Yoo Il Han, Kim WooJoong, Shim Youngkyu, Choi Sun Ah, Kim Soo Yeon, Kim Hunmin, Lim Byung Chan, Hwang Hee, Choi Jieun, Kim Ki Joong, Kim Yeseul, Hyun Jae Won, Kim Su Hyun, Choi Kyungho, Kim Ho Jin, Chae Jong Hee
Department of Pediatrics, College of Medicine, The Catholic University of Korea, St. Vincent's Hospital, Suwon, Korea.
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
J Clin Neurol. 2020 Jul;16(3):461-469. doi: 10.3988/jcn.2020.16.3.461.
The myelin oligodendrocyte glycoprotein (MOG) antibody is detected at a high rate in childhood acquired demyelinating syndrome (ADS). This study aimed to determine the diagnostic value of the MOG antibody in ADS and the spectrum of MOG-antibody-positive demyelinating diseases in children.
This study included 128 patients diagnosed with ADS (=94) or unexplained encephalitis (=34). The MOG antibody in serum was tested using an in-house live-cell-based immunofluorescence assay.
The MOG antibody was detected in 48 patients (46 ADS patients and 2 encephalitis patients, comprising 23 males and 25 females). Acute disseminated encephalomyelitis (ADEM) (35.4%) was the most-common diagnosis, followed by the unclassified form (17.4%), isolated optic neuritis (ON) (15.2%), neuromyelitis optica spectrum disorder (13.0%), multiple sclerosis (MS) (10.8%), other clinically isolated syndromes [monophasic event except ADEM, isolated ON, or transverse myelitis (TM)] (8.7%), and unexplained encephalitis (4.3%). At the initial presentation, 35 out of the 46 patients with ADS had brain lesions detected in magnetic resonance imaging, and 54% of these 35 patients had encephalopathy. Nine of the 11 patients without brain lesions exhibited only ON. Thirty-nine percent of the patients experienced a multiphasic event during the mean follow-up period of 34.9 months (range 1.4-169.0 months). Encephalopathy at the initial presentation was frequently confirmed in the monophasic group (=0.011).
MOG antibodies were identified in all pediatric ADS phenotypes except for monophasic TM. Therefore, the MOG antibody test is recommended for all pediatric patients with ADS, especially before a diagnosis of MS and for patients without a clear diagnosis.
在儿童获得性脱髓鞘综合征（ADS）中，髓鞘少突胶质细胞糖蛋白（MOG）抗体的检出率较高。本研究旨在确定MOG抗体在ADS中的诊断价值以及儿童MOG抗体阳性脱髓鞘疾病的谱。
本研究纳入了128例诊断为ADS（n = 94）或不明原因脑炎（n = 34）的患者。采用基于活细胞的内部免疫荧光测定法检测血清中的MOG抗体。
48例患者检测到MOG抗体（46例ADS患者和2例脑炎患者，包括23例男性和25例女性）。急性播散性脑脊髓炎（ADEM）（35.4%）是最常见的诊断，其次是未分类形式（17.4%）、孤立性视神经炎（ON）（15.2%）、视神经脊髓炎谱系障碍（13.0%）、多发性硬化（MS）（10.8%）、其他临床孤立综合征[除ADEM、孤立性ON或横贯性脊髓炎（TM）外的单相事件]（8.7%）和不明原因脑炎（4.3%）。在初次就诊时，46例ADS患者中有35例在磁共振成像中检测到脑部病变，这35例患者中有54%患有脑病。11例无脑部病变的患者中有9例仅表现为ON。在平均34.9个月（范围1.4 - 169.0个月）的随访期内，39%的患者经历了多相事件。在单相组中，初次就诊时的脑病情况经常得到证实（P = 0.011）。
除单相TM外，在所有儿科ADS表型中均鉴定出MOG抗体。因此，建议对所有儿科ADS患者进行MOG抗体检测，尤其是在诊断MS之前以及诊断不明确的患者中。
