抗体双阳性伴脊髓炎的自身免疫性星形细胞病临床特点  

Coexisting Autoimmune Glial Fibrillary Acidic Protein(GFAP)-IgG and Aquaporin4(AQP4)-IgG in Patients with Myelitis

作　　者：姚海燕[1] 李惠璐 江丽红 龙友明[1] 杨新光 YAO Hai-yan;LI Hui-lu;JIANG Li-hong;LONG You-ming;YANG Xin-guang(Department of Neurology,the Second Affiliated Hospital of Guangzhou Medical University,Guangzhou 510260,China;Department of Neurology,Sun Yat-sen Memorial Hospital,Sun Yat-sen University,Guangzhou 510235,China)

机构地区：[1]广州医科大学附属第二医院神经内科,广东广州510260 [2]中山大学孙逸仙纪念医院神经内科,广东广州510235

出　　处：《中山大学学报（医学科学版）》2022年第4期607-612,共6页Journal of Sun Yat-Sen University:Medical Sciences

基　　金：广州市民生科技攻关项目(202002020021);广东省医学科学技术研究基金(B2021235)。

摘　　要：【目的】探讨胶质纤维酸性蛋白抗体(GFAP-IgG)和水通道蛋白4抗体(AQP4-IgG)双阳性伴脊髓炎的自身免疫性胶质纤维酸性蛋白星形细胞病(GFAP-A)临床特点,旨在提高临床医师对此疾病的认识与诊治。【方法】本项目为一项回顾性病例对照研究,纳入伴脊髓炎的GFAP-A病例,收集伴随AQP4-IgG阳性的病例资料,汇总后进行综合分析。【结果】纳入55例GFAP-A,其主要临床症状包括头痛、发热、脊髓炎、视觉异常、行为异常、共济失调、意识障碍、癫痫发作、运动障碍、认知障碍和其他症状等。其中31例合并脊髓炎,并有8例为GFAP/AQP4双阳性伴脊髓炎。8例双阳性病例均表现有排尿便困难和感觉平面障碍,MRI检查均可见明显的脊髓病灶,其中7例有超过三个脊椎节段的长病灶。本研究选择了8例GFAP/AQP4-IgG双阳性伴脊髓炎、16例GFAP-IgG单阳性(除了AQP4-IgG阴性外,神经元抗体和少突、胶质细胞抗体均阴性)伴脊髓炎以及同时期诊断的47例AQP4-IgG单阳性(无伴随其它神经相关抗体)伴脊髓炎的视神经脊髓炎谱系疾病(NMOSD)进行了统计学分析。经统计学分析,GFAP-IgG单阳组与GFAP/AQP4-IgG双阳组的临床特点无明显统计学差异。GFAP-IgG单阳组与AQP4-IgG单阳组在性别比例、发热、头痛、共济失调、行为异常、视觉异常、MRI放射性血管样强化、脑脊液蛋白水平、脑脊液氯水平、脑脊液糖/血糖比值、脑脊液蛋白/脑脊液糖比值等均有统计学差异(P<0.05)。GFAP/AQP4-IgG双阳组与AQP4-IgG单阳组在发热、脑脊液蛋白水平有统计学差异(P<0.05)。【结论】GFAP/AQP4-IgG双阳性脊髓炎相对少见,临床表现上排尿便困难和感觉平面障碍较为突出,脊髓MRI多表现为超过三个脊椎节段的长病灶,与AQP4单阳性的脊髓炎病例的临床特点存在统计学差别。【Objective】To analyze the clinical features of patients with coexisting GFAP-IgG and AQP4-IgG in myelitis.【Methods】We performed a retrospective analysis of patients with myelitis and GFAP-IgG.【Results】Totally 55 cases of autoimmune GFAP astrocytopathy(GFAP-A)were collected.The clinical manifestations included headache,fever,myelitis,abnormal vision,abnormal behavior,ataxia,disturbance of consciousness,epilepsy,dyskinesia,cognitive dys⁃function,and other manifestations.Thirty-one cases were accompanied by myelitis,and 8 cases were GFAP/AQP4 double positive with myelitis.The 8 double-positive cases all showed dysuria and sensory plane disturbance.The MRI of spinal ord showed lesions in eight patients,seven of which had spinal cord lesions more than three segments,and three of which had Gadolinium enhancement.CSF showed the increasing level of white blood cell count or protein in six patients and decreasing level of glucose signally in one patient.In this study,8 cases of GFAP/AQP4 IgG double positive with myelitis,16 cases of GFAP IgG single positive(except AQP4 IgG negative,neuron antibody,oligodendrocyte antibody and glial cell antibody are negative)with myelitis,and 47 cases of AQP4 IgG single positive(without other neural related antibodies)with myelitis NMOSD were selected.There was statistically difference between single positive group with GFAP-IgG(sGFAP-A)and double positive group with GFAP-IgG(dGFAP-A)and AQP4-IgG in fever and CSF protein level(P<0.05).【Conclusions】GFAP/AQP4-IgG double-positive myelitis is relatively rare,which is different from the AQP4 single-positive myelitis in clinical features.The clinical manifestations include urination and defecation difficulties,sensory dysfunction.Spinal cord MRI usually manifests as long lesions extending over three vertebral segments,and cerebrospinal fluid examinations often indicate increased levels of white blood cells or protein.

关 键 词：胶质纤维酸性蛋白 自身免疫性胶质纤维酸性蛋白星形细胞病 抗体 

分 类 号：R74[医药卫生—神经病学与精神病学]