Kato Takuma, Yamamoto Atsushi, Imai Keisuke, Menjo Kanako, Ioku Tetsuya, Takewaki Daiki
Department of Neurology and Stroke Treatment, Kyoto First Red Cross Hospital.
Department of Neurology, Kyoto Prefectural University of Medicine.
Rinsho Shinkeigaku. 2022 Mar 29;62(3):217-223. doi: 10.5692/clinicalneurol.cn-001690. Epub 2022 Mar 25.
The case was a 30-year-old man. He had generalized convulsion after preceding meningitis symptoms and transferred to our emergency department. He was tentatively diagnosed with meningoencephalitis and Todd paralysis based on elevation of cell counts in cerebrospinal fluid and abnormal high signals in the right cerebral cortex on brain FLAIR-MRI, and admitted on the same day. After admission, treatment with antibiotics, dexamethasone, antiviral drug and anticonvulsants was started. Both his clinical symptoms and findings on MRI improved steadily, and then he was discharged on day 19. Subsequently, headache exacerbated again and an additional examination for his serum sample taken on first admission day revealed presence of anti myelin oligodendrocyte glycoprotein (MOG)-antibody, resulting in his diagnosis of anti-MOG antibody unilateral cerebral cortical encephalitis (MOG-UCCE) on day 42. Rehospitalization was planned for introduction of steroid therapy, but generalized convulsion recurred on day 44 and he was hospitalized again. MRI image revealed no FLAIR high signal and cerebrospinal fluid was almost normal, but his headache and mild hemiparesis and numbness on the left side deteriorated again. Therefore, he was treated with intravenous high dose methylprednisolone followed by oral steroids. His clinical symptoms gradually improved, and he was discharged with slight headache on day 71. After discharge, there has been no recurrence under continuation of low dose oral steroids for two years. This case shows the need to measure anti-MOG antibody and introduce steroid therapy in the early phase in a case of suspected MOG-UCCE in a young patient with meningoencephalitis accompanied by generalized convulsion and characteristic abnormal findings on FLAIR-MRI.
该病例为一名30岁男性。他在出现脑膜炎症状后发生全身性惊厥,随后被转诊至我院急诊科。根据脑脊液细胞计数升高以及脑部液体衰减反转恢复序列(FLAIR)磁共振成像(MRI)显示右大脑皮质异常高信号,初步诊断为脑膜脑炎和托德麻痹,并于同日入院。入院后,开始使用抗生素、地塞米松、抗病毒药物和抗惊厥药物进行治疗。他的临床症状和MRI检查结果均稳步改善,于第19天出院。随后,头痛再次加剧,对首次入院当天采集的血清样本进行的进一步检查发现存在抗髓鞘少突胶质细胞糖蛋白(MOG)抗体,导致其在第42天被诊断为抗MOG抗体单侧大脑皮质脑炎(MOG-UCCE)。计划再次住院以开始使用类固醇治疗,但在第44天再次发生全身性惊厥,于是他再次住院。MRI图像显示无FLAIR高信号,脑脊液基本正常,但他的头痛以及左侧轻度偏瘫和麻木症状再次恶化。因此,对他进行了静脉注射大剂量甲泼尼龙治疗,随后改为口服类固醇。他的临床症状逐渐改善,于第71天出院,仍有轻微头痛。出院后,在持续服用低剂量口服类固醇两年的情况下未再复发。该病例表明,对于伴有全身性惊厥且在FLAIR-MRI上有特征性异常表现的年轻脑膜脑炎患者,若怀疑为MOG-UCCE,需要在早期检测抗MOG抗体并引入类固醇治疗。