Choi Young Hun, Kim Woo Joong, Lim Byung Chan, Yoo Il Han, Cho Yeon Jin, Lee Seunghyun, Hwang Jae-Yeon, Cheon Jung-Eun
Department of Radiology, Seoul National University College of Medicine, Seoul, Republic of Korea.
Innovative Medical Technology Research Institute, Seoul National University Hospital, Seoul, Republic of Korea.
Pediatr Radiol. 2025 Aug 11. doi: 10.1007/s00247-025-06342-y.
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a notable cause of acquired central nervous system inflammatory disorders in children.
This study aimed to characterize the neuroimaging spectrum of pediatric MOGAD with brain involvement.
In this retrospective, single-center study, 55 children diagnosed with MOGAD involving the brain between January 2010 and October 2020 were included. Clinical data and neuroimaging-brain and spinal magnetic resonance imaging (MRI) at presentation-were reviewed. Imaging patterns were categorized into six radiologic phenotypes: acute disseminated encephalomyelitis (ADEM), cerebral cortical encephalitis, aseptic meningitis, tumefactive demyelinating lesion, cerebellitis/brainstem encephalitis, and miscellaneous. Imaging features were further analyzed in the ADEM subgroup.
ADEM was the most common phenotype (39 of 55 patients, 71%), though atypical features were frequent, with 62% showing at least one atypical MRI finding. Unlike classic ADEM with large confluent white matter lesions, MOGAD-associated ADEM often showed small (31%) or subcortical (44%) white matter lesions. Spinal lesions typically appeared as longitudinally extensive myelitis with central gray matter involvement. Other phenotypes included cortical encephalitis (three patients), aseptic meningitis (six), tumefactive demyelinating lesions (three), cerebellitis/brainstem encephalitis (two), and two miscellaneous patterns. Non-ADEM phenotypes presented at an older age than ADEM (11.5 years vs. 5.2 years, P < 0.01), with a threshold of 7.6 years.
Pediatric MOGAD with brain involvement presents a range of imaging patterns. ADEM is most frequent but often displays atypical features. Non-ADEM phenotypes tend to occur in older children.
髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)是儿童获得性中枢神经系统炎性疾病的一个显著病因。
本研究旨在描述脑部受累的儿童MOGAD的神经影像学特征。
在这项回顾性单中心研究中,纳入了2010年1月至2020年10月期间诊断为脑部受累的MOGAD的55名儿童。回顾了临床资料以及就诊时的神经影像学检查——脑部和脊髓磁共振成像(MRI)。成像模式被分为六种放射学表型:急性播散性脑脊髓炎(ADEM)、大脑皮质脑炎、无菌性脑膜炎、瘤样脱髓鞘病变、小脑炎/脑干脑炎以及其他。在ADEM亚组中进一步分析成像特征。
ADEM是最常见的表型(55例患者中有39例,占71%),尽管非典型特征很常见,62%的患者至少有一项非典型MRI表现。与具有大片融合性白质病变的经典ADEM不同,MOGAD相关的ADEM常表现为小的(31%)或皮质下(44%)白质病变。脊髓病变通常表现为纵向广泛的脊髓炎并累及中央灰质。其他表型包括皮质脑炎(3例患者)、无菌性脑膜炎(6例)、瘤样脱髓鞘病变(3例)、小脑炎/脑干脑炎(2例)以及两种其他模式。非ADEM表型出现的年龄比ADEM大(11.5岁对5.2岁,P<0.01),阈值为7.6岁。
脑部受累的儿童MOGAD呈现出一系列成像模式。ADEM最常见,但常表现出非典型特征。非ADEM表型倾向于在年龄较大的儿童中出现。