression of accumulated neurological disability and to identify the factors to affect the progression of neurological disability in MS and NMOSD, we followed 62 consecutive MS patients and 33 consecutive NMOSD patients for more than 5 years with the clinical symptoms, relapse occurrence, and Expanded Disability Status Scale (EDSS) in the chronic phase. All enrolled MS patients were confirmed to be negative for serum anti-myelin oligodendrocyte glycoprotein antibody. As a result, patients with NMOSD showed significantly severer neurological disability at 5 years from onset than MS patients. Progression in EDSS score was almost exclusively seen after clinical attacks in NMOSD, whereas progression could be observed apart from relapses in MS. Neurological disability did not change without attacks in NMOSD, whereas it sometimes spontaneously improved or deteriorated apart from relapses in MS (p < 0.001). In patients with MS, those with responsible lesions primarily in spinal cord were more likely to show such spontaneous improvement. In conclusion, clinical deterioration in NMOSD patients is irreversible and almost exclusively takes place at the timing of clinical attacks with stepwise accumulation of neurological disability. Meanwhile, changes in EDSS score can be seen apart from relapses in MS patients. Neurological disability in MS patients is partly reversible, and the patients with disease modifying drugs sometimes present spontaneous improvement of the neurological disability.

摘要

多发性硬化症 (MS) 和视神经脊髓炎谱系障碍 (NMOSD) 的神经功能障碍呈进行性发展,临床复发对这些疾病神经功能障碍的进展意义尚未完全阐明。在这项研究中,为了阐明复发对累积神经功能障碍进展的影响,并确定影响 MS 和 NMOSD 神经功能障碍进展的因素,我们对 62 例连续 MS 患者和 33 例连续 NMOSD 患者进行了超过 5 年的随访,包括临床症状、复发发生情况和慢性期的扩展残疾状态量表 (EDSS)。所有入组的 MS 患者均被证实血清抗髓鞘少突胶质细胞糖蛋白抗体阴性。结果显示,NMOSD 患者在发病后 5 年的神经功能障碍明显比 MS 患者更严重。EDSS 评分的进展几乎仅见于 NMOSD 的临床发作后,而 MS 除了复发外也可观察到进展。NMOSD 无发作时神经功能障碍无变化,而 MS 除了复发外有时会自发改善或恶化 (p<0.001)。在 MS 患者中,那些主要累及脊髓的病变更有可能出现这种自发改善。综上所述,NMOSD 患者的临床恶化是不可逆转的,几乎仅在有临床发作时发生,且神经功能障碍呈逐步累积。同时,MS 患者的 EDSS 评分变化除了与复发有关外,还可能与其他因素有关。MS 患者的神经功能障碍部分是可逆的,且接受疾病修正治疗的患者有时会出现神经功能障碍的自发改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d88/7431838/099956eed771/41598_2020_70919_Fig1_HTML.jpg

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