Zhou Daniel J, Powers Andria M, Cave Caleb A, Dickas Emily K, Rickard Mary C, Rathore Geetanjali, Wright Rhonda R, Zabad Rana K, Koh Sookyong
Department of Neurological Sciences, University of Nebraska Medical Center, Omaha, NE, USA.
Department of Radiology, Children's Hospital and Medical Center, Omaha, NE, USA.
Neurohospitalist. 2023 Oct;13(4):438-444. doi: 10.1177/19418744231192159. Epub 2023 Jul 25.
We report two distinct challenging initial presentations of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Case 1 describes a 12-year-old boy who developed headaches refractory to pain medication followed by cranial neuropathies and intracranial hypertension, confirmed by lumbar puncture with an opening pressure >36 cm HO. Case 2 describes a 3-year-old boy who developed new-onset seizures refractory to antiseizure medications, a presentation of FLAIR-hyperintense lesions in MOG-antibody associated encephalitis with seizures (FLAMES). On repeat magnetic resonance imaging, both patients were found to have cortical T2 hyperintensities, leptomeningeal contrast enhancement, and bilateral optic nerve enhancement. In the cerebrospinal fluid, both patients had CSF pleocytosis with neutrophilic predominance. The patients were treated with intravenous immunoglobulins, plasma exchange, and high-dose corticosteroids. The first patient achieved disease remission, whereas the second patient required the addition of rituximab for management of seizures. The two cases highlight the pleomorphic clinical phenotypes of MOGAD.
我们报告了两例具有挑战性的髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）的初始表现。病例1为一名12岁男孩，出现对止痛药无效的头痛，随后出现颅神经病变和颅内高压，腰椎穿刺证实初压>36cmH₂O。病例2为一名3岁男孩，出现对抗癫痫药物无效的新发癫痫，这是MOG抗体相关脑炎伴癫痫（FLAMES）的一种表现，即液体衰减反转恢复序列（FLAIR）高信号病变。在重复磁共振成像检查中，两名患者均发现有皮质T2高信号、软脑膜强化和双侧视神经强化。脑脊液检查显示，两名患者均有以中性粒细胞为主的脑脊液细胞增多。患者接受了静脉注射免疫球蛋白、血浆置换和大剂量皮质类固醇治疗。第一名患者病情缓解，而第二名患者需要加用利妥昔单抗来控制癫痫发作。这两个病例突出了MOGAD的多形性临床表型。
