Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom.
Department of Neuroradiology, Oxford University Hospitals National Health Service Trust, Oxford, United Kingdom.
JAMA Netw Open. 2019 Oct 2;2(10):e1912732. doi: 10.1001/jamanetworkopen.2019.12732.
Recognizing the differences between transverse myelitis (TM) associated with myelin oligodendrocyte glycoprotein (MOG) antibody (Ab) disease vs aquaporin-4 (AQP4)-Ab disease and prognosticating patients within each group may be an important factor for better clinical treatment for these respective patients.
To compare the clinical and radiological findings of the first TM episode in patients with MOG-Ab disease vs patients with AQP4-Ab disease and to assess factors associated with worse outcomes and relapse risk.
DESIGN, SETTING, AND PARTICIPANTS: This retrospective cross-sectional study used data collected from the Oxford Neuromyelitis Optica Service database, a national service that serves the south of England, including detailed clinical data, and high-quality imaging from within 4 weeks of the first TM episode from patients with MOG-Ab disease or AQP4-Ab disease and a confirmed history of TM from April 2018 to January 2019. Data analyses were conducted from February 2019 to April 2019.
Onset features of each condition measured using the Expanded Disability Status Scale (EDSS) score, time to an EDSS score of 6, time to relapse, and residual sphincter dysfunction at least 6 months after the first TM episode and at last follow-up.
The total cohort included 115 adult patients, including 46 patients with MOG-Ab disease and 69 patients with AQP4-Ab disease. Patients with AQP4-Ab disease, compared with patients with MOG-Ab disease, tended to be older at onset of disease (mean [SD] age, 48.5 [14.9] years vs 33.7 [1.2] years) and female (57 [83%] women vs 24 [52%] women). Transverse myelitis occurred at onset of disease for 32 patients (70%) with MOG-Ab disease and 57 patients (78%) with AQP4-Ab disease. Onset severity did not differ between groups. An acute disseminated encephalomyelitis-like presentation occurred at the time of the TM in 4 patients (9%) with MOG-Ab disease but no patients with AQP4-Ab disease. Compared with patients with AQP4-Ab disease, patients with MOG-Ab disease were more likely to have short cord lesions (22 patients [48%] vs 10 patients [15%]; P < .001) and multiple cord lesions (18 patients [39%] vs 7 patients [10%]; P < .001). Approximately 50% of patients with MOG-Ab disease had only short cord lesions when the TM occurred as a relapse. Median (range) recovery EDSS score was lower in patients with MOG-Ab disease than patients with AQP4-Ab disease (1.8 [1.0-8.0] vs 3.0 [1.0-8.0]). Persistent bladder dysfunction associated with an increased prevalence of conus lesions occurred more frequently in patients with MOG-Ab disease than in patients with AQP4-Ab disease (27 patients [59%] vs 33 patients [48%]). Long-term catheter requirement was roughly equal between groups (9 patients [20%] vs 16 patients [23%]). Relapses after TM occurred in 17 patients with MOG-Ab disease (37%) and 36 patients with AQP4-Ab disease (52%). Concomitant brainstem lesions in patients with MOG-Ab disease were associated with a higher mean (SD) EDSS score at recovery (3.5 [2.3] vs 1.4 [0.9]; P < .001). In patients with AQP4-Ab disease, those younger than 50 years were more likely to relapse (27 of 36 patients aged <50 years [75%] vs 9 of 33 patients aged ≥50 years [27%]; P < .001) and those 50 years and older were more likely to reach an EDSS score of 6 (19 of 33 patients aged ≥50 years [58%] vs 11 of 36 patients aged <50 years [31%]; P = .03).
This study found that in patients who experienced a TM episode, short and multiple lesions at onset were more common in those with MOG-Ab disease than among those with AQP4-Ab disease. The presence of a brainstem lesion at the time of a TM episode in patients with MOG-Ab disease was associated with a worse recovery. In patients with AQP4-Ab disease, those 50 years and older at disease onset had more disability, and those younger than 50 years at disease onset had more relapses.
