Fellow LVPEI, Standard Chartered LVPEI Academy of Eye Care Education, LV Prasad Eye Institute, Hyderabad, Telangana, India.
Child Sight Institute, Jasti V Ramanamma Children's Eye Care Centre, LV Prasad Eye Institute, Hyderabad, Telangana, India
BMJ Case Rep. 2022 Jul 7;15(7):e249398. doi: 10.1136/bcr-2022-249398.
A preschool girl presented with sudden-onset bilateral painless loss of vision from 2 days prior. Child's examination showed light perception vision, sluggishly reacting pupils, otherwise normal anterior segment, healthy optic disc and retina in both eyes. MRI of brain and orbit with contrast revealed thickened left part of the optic chiasm with contrast enhancement extending proximally to bilateral optic tract and hyperintensities in the left thalamus and periventricular white mater. Considering the topographical distribution of lesions in the brain, neuromyelitis optica spectrum disorder was suspected. The child was started on intravenous methylprednisolone followed by tapering oral steroids. Serological testing was positive for myelin oligodendrocyte glycoprotein (MOG) and negative for aquaporin-4 antibodies. This case represents an unusual case of MOG associated demyelination disorder where the distribution of lesions showed chiasmal involvement along with optic tract, thalamus and deep white mater lesions.
一位学龄前女孩因双眼无痛性视力丧失,于 2 天前就诊。患儿检查发现光感视力,瞳孔反应迟钝,双眼前段正常,视盘和视网膜均健康。头颅和眼眶对比 MRI 显示视交叉左侧增厚,伴有对比增强,向双侧视神经束近端延伸,并在左侧丘脑和脑室周围白质出现高信号。考虑到病变在脑部的分布位置,疑诊为视神经脊髓炎谱系疾病。患儿开始静脉注射甲基泼尼松龙,随后逐渐减少口服类固醇。血清学检查髓鞘少突胶质细胞糖蛋白(MOG)阳性,水通道蛋白 4 抗体阴性。本例为 MOG 相关性脱髓鞘疾病的不典型病例,病变分布显示视交叉受累,伴有视神经束、丘脑和深部白质病变。