Anti-GFAP Antibody-Associated Hypertrophic Pachymeningitis

作者全名："Tan, Chengbing; Zhong, Min; Yao, Zhengxiong; Hong, Siqi; Jiang, Li; Jiang, Yan"

作者地址："[Tan, Chengbing; Zhong, Min; Yao, Zhengxiong; Hong, Siqi; Jiang, Li; Jiang, Yan] Chongqing Med Univ, Natl Clin Res Ctr Child Hlth & Disorders, Dept Neurol, Minist Educ,Key Lab Child Dev & Disorders,Childre, Chongqing, Peoples R China; [Tan, Chengbing; Zhong, Min; Yao, Zhengxiong; Hong, Siqi; Jiang, Li; Jiang, Yan] Chongqing Key Lab Translat Med Res Cognit Dev & L, Chongqing, Peoples R China"

通信作者："Jiang, Y (通讯作者)，136 Zhongshan 2nd Rd, Chongqing 400014, Peoples R China."

来源：NEUROPEDIATRICS

ESI学科分类：NEUROSCIENCE & BEHAVIOR

WOS号：WOS:000754050200001

JCR分区：Q3

影响因子：1.4

年份：2022

卷号：53

期号：2

开始页：143

结束页：145

文献类型：Article

关键词：autoimmune glial fibrillary acidic protein astrocytopathy; hypertrophic pachymeningitis; autoimmune inflammation; MRI

摘要："Background Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is an inflammatory central nervous system (CNS) disorder that usually presents as steroid responsive encephalitis, meningitis, myelitis, or meningoencephalomyelitis. Hypertrophic pachymeningitis (HP) is an uncommon disorder that causes a localized or diffuse thickening of the dura mater. Depending on the etiology, HP can be idiopathic or secondary to a wide variety of other diseases. There are no reports of autoimmune GFAP astrocytopathy presenting as HP. Methods In this case report, we describe a rare case of pediatric HP possibly associated with anti-GFAP antibody. Results A 13-year-old previously healthy girl presented with headache for nearly 8 months with left-sided peripheral facial palsy and left-sided abductor nerve palsy in the second month of course. Magnetic resonance imaging (MRI) of the brain revealed contrast enhancement of hypertrophic dura mater. Anti-GFAP alpha antibodies were positive in serum and cerebrospinal fluid. The patient improved clinically after steroid treatment with partial resolution of abnormal intracranial MRI lesions. Conclusion The present study suggests that HP may be one of the clinical phenotypes for autoimmune GFAP astrocytopathy or GFAP antibody is a biomarker for HP."

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