Department of Neurology, Beijing Fengtai You'anmen Hospital, Beijing, China.
Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China.
Medicine (Baltimore). 2023 Nov 24;102(47):e36359. doi: 10.1097/MD.0000000000036359.
Autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) is a group of neurological syndromes involving the meninges, brain, spinal cord, and optic nerves and is characterized by sensitivity to steroid therapy. Due to the diverse clinical presentation and lack of uniform diagnostic criteria, GFAP-A can easily be overlooked or diagnosed as another disease. It is even rarer when presenting as an isolated spinal cord lesion.
We report the case of a 70-year-old man with initial symptoms of numbness and weakness in both lower limbs, followed by difficulty in urination and defecation, and progression of numbness upward to the hands. Magnetic resonance imaging (MRI) showed a lesion in the spinal cord from cervical level 2 to thoracic 7 in a T2-weighted image. T1-weighted image showed a punctate, lamellar strengthening lesion with significant spinal strengthening. GFAP immunoglobulin G (IgG) was detected in the cerebrospinal fluid and blood. After treatment with intravenous gamma globulin (IVIG), the patient symptoms improved and spinal cord enhancement was reduced.
Long segment cases with punctate and patchy enhancement of the spinal cord are difficult to distinguish from CLAPPERS, so GFAP-A antibody detection is very important. This atypical case also increases neurologists' understanding of GFAP-A.
自身免疫性神经胶质纤维酸性蛋白星形胶质细胞病（GFAP-A）是一组累及脑膜、脑、脊髓和视神经的神经系统综合征，其特征是对类固醇治疗敏感。由于临床表现多样，缺乏统一的诊断标准，GFAP-A 容易被忽视或误诊为其他疾病。当仅表现为孤立性脊髓病变时更为罕见。
我们报告了一例 70 岁男性患者，最初表现为双下肢麻木无力，随后出现排尿和排便困难，麻木向上进展至手部。磁共振成像（MRI）显示 T2 加权图像中颈 2 至胸 7 脊髓有病变。T1 加权图像显示点状、层状强化病变，脊髓强化明显。脑脊液和血液中检测到神经胶质纤维酸性蛋白 IgG（GFAP-IgG）。静脉注射免疫球蛋白（IVIG）治疗后，患者症状改善，脊髓强化减轻。
脊髓长节段点状和斑片状强化的病例与 CLAPPERS 难以区分，因此 GFAP-A 抗体检测非常重要。这个不典型病例也增加了神经科医生对 GFAP-A 的认识。
