Department of Neurology, The First Affiliated Hospital of Guangxi Medical University, Nanning, China.
Department of Neurology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.
Eur J Neurol. 2024 Sep;31(9):e16377. doi: 10.1111/ene.16377. Epub 2024 Jun 11.
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).
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.
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.
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.
本研究旨在描述并比较下丘脑在髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）、视神经脊髓炎谱系疾病（NMOSD）和多发性硬化（MS）中的作用。
本研究为回顾性研究，纳入了 2013 年 1 月至 2020 年 5 月期间在我院诊断为 MOGAD、NMOSD 或 MS 的患者，对其下丘脑病变进行分析。记录患者的人口统计学、临床和影像学特征。通过体格检查、生化检测、睡眠监测和磁共振成像评估下丘脑功能障碍和预后。
96 例 MOGAD 患者中有 7 例（7.3%）、536 例 NMOSD 患者中有 34 例（6.3%）和 356 例 MS 患者中有 16 例（4.5%）存在下丘脑病变（p=0.407）。MOGAD 患者从发病到出现下丘脑病变的时间最短（12 个月）。MOGAD 患者双侧下丘脑病变的发生率最低（p=0.008）。MOGAD 患者下丘脑功能障碍的发生率为 28.6%，低于 NMOSD 患者（70.6%）但高于 MS 患者（18.8%；p=0.095 和 p=0.349）。MOGAD 患者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嗜睡。MOGAD 患者中完全缓解下丘脑病变的比例（100%）显著高于 NMOSD 患者（41.7%）和 MS 患者（18.2%；p=0.007 和 p=0.001）。免疫治疗后，所有 MOGAD 患者的下丘脑功能障碍均有改善。
MOGAD 患者存在较高比例的无症状性下丘脑病变。MOGAD 患者下丘脑受累的总体预后良好，病变完全消退，免疫治疗后功能障碍改善。
