摘要
目的探讨自身免疫性胶质纤维酸性蛋白(glialfibrillaryacidicprotein,GFAP)星形细胞病患者的临床特征。方法回顾性分析2019年1月~2021年5月在华中科技大学同济医学院附属同济医院就诊的脑脊液GFAP抗体阳性的27例自身免疫性GFAP星形细胞病患者病例资料,采用改良的Rankin量表(mRS)评估预后(0~2分为预后好)。结果患者平均发病年龄44岁(14~72岁),74%为男性。主要首发表现为发热(21/27)、头痛(19/27)、视物模糊(9/27)、精神症状(8/27)、自主神经功能障碍(8/27)、肢体无力(6/27)和意识障碍(3/27)。单纯脊髓炎少见(3/27)。74%的患者病情恶化,10例因呼吸衰竭需机械通气(37%),6例(22%)出现周围神经损害。头部MRI病变主要位于脑室旁白质(16/24)、基底节(12/24)、脑干(10/24)、丘脑(8/24)和小脑(7/24);脊髓病变多长节段累及颈髓(10/19)和胸髓(13/19);最常见脑室旁线样放射状强化(11/24)和室管膜周强化(7/24)以及脊膜强化(12/19)和脊髓中央管强化(9/19)。85%的患者脑脊液呈淋巴细胞为主的炎性反应。9例患者(33%)伴其他自身抗体阳性,无患者发现肿瘤。多数患者对激素和免疫球蛋白治疗反应好,中位随访时间16个月,超过80%患者预后好。结论自身免疫性GFAP星形细胞病并不罕见,临床表现缺乏特异性。特征性MRI影像[脑室旁线样放射状强化、室管膜周强化和(或)脊髓长节段受累伴中央管强化]和淋巴细胞为主的脑脊液炎性反应有重要提示意义,应及时检测脑脊液GFAP-IgG。多数患者对免疫治疗有效,预后好。
Objective To describe the clinical characteristics of patients with autoimmune glial fibrillary acidic protein(GFAP)astrocytopathy.Methods From January 2019 to May 2021,data from 27 new patients with autoimmune GFAP astrocytopathy confirmed by detection of GFAP-IgG in cerebrospinal fluid(CSF)were retrospectively reviewed.Clinical outcomes were defined by modified Rankin scale(mRS)score as good(0-2)vs.poor(>2).Results Average age at symptom onset was 44 years(range 14-72),and 74%were male.The initial symptoms included fever(21/27),headache(19/27),vision blur(9/27),psychosis(8/27),autonomic dysfunction(8/27),limb weakness(6/27),and consciousness disturbance(3/27).The clinical manifestations of 74%patients deteriorated during hospitalization.Ten patients(37%)with respiratory failure needed mechanical ventilation,6 cases(22%)had peripheral neuropathy.Isolated myelitis was rare(3/27).Twenty-four patients had brain magnetic resonance imaging(MRI)abnormalities in the cerebral white matter(16/24),basal ganglia(12/24),brainstem(10/24),thalamus(8/24)and cerebellum(7/24).Radial linear periventricular enhancement(11/24)and ependymal enhancement(7/24)were notable on T1-weighted postgadolinium sequences.Spine MRI images revealed longitudinally extensive spinal cord lesions in cervical cord(10/19)and thoracic cord(13/19)accompanied by leptomeningeal enhancement(12/19)and central canal enhancement(9/19).Twenty-three patients(85%)showed inflammatory CSF findings with lymphocyte-predominant pleocytosis.Nine patients(33%)had other autoimmune-related antibodies,and no patients had tumors.With a median follow-up of 16 months,most patients responded well to immunotherapy(corticosteroids and/or IVIg),and 80%had a good outcome.Conclusion Autoimmune GFAP astrocytopathy is not rare,and the clinical manifestations are diverse and lack specificity.Characteristic MRI manifestations(radial linear periventricular enhancement,ependymal enhancement and/or longitudinally extensive lesions of spinal cord with central canal enhancement)and CSF findings of lymphocyte-predominant plecytosis are important indicators.Prompt detection of GFAP-IgG in CSF is essential for differentiation.Most patients are sensitive to immunotherapy and have a good outcome.
作者
梁奇明
许峰
张萍
邓刚
季苏琼
戴慧慧
王芙蓉
朱遂强
连立飞
Liang Qiming;Xu Feng;Zhang Ping(Department of Neurology,Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430030,China)
出处
《华中科技大学学报（医学版）》
CAS
CSCD
北大核心
2021年第5期620-625,共6页
Acta Medicinae Universitatis Scientiae et Technologiae Huazhong
基金
湖北省卫生健康委员会面上项目(No.WJ2019M121)。
关键词
胶质纤维酸性蛋白星形细胞病
脑炎
脑膜脑炎
脊髓炎
自身免疫性脑脊髓膜炎
glial fibrillary acidic protein astrocytopathy
encephalitis
meningoencephalitis
myelitis
autoimmune meningoencephalomyelitis
