急性播散性脑脊髓炎（ADEM）与抗髓鞘少突胶质细胞糖蛋白抗体相关的颅内压增高。
Acute Disseminated Encephalomyelitis (ADEM) and Increased Intracranial Pressure Associated With Anti-Myelin Oligodendrocyte Glycoprotein Antibodies.
机构信息
Department of Neurology, University of Texas Southwestern, Department of Neurology and Neurotherapeutics, Dallas, Texas.
Department of Neurology, University of Texas Southwestern, Department of Neurology and Neurotherapeutics, Dallas, Texas.
出版信息
Pediatr Neurol. 2019 Oct;99:64-68. doi: 10.1016/j.pediatrneurol.2019.03.009. Epub 2019 Mar 22.
BACKGROUND
Antibodies to the myelin oligodendrocyte glycoprotein (MOG) have been identified in about 40% of children with acute disseminated encephalomyelitis (ADEM). The objective of this report is to describe three individuals with fulminant ADEM complicated by increased intracranial pressure associated with the presence of the anti-MOG antibodies.
METHODS
This is a retrospective case series. Informed consent was obtained from the concerned patients or caregivers.
RESULTS
High intracranial pressure associated with ADEM in the presence of MOG antibodies can result in cerebral edema, herniation, prolonged hospital stay (average intensive care unit stay: 22 days, average hospital stay: 50.6 days), and long-term disability.
CONCLUSION
Increased intracranial pressure complicating MOG antibody-related ADEM is a unique finding in our cases. This can complicate the clinical picture of ADEM and confers high morbidity. Long-term immunosuppression is warranted in selected cases with persistent seropositivity.
背景
髓鞘少突胶质细胞糖蛋白（MOG）抗体已在约 40%的急性播散性脑脊髓炎（ADEM）患儿中被发现。本报告的目的是描述三例伴有颅内压增高的暴发性 ADEM 患者，其与 MOG 抗体的存在有关。
方法
这是一项回顾性病例系列研究。获得了相关患者或其照顾者的知情同意。
结果
MOG 抗体阳性的 ADEM 合并高颅压可导致脑水肿、脑疝、延长住院时间（平均重症监护病房住院时间：22 天，平均住院时间：50.6 天）和长期残疾。
结论
MOG 抗体相关性 ADEM 合并高颅压是我们病例中的一个独特发现。这会使 ADEM 的临床情况复杂化，并导致高发病率。在持续血清阳性的情况下，选择合适的病例进行长期免疫抑制是有必要的。
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