PDF(Pubmed)
Abstract：
OBJECTIVE: We aimed to characterize hypothalamic involvement in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and compare it with neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS).
METHODS: A retrospective study was performed to identify hypothalamic lesions in patients diagnosed with MOGAD, NMOSD, or MS from January 2013 to May 2020. The demographic, clinical, and radiological features were recorded. Hypothalamic dysfunction and prognosis were assessed through physical examination, biochemical testing, sleep monitoring, and magnetic resonance imaging.
RESULTS: Hypothalamic lesions were observed in seven of 96 patients (7.3%) with MOGAD, 34 of 536 (6.3%) with NMOSD, and 16 of 356 (4.5%) with MS (p = 0.407). The time from disease onset to development of hypothalamic lesions was shortest in MOGAD (12 months). The frequency of bilateral hypothalamic lesions was the lowest in MOGAD (p = 0.008). The rate of hypothalamic dysfunction in MOGAD was 28.6%, which was lower than that in NMOSD (70.6%) but greater than that in MS patients (18.8%; p = 0.095 and p = 0.349, respectively). Hypothalamic dysfunction in MOGAD manifests as hypothalamic-pituitary-adrenal axis dysfunction and hypersomnia. The proportion of complete regression of hypothalamic lesions in MOGAD (100%) was much greater than that in NMOSD (41.7%) and MS patients (18.2%; p = 0.007 and p = 0.001, respectively). An improvement in hypothalamic dysfunction was observed in all MOGAD patients after immunotherapy.
CONCLUSIONS: MOGAD patients have a relatively high incidence of asymptomatic hypothalamic lesions. The overall prognosis of patients with hypothalamic involvement is good in MOGAD, as the lesions completely resolve, and dysfunction improves after immunotherapy.
METHODS: A retrospective study was performed to identify hypothalamic lesions in patients diagnosed with MOGAD, NMOSD, or MS from January 2013 to May 2020. The demographic, clinical, and radiological features were recorded. Hypothalamic dysfunction and prognosis were assessed through physical examination, biochemical testing, sleep monitoring, and magnetic resonance imaging.
RESULTS: Hypothalamic lesions were observed in seven of 96 patients (7.3%) with MOGAD, 34 of 536 (6.3%) with NMOSD, and 16 of 356 (4.5%) with MS (p = 0.407). The time from disease onset to development of hypothalamic lesions was shortest in MOGAD (12 months). The frequency of bilateral hypothalamic lesions was the lowest in MOGAD (p = 0.008). The rate of hypothalamic dysfunction in MOGAD was 28.6%, which was lower than that in NMOSD (70.6%) but greater than that in MS patients (18.8%; p = 0.095 and p = 0.349, respectively). Hypothalamic dysfunction in MOGAD manifests as hypothalamic-pituitary-adrenal axis dysfunction and hypersomnia. The proportion of complete regression of hypothalamic lesions in MOGAD (100%) was much greater than that in NMOSD (41.7%) and MS patients (18.2%; p = 0.007 and p = 0.001, respectively). An improvement in hypothalamic dysfunction was observed in all MOGAD patients after immunotherapy.
CONCLUSIONS: MOGAD patients have a relatively high incidence of asymptomatic hypothalamic lesions. The overall prognosis of patients with hypothalamic involvement is good in MOGAD, as the lesions completely resolve, and dysfunction improves after immunotherapy.
摘要：
目的：我们旨在表征下丘脑受累于髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD），并将其与视神经脊髓炎谱系障碍（NMOSD）和多发性硬化（MS）进行比较。
方法：进行了一项回顾性研究，以确定诊断为MOGAD的患者的下丘脑病变，NMOSD,或MS从2013年1月到2020年5月。人口统计，临床,并记录了放射学特征。通过体格检查评估下丘脑功能障碍和预后。生化检测,睡眠监测,和磁共振成像。
结果：在96例MOGAD患者中有7例（7.3％）观察到下丘脑病变，536人中的34人（6.3%）使用NMOSD，356人中有16人(4.5%)患有MS(p=0.407)。MOGAD从发病到下丘脑病变发展的时间最短(12个月)。MOGAD中，双侧下丘脑病变的频率最低（p=0.008）。MOGAD患者下丘脑功能障碍发生率为28.6%,低于NMOSD（70.6％），但高于MS患者（18.8％；分别为p=0.095和p=0.349）。MOGAD的下丘脑功能障碍表现为下丘脑-垂体-肾上腺轴功能障碍和睡眠过度。MOGAD患者下丘脑病变完全消退的比例（100％）远高于NMOSD（41.7％）和MS患者（18.2％；分别为p=0.007和p=0.001）。免疫治疗后，所有MOGAD患者下丘脑功能障碍均得到改善。
结论：MOGAD患者无症状下丘脑病变的发生率相对较高。MOGAD患者下丘脑受累的总体预后良好，当病变完全消退时，免疫疗法后功能障碍改善。
方法：进行了一项回顾性研究，以确定诊断为MOGAD的患者的下丘脑病变，NMOSD,或MS从2013年1月到2020年5月。人口统计，临床,并记录了放射学特征。通过体格检查评估下丘脑功能障碍和预后。生化检测,睡眠监测,和磁共振成像。
结果：在96例MOGAD患者中有7例（7.3％）观察到下丘脑病变，536人中的34人（6.3%）使用NMOSD，356人中有16人(4.5%)患有MS(p=0.407)。MOGAD从发病到下丘脑病变发展的时间最短(12个月)。MOGAD中，双侧下丘脑病变的频率最低（p=0.008）。MOGAD患者下丘脑功能障碍发生率为28.6%,低于NMOSD（70.6％），但高于MS患者（18.8％；分别为p=0.095和p=0.349）。MOGAD的下丘脑功能障碍表现为下丘脑-垂体-肾上腺轴功能障碍和睡眠过度。MOGAD患者下丘脑病变完全消退的比例（100％）远高于NMOSD（41.7％）和MS患者（18.2％；分别为p=0.007和p=0.001）。免疫治疗后，所有MOGAD患者下丘脑功能障碍均得到改善。
结论：MOGAD患者无症状下丘脑病变的发生率相对较高。MOGAD患者下丘脑受累的总体预后良好，当病变完全消退时，免疫疗法后功能障碍改善。