识别与髓鞘少突胶质细胞糖蛋白(MOG)抗体(Ab)疾病相关的横向脊髓炎(TM)与水通道蛋白-4(AQP4)-Ab 疾病之间的差异,以及对每个组内的患者进行预后,可能是为这些患者提供更好临床治疗的重要因素。
比较 MOG-Ab 病与 AQP4-Ab 病患者首次 TM 发作的临床和影像学表现,并评估与预后不良和复发风险相关的因素。
设计、地点和参与者:本回顾性横断面研究使用了来自牛津神经免疫性视神经脊髓炎服务数据库的数据,该数据库是一个服务于英格兰南部的全国性服务,包括详细的临床数据和来自 2018 年 4 月至 2019 年 1 月 MOG-Ab 病或 AQP4-Ab 病患者首次 TM 发作后 4 周内的高质量影像学检查,这些患者均有 TM 病史。数据分析于 2019 年 2 月至 2019 年 4 月进行。
使用扩展残疾状况量表(EDSS)评分衡量每种疾病的发病特征、EDSS 评分达到 6 的时间、复发时间以及首次 TM 发作后至少 6 个月和最后一次随访时的残留括约肌功能障碍。
总队列包括 115 名成年患者,其中 46 名患者患有 MOG-Ab 病,69 名患者患有 AQP4-Ab 病。与 MOG-Ab 病患者相比,AQP4-Ab 病患者的疾病发病年龄更大(平均[标准差]年龄,33.7[1.2]岁 vs 48.5[14.9]岁),且女性更多(57[83%]名女性 vs 24[52%]名女性)。32 名(70%)MOG-Ab 病患者和 57 名(78%)AQP4-Ab 病患者在疾病发病时发生 TM。两组的发病严重程度无差异。4 名(9%)MOG-Ab 病患者在 TM 时出现急性播散性脑脊髓炎样表现,而 AQP4-Ab 病患者无此表现。与 AQP4-Ab 病患者相比,MOG-Ab 病患者更有可能出现短脊髓病变(22 名[48%] vs 10 名[15%];P < .001)和多个脊髓病变(18 名[39%] vs 7 名[10%];P < .001)。当 TM 作为复发病例发生时,约 50%的 MOG-Ab 病患者仅存在短脊髓病变。MOG-Ab 病患者的中位(范围)恢复 EDSS 评分低于 AQP4-Ab 病患者(1.8[1.0-8.0] vs 3.0[1.0-8.0])。MOG-Ab 病患者更常出现与圆锥病变相关的持续性膀胱功能障碍,发生率高于 AQP4-Ab 病患者(27 名[59%] vs 33 名[48%])。两组长期置管需求大致相当(9 名[20%] vs 16 名[23%])。17 名 MOG-Ab 病患者(37%)和 36 名 AQP4-Ab 病患者(52%)发生 TM 后复发。MOG-Ab 病患者同时存在脑干病变与恢复时更高的平均(标准差)EDSS 评分相关(3.5[2.3] vs 1.4[0.9];P < .001)。在 AQP4-Ab 病患者中,年龄小于 50 岁的患者更有可能复发(36 名年龄小于 50 岁的患者中有 27 名[75%]复发 vs 33 名年龄大于 50 岁的患者中有 9 名[27%]复发;P < .001),年龄大于 50 岁的患者更有可能达到 EDSS 评分 6(33 名年龄大于 50 岁的患者中有 19 名[58%]达到 EDSS 评分 6 vs 36 名年龄小于 50 岁的患者中有 11 名[31%]达到 EDSS 评分 6;P = .03)。
本研究发现,在经历 TM 发作的患者中,MOG-Ab 病患者的首发时更常见短脊髓病变和多个脊髓病变。MOG-Ab 病患者 TM 发作时存在脑干病变与预后不良相关。在 AQP4-Ab 病患者中,发病时年龄大于 50 岁的患者残疾程度更高,而发病时年龄小于 50 岁的患者更易复发。