目的:评估首例累及脑干的髓磷脂少突胶质细胞糖蛋白(MOG)抗体相关疾病(MOGAD)的特征,并与水通道蛋白4-IgG血清阳性视神经脊髓炎(AQP4-IgG-NMOSD)和多发性硬化症(MS)进行比较。
方法:从2017年到2022年,这项研究确定了MOG-IgG阳性患者在第一次发作中有脑干或脑干和小脑病变。作为一个比较组,纳入AQP4-IgG-NMOSD(n=30)和MS(n=30)的BSIFE患者。
结果:35例(35/146,24.0%)为MOGAD的BSIFE。35例MOGAD患者中有9例(25.7%)发生了孤立的脑干发作,与MS相似(7/30,23.3%),但低于AQP4-IgG-NMOSD(17/30,56.7%,P=0.011)。庞斯(21/35,60.0%),延髓(20/35,57.1%)和小脑中梗(MCP,19/35,54.3%)是受影响最频繁的地区。顽固性恶心(n=7),MOGAD患者发生呕吐(n=8)和打嗝(n=2),末次随访时,MOGAD的EDSS低于AQP4-IgG-NMOSD(P=0.001)。有或没有BSIFE的MOGAD患者在ARR方面没有显着差异(P=0.102),mRS(P=0.823),或EDSS(P=0.598)在最近的随访。除MS(20/30,66.7%)外,MOGAD(13/33,39.4%)和AQP4-IgG-NMOSD(7/24,29.2%)均出现特异性寡克隆条带。在这项研究中,有14名MOGAD患者(40.0%)出现了复发。当脑干参与第一次攻击时,在同一位置发生第二次发作的可能性增加(OR=12.22,95CI2.79~53.59,P=0.001).如果第一个和第二个事件都在脑干,第三个事件可能发生在同一地点(OR=66.00,95CI3.47~1254.57,P=0.005).4例患者在MOG-IgG转阴后复发。
结论:BSIFE发生在24.0%的MOGAD中。Pons,延髓和MCP是最常见的受累区域。顽固性恶心,MOGAD和AQP4-IgG-NMOSD发生呕吐和打嗝,但不是女士MOGAD的预后优于AQP4-IgG-NMOSD。与MS相比,BSIFE可能不表明MOGAD的预后较差。当BSIFE患者,MOGAD帐篷在脑干中再次出现。在MOG-IgG测试变为阴性后,14名复发的MOGAD患者中有4名复发。
OBJECTIVE: To assess the characteristics of Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disorder (MOGAD) with brainstem involvement in the first event (BSIFE) and make comparisons with aquaporin-4-IgG seropositive neuromyelitis optica spectrum disorder (AQP4-IgG-NMOSD) and multiple sclerosis (MS).
METHODS: From 2017 to 2022, this study identified MOG-IgG-positive patients with brainstem or both brainstem and cerebellum lesions in the first episode. As a comparison group, AQP4-IgG-NMOSD (n = 30) and MS (n = 30) patients with BSIFE were enroled.
RESULTS: Thirty-five patients (35/146, 24.0%) were the BSIFE of MOGAD. Isolated brainstem episodes occurred in 9 of the 35 (25.7%) MOGAD patients, which was similar to MS (7/30, 23.3%) but was lower than AQP4-IgG-NMOSD (17/30, 56.7%, P = 0.011). Pons (21/35, 60.0%), medulla oblongata (20/35, 57.1%) and middle cerebellar peduncle (MCP, 19/35, 54.3%) were the most frequently affected areas. Intractable nausea (n = 7), vomiting (n = 8) and hiccups (n = 2) happened in MOGAD patients, but EDSS of MOGAD was lower than AQP4-IgG-NMOSD (P = 0.001) at the last follow-up. MOGAD patients with or without BSIFE did not significantly differ in terms of the ARR (P = 0.102), mRS (P = 0.823), or EDSS (P = 0.598) at the most recent follow-up. Specific oligoclonal bands appeared in MOGAD (13/33, 39.4%) and AQP4-IgG-NMOSD (7/24, 29.2%) in addition to MS (20/30, 66.7%). Fourteen MOGAD patients (40.0%) experienced relapse in this study. When the brainstem was involved in the first attack, there was an increased likelihood of a second attack occurring at the same location (OR=12.22, 95%CI 2.79 to 53.59, P = 0.001). If the first and second events were both in the brainstem, the third event was likely to occur at the same location (OR=66.00, 95%CI 3.47 to 1254.57, P = 0.005). Four patients experienced relapses after the MOG-IgG turned negative.
CONCLUSIONS: BSIFE occurred in 24.0% of MOGAD. Pons, medulla oblongata and MCP were the most frequently involved regions. Intractable nausea, vomiting and hiccups occurred in MOGAD and AQP4-IgG-NMOSD, but not MS. The prognosis of MOGAD was better than AQP4-IgG-NMOSD. In contrast to MS, BSIFE may not indicate a worse prognosis for MOGAD. When patients with BSIFE, MOGAD tent to reoccur in the brainstem. Four of the 14 recurring MOGAD patients relapsed after the MOG-IgG test turned negative.