Department of Child Health, College of Medicine, King Khalid University, Abha, Kingdom of Saudi Arabia.
Department of Pediatrics, Neurology Division, Alhada Armed Forces Hospital, Taif, Kingdom of Saudi Arabia.
Medicine (Baltimore). 2023 Feb 22;102(8):e32986. doi: 10.1097/MD.0000000000032986.
Myelin oligodendrocyte glycoprotein antibody (MOGA) associated diseases are inflammatory immune-mediated demyelinating disorders with relapse potential involving the central nervous system. Multiple unusual clinical manifestations of those disorders were reported, making treatment decisions difficult.
A healthy 12-year-old obese boy presented with headache and bilateral asymmetric papilledema. The patient had a negative medical history. His neurological and general examinations were unremarkable, his initial magnetic resonance imaging showed elevated intracranial pressure (ICP) only. A lumbar puncture revealed increased opening pressure and pleocytosis. The MOGA titer was 1:320. He needed acetazolamide and steroid therapy. After 2 months of medication, weight loss, exercise, the patient symptoms significantly improved, papilledema resolved, and visual function improved.
MOGA-associated disorders have a variety of clinical features, so a high index of suspicion is required for their diagnosis. Papilledema and an elevated ICP are 2 of the chameleons of MOGA-associated disorders. MOGA test may be useful in patients with elevated ICP and inflammatory cerebrospinal fluid profiles. An investigation of the possible association between those disorders and high ICP is warranted.
髓鞘少突胶质细胞糖蛋白抗体（MOGA）相关疾病是具有复发潜力的中枢神经系统炎症性免疫介导脱髓鞘疾病。这些疾病有多种不常见的临床表现，使得治疗决策变得困难。
一位健康的 12 岁肥胖男孩因头痛和双侧不对称性视盘水肿就诊。患者无既往病史。他的神经系统和全身检查均无明显异常，最初的磁共振成像仅显示颅内压升高（ICP）。腰椎穿刺显示颅内压升高和白细胞增多。MOGA 滴度为 1:320。他需要乙酰唑胺和类固醇治疗。经过 2 个月的药物治疗，体重减轻、运动后，患者症状明显改善，视盘水肿消退，视力功能改善。
MOGA 相关疾病具有多种临床特征，因此需要高度怀疑其诊断。视盘水肿和颅内压升高是 MOGA 相关疾病的 2 个变色龙。MOGA 检测可能对颅内压升高和炎症性脑脊液特征的患者有用。有必要调查这些疾病与高颅内压之间的可能关联。
