Department of Neurology, Gifu University Graduate School of Medicine, Japan.
Intern Med. 2020 Nov 1;59(21):2777-2781. doi: 10.2169/internalmedicine.5074-20. Epub 2020 Jul 14.
We report a 65-year-old man with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) who presented with gait disturbance that he had experienced for approximately half a year. On neurological examination, he displayed spastic paraplegia and autonomic dysfunctions including dysuria and constipation. Spinal cord magnetic resonance imaging showed longitudinally extensive spinal cord lesions (LESCLs) extending from the cervical to the thoracic cords. The patient was negative for anti-myelin oligodendrocyte glycoprotein and anti-aquaporin 4 antibodies. Treatment with corticosteroids and intravenous immunoglobulin resulted in a clinical improvement. It is important to distinguish GFAP-A from slowly progressive myelitis with LESCLs.
我们报告了一例 65 岁的自身免疫性胶质纤维酸性蛋白星形胶质细胞病（GFAP-A）患者，他因步态障碍就诊，该障碍已持续约半年。神经系统检查显示痉挛性截瘫和自主神经功能障碍，包括排尿困难和便秘。脊髓磁共振成像显示从颈段到胸段的长节段脊髓病变（LESCL）。患者抗髓鞘少突胶质细胞糖蛋白和抗水通道蛋白 4 抗体均为阴性。皮质类固醇和静脉注射免疫球蛋白治疗后临床症状改善。区分 GFAP-A 与伴有 LESCL 的缓慢进展性脊髓炎很重要。
