Brain Autoimmunity Group, Kids Neuroscience Centre at the Children's Hospital at Westmead, Sydney Medical School, University of Sydney, Sydney, NSW, Australia.
Department of Neurology, Westmead Hospital, Sydney, NSW, Australia.
Dev Med Child Neurol. 2019 May;61(5):610-614. doi: 10.1111/dmcn.14032. Epub 2018 Sep 17.
Myelin oligodendrocyte glycoprotein (MOG) antibodies have a strong association with acute disseminated encephalomyelitis (ADEM) in children, and bilateral and recurrent optic neuritis in children and adults. Recent reports suggest that seizures and encephalopathy may occur in children and adults with MOG antibody-associated disease. We describe the clinical, laboratory, and radiological course of four MOG antibody-positive children who first presented with isolated seizures without fulfilling clinical or radiological criteria for ADEM or other central nervous system demyelination syndromes, who months to years later developed more typical demyelination. This case series highlights a novel observation that isolated seizures in the absence of ADEM may be the index presentation for MOG antibody-associated disease, which should therefore be considered a form of autoimmune epilepsy. It would be reasonable to test for MOG antibodies in children with seizures accompanied by subtle inflammatory changes on magnetic resonance imaging or cerebrospinal fluid analyses, particularly if followed by demyelination, given the clinical and therapeutic implications of an expedited diagnosis in minimizing long-term disability. WHAT THIS PAPER ADDS: Isolated seizures in the absence of acute disseminated encephalomyelitis may be the index presentation for myelin oligodendrocyte glycoprotein antibody-associated demyelination.
髓鞘少突胶质细胞糖蛋白 (MOG) 抗体与儿童急性播散性脑脊髓炎 (ADEM) 以及儿童和成人双侧和复发性视神经炎密切相关。最近的报告表明,MOG 抗体相关疾病的儿童和成人可能会出现癫痫发作和脑病。我们描述了 4 名 MOG 抗体阳性儿童的临床、实验室和影像学病程,这些儿童最初表现为孤立性癫痫发作,不符合 ADEM 或其他中枢神经系统脱髓鞘综合征的临床或影像学标准,数月至数年后出现更典型的脱髓鞘。该病例系列强调了一个新的观察结果,即无 ADEM 的孤立性癫痫发作可能是 MOG 抗体相关疾病的首发表现,因此应将其视为自身免疫性癫痫的一种形式。如果随后发生脱髓鞘,伴有磁共振成像或脑脊液分析中细微炎症改变的癫痫发作患儿,出于快速诊断在减少长期残疾方面的临床和治疗意义,检测 MOG 抗体是合理的。本文的重要发现:无急性播散性脑脊髓炎的孤立性癫痫发作可能是髓鞘少突胶质细胞糖蛋白抗体相关脱髓鞘的首发表现。