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以局灶性癫痫发作为表现形式的MOGAD的混淆性起病

Confusing Onset of MOGAD in the Form of Focal Seizures.

作者信息

Jączak-Goździak Małgorzata, Steinborn Barbara

机构信息

Department of Developmental Neurology, Poznan University of Medical Sciences, 61-701 Poznań, Poland.

出版信息

Neurol Int. 2025 Feb 27;17(3):37. doi: 10.3390/neurolint17030037.

Abstract

MOGAD is a demyelinating syndrome with the presence of antibodies against myelin oligodendrocyte glycoprotein, which is, next to multiple sclerosis and the neuromyelitis optica spectrum, one of the manifestations of the demyelinating process, more common in the pediatric population. MOGAD can take a variety of clinical forms: acute disseminated encephalomyelitis (ADEM), retrobulbar optic neuritis, often binocular (ON), transverse myelitis (TM), or NMOSD-like course (neuromyelitis optica spectrum disorders), less often encephalopathy. The course may be monophasic (40-50%) or polyphasic (50-60%), especially with persistently positive anti-MOG antibodies. Very rarely, the first manifestation of the disease, preceding the typical symptoms of MOGAD by 8 to 48 months, is focal seizures with secondary generalization, without typical demyelinating changes on MRI of the head. The paper presents a case of a 17-year-old patient whose first symptoms of MOGAD were focal epileptic seizures in the form of turning the head to the right with the elevation of the left upper limb and salivation. Seizures occurred after surgical excision of a tumor of the right adrenal gland (ganglioneuroblastoma). Then, despite a normal MRI of the head and the exclusion of onconeural antibodies in the serum and cerebrospinal fluid after intravenous treatment, a paraneoplastic syndrome was suspected. After intravenous steroid treatment and immunoglobulins, eight plasmapheresis treatments, and the initiation of antiepileptic treatment, the seizures disappeared, and no other neurological symptoms occurred for nine months. Only subsequent relapses of the disease with typical radiological and clinical picture (ADEM, MDEM, recurrent ON) allowed for proper diagnosis and treatment of the patient both during relapses and by initiating supportive treatment. The patient's case allows us to analyze the multi-phase, clinically diverse course of MOGAD and, above all, indicates the need to expand the diagnosis of epilepsy towards demyelinating diseases: determination of anti-MOG and anti-AQP4 antibodies.

摘要

MOGAD是一种脱髓鞘综合征,存在针对髓鞘少突胶质细胞糖蛋白的抗体,它是继多发性硬化症和视神经脊髓炎谱系病之后脱髓鞘过程的表现之一,在儿科人群中更为常见。MOGAD可呈现多种临床形式:急性播散性脑脊髓炎(ADEM)、球后视神经炎,通常为双眼性(ON)、横贯性脊髓炎(TM)或视神经脊髓炎谱系障碍(NMOSD)样病程,较少见脑病。病程可为单相性(40 - 50%)或多相性(50 - 60%),尤其是抗MOG抗体持续阳性时。非常罕见的是,在出现MOGAD典型症状前8至48个月,疾病的首发表现是伴有继发性全身性发作的局灶性癫痫发作,头部MRI无典型脱髓鞘改变。本文介绍了一名17岁患者的病例,其MOGAD的首发症状是以头部向右侧转动、左上肢抬起和流涎为表现的局灶性癫痫发作。癫痫发作发生在右侧肾上腺肿瘤(神经节神经母细胞瘤)手术切除后。之后,尽管头部MRI正常且静脉治疗后血清和脑脊液中排除了肿瘤相关神经抗体,但仍怀疑为副肿瘤综合征。经过静脉类固醇治疗和免疫球蛋白治疗、八次血浆置换治疗以及开始抗癫痫治疗后,癫痫发作消失,九个月内未出现其他神经症状。仅随后疾病复发时出现典型的放射学和临床症状(ADEM、MDEM、复发性ON),才使得在复发期间以及通过启动支持性治疗对患者进行正确诊断和治疗成为可能。该患者的病例使我们能够分析MOGAD多阶段、临床多样的病程,最重要的是,表明有必要将癫痫的诊断范围扩大到脱髓鞘疾病:检测抗MOG和抗AQP4抗体。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1c33/11946578/9331928cdd78/neurolint-17-00037-g001.jpg

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