PDF(Pubmed)
Abstract：
BACKGROUND: 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.
METHODS: 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.
CONCLUSIONS: 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.
METHODS: 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.
CONCLUSIONS: 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.
摘要：
背景：自身免疫性胶质纤维酸性蛋白星形细胞病（GFAP-A）是一组涉及脑膜的神经系统综合征，大脑,脊髓,和视神经，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很容易被忽视或被诊断为另一种疾病。当表现为孤立的脊髓病变时，它甚至更罕见。
方法：我们报告了一名70岁的男性患者，其首发症状为双下肢麻木无力，其次是排尿和排便困难，麻木向上延伸到手。磁共振成像（MRI）在T2加权图像中显示了从颈2级到胸7级的脊髓病变。T1加权图像显示点状，板层强化病变伴显著脊柱强化。在脑脊液和血液中检测到GFAP免疫球蛋白G（IgG）。静脉注射丙种球蛋白（IVIG）治疗后，患者症状改善，脊髓强化减少。
结论：脊髓点状和斑片状强化的长段病例很难与CLAPPERS区分开，所以GFAP-A抗体检测非常重要。这种非典型病例也增加了神经学家对GFAP-A的理解。
方法：我们报告了一名70岁的男性患者，其首发症状为双下肢麻木无力，其次是排尿和排便困难，麻木向上延伸到手。磁共振成像（MRI）在T2加权图像中显示了从颈2级到胸7级的脊髓病变。T1加权图像显示点状，板层强化病变伴显著脊柱强化。在脑脊液和血液中检测到GFAP免疫球蛋白G（IgG）。静脉注射丙种球蛋白（IVIG）治疗后，患者症状改善，脊髓强化减少。
结论：脊髓点状和斑片状强化的长段病例很难与CLAPPERS区分开，所以GFAP-A抗体检测非常重要。这种非典型病例也增加了神经学家对GFAP-A的理解。
