Division of Neurosurgery, Children's Hospital of Philadelphia, Department of Neurosurgery, University of Pennsylvania, Perelman School of Medicine, 3401 Civic Center Blvd, 6 Wood Center, Philadelphia, PA, 19104, USA.
Division of Neurology, Children's Hospital of Philadelphia, Department of Neurology, University of Pennsylvania, Perelman School of Medicine, Philadelphia, PA, USA.
Childs Nerv Syst. 2022 Jan;38(1):185-189. doi: 10.1007/s00381-021-05139-2. Epub 2021 Apr 2.
Acquired demyelination of the central nervous system in children can manifest as multiple sclerosis, neuromyelitis optica, myelin oligodendrocyte glycoprotein (MOG)-associated demyelination, or as an acute monophasic illness without serum antibodies. Rarely do patients with demyelinating disease need surgical intervention for fulminant crises.
We report a case of anti-MOG antibody-related tumefactive demyelination in a 10-year-old female who required urgent hemicraniectomy and external ventricular drain placement for progressive white matter edema with obstructive hydrocephalus, subfalcine, and transtentorial herniation.
儿童中枢神经系统获得性脱髓鞘可表现为多发性硬化症、视神经脊髓炎、髓鞘少突胶质细胞糖蛋白（MOG）相关性脱髓鞘，也可表现为血清抗体阴性的急性单相病程。脱髓鞘疾病患者很少因暴发性危象而需要手术干预。
我们报告了一例 10 岁女性抗 MOG 抗体相关性肿块样脱髓鞘病例，该患者因进行性脑白质水肿伴梗阻性脑积水、镰下和小脑幕切迹疝，需要紧急行半颅脑切除术和外置脑室引流术。
