Kang Qingyun, Kang Hui, Liu Shulei, Feng Mei, Zhou Zhen, Jiang Zhi, Wu Liwen
Department of Neurology, Hunan Children's Hospital, Changsha, China.
Department of Orthopaedics, General Hospital of Central Theater Command, Wuhan, Hubei, China.
Front Neurol. 2024 Jan 5;14:1279211. doi: 10.3389/fneur.2023.1279211. eCollection 2023.
The cases of MOG-AD (MOG antibody-associated disorder) and anti-NMDAR encephalitis overlapping syndrome (MNOS) are rare, especially among pediatric patients, and their clinical understanding is limited. This study aimed to investigate the clinical manifestations, imaging findings, treatments, and prognosis of Chinese pediatric patients who tested positive for anti-NMDAR and MOG antibodies.
This retrospective study enrolled 10 MNOS pediatric patients, 50 MOG-AD (anti-NMDAR antibody-negative), and 81 anti-NMDAR encephalitis (MOG antibody-negative) pediatric patients who were admitted from July 2016 to June 2022 and used their clinical data for comparison.
The MNOS patients had a significantly lower incidence of psycho-behavioral abnormalities and involuntary movements than anti-NMDAR antibody (+)/MOG antibody (-) patients and had a significantly higher incidence of sleep disorders, seizures, and psycho-behavioral abnormalities than MOG antibody (+)/anti-NMDAR antibody (-) patients. The MNOS patients had a significantly higher incidence of MRI abnormalities than the anti-NMDAR antibody (+)/MOG antibody (-) patients, while there was no significant difference in the incidence between the MNOS patients and the MOG antibody (+)/anti-NMDAR antibody (-) patients. No significant difference was seen in the initial mRS score between the three groups of patients. The anti-NMDAR antibody (+)/MOG antibody (-) patients had a higher rate of admission to the ICU, a longer length of in-hospital stay, and a higher rate of introduction to second-line treatment than the other two groups of patients. No significant difference was seen in the mRS score at the last follow-up and in the disease recurrence rate between the three groups. All these patients respond well to immunosuppressive therapy.
In the presence of psycho-behavioral abnormalities, sleep disorders, and frequent seizures in MOG-AD patients or demyelinating symptoms of the central nervous system or demyelinating lesions on head MRI in anti-NMDAR encephalitis patients, the coexistence of MOG and anti-NMDAR antibodies should be considered and would suggest a diagnosis of MNOS for these patients. Immunotherapy is effective among these patients and should be given possibly earlier.
MOG抗体相关疾病（MOG-AD）和抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎重叠综合征（MNOS）的病例较为罕见，尤其是在儿科患者中，人们对其临床了解有限。本研究旨在调查抗NMDAR和MOG抗体检测呈阳性的中国儿科患者的临床表现、影像学表现、治疗方法及预后情况。
本回顾性研究纳入了2016年7月至2022年6月期间收治的10例MNOS儿科患者、50例MOG-AD（抗NMDAR抗体阴性）儿科患者和81例抗NMDAR脑炎（MOG抗体阴性）儿科患者，并使用他们的临床数据进行比较。
与抗NMDAR抗体阳性/MOG抗体阴性患者相比，MNOS患者出现精神行为异常和不自主运动的发生率显著更低；与MOG抗体阳性/抗NMDAR抗体阴性患者相比，MNOS患者出现睡眠障碍、癫痫发作和精神行为异常的发生率显著更高。与抗NMDAR抗体阳性/MOG抗体阴性患者相比，MNOS患者MRI异常的发生率显著更高，而MNOS患者与MOG抗体阳性/抗NMDAR抗体阴性患者之间的发生率无显著差异。三组患者的初始改良Rankin量表（mRS）评分无显著差异。与其他两组患者相比，抗NMDAR抗体阳性/MOG抗体阴性患者入住重症监护病房（ICU）的比例更高、住院时间更长、接受二线治疗的比例更高。三组患者在末次随访时的mRS评分及疾病复发率无显著差异。所有这些患者对免疫抑制治疗反应良好。
对于MOG-AD患者出现精神行为异常、睡眠障碍和频繁癫痫发作，或抗NMDAR脑炎患者出现中枢神经系统脱髓鞘症状或头部MRI显示脱髓鞘病变的情况，应考虑MOG和抗NMDAR抗体共存，并提示这些患者可诊断为MNOS。免疫治疗对这些患者有效，且应尽早给予。
