Bernardi Luca, Mussi Nicole, Grandinetti Roberto, Turco Emanuela, Piccolo Benedetta, Ormitti Francesca, Principi Nicola, Esposito Susanna
Pediatric Clinic, Department of Medicine and Surgery, Azienda Ospedaliero-Universitaria di Parma, 43126 Parma, Italy.
Unit of Neuroradiology, Azienda Ospedaliero-Universitaria di Parma, 43126 Parma, Italy.
Children (Basel). 2024 Jun 27;11(7):778. doi: 10.3390/children11070778.
Myelin oligodendrocyte glycoprotein (MOG)-IgG-associated disease (MOGAD) is a relatively uncommon autoantibody demyelinating disorder of the central nervous system (CNS) with heterogeneous clinical manifestations and magnetic resonance imaging (MRI) findings. In recent years, a rare MOGAD subtype characterized by distinct clinical and MRI findings has been described. Seizures and cortical hyperintensities best seen on MRI T2-weighted fluid-attenuated inversion recovery (FLAIR) sequences, associated with headache and cerebral spine fluid (CSF) pleocytosis, are the most important characteristics of this MOGAD entity that is named FLAMES (FLAIR hyperintense cortical lesions in MOG-associated encephalitis with seizures). Because of its rarity and the peculiarities of the brain damage and clinical manifestations, it can be under-recognized and confused with focal viral encephalitis, meningitis, subarachnoid hemorrhage, CNS vasculitis, or mitochondrial cytopathy. We described the case of a 4-year-old previously healthy girl who was admitted for focal-onset, tonic-clonic seizures, fever, and headache, combined with optic neuritis. MRI was characterized by FLAIR imaging showing hyperintense cortical lesions, and a mild leukocytosis in the CSF was detected. Efficacy and rapid response to steroid therapy was observed, and no recurrences of neurological problems or further seizures were reported in the following 12 months. This case report can help in understanding FLAMES characteristics in pediatrics in order to favor early diagnosis and prompt therapy.
髓鞘少突胶质细胞糖蛋白（MOG）-IgG相关疾病（MOGAD）是一种相对罕见的中枢神经系统（CNS）自身抗体介导的脱髓鞘疾病，临床表现和磁共振成像（MRI）表现具有异质性。近年来，已描述了一种以独特的临床和MRI表现为特征的罕见MOGAD亚型。癫痫发作和MRI T2加权液体衰减反转恢复（FLAIR）序列上最易见的皮质高信号，伴有头痛和脑脊液（CSF）细胞增多，是这种被命名为FLAMES（伴有癫痫发作的MOG相关脑炎中的FLAIR高信号皮质病变）的MOGAD实体的最重要特征。由于其罕见性以及脑损伤和临床表现的特殊性，它可能未得到充分认识，并与局灶性病毒性脑炎、脑膜炎、蛛网膜下腔出血、中枢神经系统血管炎或线粒体细胞病相混淆。我们描述了一名4岁以前健康女孩的病例，她因局灶性发作、强直阵挛性癫痫发作、发热和头痛入院，同时伴有视神经炎。MRI的特征是FLAIR成像显示皮质高信号病变，脑脊液中检测到轻度白细胞增多。观察到对类固醇治疗有效且反应迅速，在接下来的12个月中未报告神经问题复发或进一步的癫痫发作。本病例报告有助于了解儿科FLAMES的特征，以便早期诊断和及时治疗。
