两个儿童MOGAD的初始表现令人困惑:颅内高压和新发癫痫。

IF 0.9 Q4 CLINICAL NEUROLOGY Neurohospitalist Pub Date : 2023-10-01 Epub Date: 2023-07-25 DOI:10.1177/19418744231192159
Daniel J Zhou, Andria M Powers, Caleb A Cave, Emily K Dickas, Mary C Rickard, Geetanjali Rathore, Rhonda R Wright, Rana K Zabad, Sookyong Koh
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引用次数: 0

摘要

我们报告了髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)的两种不同的具有挑战性的初始表现。病例1描述了一名12岁男孩,他出现了对止痛药难以治疗的头痛,随后出现了颅内神经病变和颅内高压,经腰椎穿刺证实,开口压力>36 cm H2O。病例2描述了一名3岁男孩,他出现了对抗癫痫药物难治的新发性癫痫发作,在MOG抗体相关的癫痫发作脑炎(FLAMES)中表现为FLAIR高信号病变。在重复磁共振成像中,发现两名患者都有皮质T2高信号、软脑膜造影增强和双侧视神经增强。在脑脊液中,两名患者都有以中性粒细胞为主的脑脊液白细胞增多症。患者接受静脉注射免疫球蛋白、血浆置换和高剂量皮质类固醇治疗。第一名患者病情缓解,而第二名患者需要添加利妥昔单抗来治疗癫痫。这两个病例突出了MOGAD的多形性临床表型。
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Perplexing Initial Presentations of MOGAD in Two Children: Intracranial Hypertension and New-Onset Seizure.

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 H2O. 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.

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来源期刊
Neurohospitalist
Neurohospitalist CLINICAL NEUROLOGY-
CiteScore
1.60
自引率
0.00%
发文量
108
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