Frade Heitor C, Elnaeem Awab, Banerjee Pankhuri, Sharma Tripti, Wu Laura, Dabi Alok
Neurology, University of Texas Medical Branch, Galveston, USA.
Cureus. 2024 Sep 3;16(9):e68563. doi: 10.7759/cureus.68563. eCollection 2024 Sep.
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a central nervous system demyelinating disease that has become a major source of morbidity among children and adults. In the first case, we present an 18-year-old Hispanic female with a recently resolved upper respiratory infection who presented with fever, headache, progressive quadriparesis, urinary retention, and encephalopathy. The hospital course involved autonomic dysfunction and prolonged intubation requiring tracheostomy and gastrostomy. Cerebrospinal fluid (CSF) showed pleocytosis and a positive MOG titer (1:40). Magnetic resonance imaging (MRI) showed longitudinally extensive cervicothoracic T2 hyperintensity and brain multifocal T2 hyperintensities. After high-dose intravenous methylprednisolone (IVMP) and intravenous immunoglobulin (IVIG), she had full neurological recovery by the last follow-up. The second case is of a 22-year-old Hispanic male who presented with progressive lower extremity paresthesia and weakness over six weeks. CSF demonstrated pleocytosis, elevated protein, oligoclonal bands, and MOG antibody. MRI revealed multiple subcortical T2-hyperintense lesions and enhancing midcervical and lower thoracic lesions. Treatment with IVMP led to minor improvement with discharge on steroid taper and azathioprine. The patient's disease progressed with a fluctuating course requiring two readmissions with upper extremity weakness, right optic neuritis, and urinary sphincteric dysfunction with neuroradiologic worsening. Treatment throughout multiple admissions included intravenous steroids, IVIG, plasmapheresis, mycophenolate mofetil, and rituximab with minimal improvement, symptom recurrence, and progression of multifocal lesions. The patient died four months after the symptom onset. These cases had markedly different treatment responses despite similar baseline characteristics. The difference in morbidity and disability burden highlights the importance of further investigation of this condition through clinical trials.
髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）是一种中枢神经系统脱髓鞘疾病，已成为儿童和成人发病的主要原因。在第一个病例中，我们介绍了一名18岁的西班牙裔女性，她近期上呼吸道感染已痊愈，随后出现发热、头痛、进行性四肢瘫、尿潴留和脑病。住院过程中出现自主神经功能障碍，需要长期插管，并进行气管造口术和胃造口术。脑脊液（CSF）显示细胞增多，MOG滴度阳性（1:40）。磁共振成像（MRI）显示颈胸段纵向广泛T2高信号和脑内多发T2高信号。在接受大剂量静脉注射甲基强的松龙（IVMP）和静脉注射免疫球蛋白（IVIG）治疗后，她在最后一次随访时神经功能完全恢复。第二个病例是一名22岁的西班牙裔男性，他在六周内出现进行性下肢感觉异常和无力。脑脊液显示细胞增多、蛋白升高、寡克隆带和MOG抗体。MRI显示多个皮质下T2高信号病变以及颈段中部和胸段下部病变强化。IVMP治疗后有轻微改善，出院时逐渐减少类固醇剂量并服用硫唑嘌呤。患者病情呈波动进展，因上肢无力、右眼视神经炎和尿道括约肌功能障碍伴神经影像学恶化而两次再次入院。多次入院期间的治疗包括静脉注射类固醇、IVIG、血浆置换、霉酚酸酯和利妥昔单抗，但改善甚微，症状复发，多灶性病变进展。患者在症状出现四个月后死亡。尽管基线特征相似，但这些病例的治疗反应明显不同。发病率和残疾负担的差异凸显了通过临床试验进一步研究这种疾病的重要性。
