儿童抗髓鞘少突胶质细胞糖蛋白IgG相关疾病的MRI征象分析

Analysis of magnetic resonance imaging features in children with anti-myelin oligodendrocyte glycoprotein-IgG associated disorders

目的探讨儿童抗髓鞘少突胶质细胞糖蛋白IgG相关疾病(MOGAD)的MRI特征。方法病例系列报告。纳入2018年1月—2021年12月聊城市人民医院确诊MOGAD的10例患儿, 其中男6例、女4例, 年龄2~9(5.9±2.4)岁。10例患儿治疗前行颅脑MR常规扫描, 9例行全脊柱MR常规扫描, 4例行眼眶MR常规扫描。观察指标:(1)记录MRI对MOGAD颅脑、脊髓和视神经病变的检出情况;(2)总结颅脑MOGAD病变分布位置、形态及信号特点;(3)观察脊髓MOGAD病变部位、脊髓有无肿胀, 统计长节段脊髓MOGAD病变的患儿数量;(4)观察视神经病变部位、视神经有无肿胀;(5)将患儿末次随访时复查MRI与治疗前进行对比, 观察病变转归情况。结果治疗前MRI显示:(1)颅脑病变10例, 脊髓病变5例, 视神经病变2例。(2)10例MOGAD颅脑病变均为多发, 9例双侧、1例单侧。4例仅累及幕上, 1例仅累及幕下, 5例同时累及幕上和幕下。幕上病变位于皮层下白质7例, 脑室周围白质7例, 丘脑5例, 基底节区4例, 胼胝体2例, 单侧皮质1例;幕下病变位于桥脑6例, 同时累及小脑4例、中脑2例。病变均为非对称性。MOGAD病变形态均表现为无定形斑片状、斑点状, 其中2例同时出现斑块样改变;2例病变DWI序列显示弥散受限。(3)MOGAD脊髓病变5例, 均为单发、累及胸段脊髓的长节段脊髓病变, 其中4例同时累及颈段脊髓, 未发现腰段脊髓受累;2例出现脊髓肿胀。(4)MOGAD视神经病变2例, 均为双侧, 其中1例视神经全程受累、1例视神经前部受累, 视交叉及视束均无受累。2例视神经均无肿胀。(5)10例患儿经治疗后均好转出院, 出院后随访8~29(12.9±5.8)个月。随访期间患儿定期复查MRI, 均未见复发。末次随访MRI显示, 2例患儿颅脑病变完全消失, 1例脊髓病变完全消失, 其余7例患儿颅脑、脊髓或视神经MOGAD病变范围均减小或病灶数均减少。结论儿童MOGAD中, 颅脑病变最常见, 其次为脊髓和视神经;不同部位的MOGAD MRI表现具有一定特征性, 认识这些影像学特征, 可以提高临床医生诊断儿童MOGAD的准确率, 为早期治疗提供重要参考。

Objective To investigate MRI features in children with anti-myelin oligodendrocyte glycoprotein-IgG associated disorders(MOGAD).Methods A retrospective case series study was performed.Ten children diagnosed with MOGAD in Liaocheng People's Hospital from January 2018 to December 2021 were enrolled.Among the patients,6 were males,and 4 were females,with a mean age of 2-9(5.9±2.4)years.All 10 children underwent routine cerebral MRI scans,9 underwent routine full-spinal MRI scans,and 4 underwent routine orbital MRI scans before treatment.The detection of MOGAD lesions in the brain,spinal cord,and optic nerve was recorded via MRI.The distribution,morphology,and signal characteristics of brain MOGAD lesions were evaluated.In addition,the lesion site and swelling of the spinal cord were evaluated,and the number of children with a long-segment MOGAD lesion was determined.The lesion site and swelling of the optic nerve were also evaluated,and MRI images of the last follow-up examination were compared with those taken before treatment to observe the outcome of the disease.Results MRI before treatment revealed the following points.(1)The MRI showed brain lesions in all 10 cases,spinal cord lesions in 5 cases,and optic nerve lesions in 2 cases.(2)Multiple MRI lesions were observed in all 10 cases.Nine cases were bilateral,and one case was unilateral.All of the 10 cases presented an asymmetric distribution,4 cases involved only the supratentorial area,one case involved only the subtentorial area and 5 cases involved the supratentorial and subtentorial areas.The supratentorial lesions were located in subcortical white matter in 7 cases,in white matter around the lateral ventricle in 7 cases,in the thalamus in 5 cases,in the basal ganglia in 4 cases,in the corpus callosum in 2 cases,and in the lateral cortex in one case.The subtentorial lesions were located in pons in 6 cases,in the cerebellum in 4 cases,and in the midbrain in 2 cases.All 10 cases with MRI lesions showed an amorphous patchy morphology,and 2 cases showed patch-like changes simultaneously;in the 2 cases,the DWI sequence exhibited limited diffusion.(3)MRI showed 5 MOGAD myelopathy cases,all of which were single.Lesions of the thoracic spinal cord were observed in all 5 cases,and lesions of the cervical spinal cord were observed in 4 cases;no lumbar spinal cord involvement was observed,and 2 showed spinal cord swelling.All 5 cases of spinal MOGAD presented long-segment myelopathy.(4)MRI showed MOGAD optic neuropathy in 2 cases,both of which were bilateral.One case involved the whole optic nerve,and the other case involved the anterior optic nerve.The optic chiasma and optic tract were not involved.No swelling of the optic nerve was observed in 2 cases.(5)After treatment,all 10 children improved and were discharged.After discharge,they were followed up for 8-29(12.9±5.8)months.During the follow-up period,MRI scans were examined regularly,and no recurrence was found.The last MR examination showed that the craniocerebral lesions disappeared in 2 cases;myelopathy disappeared in one case;and the MOGAD lesions in the brain,spinal cord,or optic nerve were reduced in scope or number in the 7 remaining cases.Conclusion In children with MOGAD,brain lesions are the most common,followed by spinal cord and optic nerve lesions.The MRI findings for different parts have certain characteristics.Understanding the imaging features of MOGAD can improve the accuracy of clinical diagnosis of MOGAD in children and provide an important reference for early treatment.