Magnetic Resonance Imaging Characteristics of Autoimmune Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy: A Pediatric Series in Southwest China

作者全名："Cheng, Weiqin; He, Ling; Luo, Hechuan; Jiang, Yan; Tan, Chengbing; Fan, Xiao"

作者地址："[Cheng, Weiqin; He, Ling; Luo, Hechuan; Fan, Xiao] Chongqing Med Univ, Natl Clin Res Ctr Child Hlth & Disorders, Dept Radiol, Childrens Hosp,Minist Educ,Key Lab Child Dev & Dis, Chongqing, Peoples R China; [Jiang, Yan; Tan, Chengbing] Chongqing Med Univ, Natl Clin Res Ctr Child Hlth & Disorders, Dept Neurol, Childrens Hosp,Chongqing Key Lab Pediat,Minist Edu, Chongqing, Peoples R China; [Fan, Xiao] Chongqing Med Univ, Childrens Hosp, Dept Radiol, 136 Zhongshan Er Rd, Chongqing 400014, Peoples R China"

通信作者："Fan, X (通讯作者)，Chongqing Med Univ, Childrens Hosp, Dept Radiol, 136 Zhongshan Er Rd, Chongqing 400014, Peoples R China."

来源：NEUROPSYCHIATRIC DISEASE AND TREATMENT

ESI学科分类：NEUROSCIENCE & BEHAVIOR

WOS号：WOS:001041942600001

JCR分区：Q2

影响因子：2.5

年份：2023

卷号：19

期号：　

开始页：1685

结束页：1693

文献类型：Article

关键词：glial fibrillary acidic protein; GFAP; astrocytopathy; autoimmune; magnetic resonance imaging; children

摘要："Objective: To investigate and summarize the magnetic resonance imaging (MRI) manifestations of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children.Methods: We retrospectively analyzed data from 17 pediatric patients with autoimmune GFAP astrocytopathy confirmed by the detection of GFAP autoantibodies in cerebrospinal fluid in our single-center. Furthermore, we reviewed current literature and summarized previous findings on the MRI characteristics of this disease in children.Results: In these 17 patients, the clinical manifestations and results of CSF analysis were suggestive of autoimmune disorder, with a good improvement. The lesions on MRI were most commonly located in the bilateral basal ganglia (70.6%), thalamus (64.7%), cerebral white matter (29.4%). 93.3% of the cerebral lesions were relatively scattered and small, 80% of the spinal lesions presented as longitudinally extensive ones. Both periventricular radial linear (PVRL) (53.8%) and punctate or linear enhancement in basal ganglia and thalamus (53.8%) were commonly observed, followed by the leptomeningeal enhancement (46.2% in the brain and 62.5% in the spinal cord). We then included 55 pediatric patients with MRI data from current literature in our analysis (n = 72, 44 males). Our results revealed similar MRI findings but the enhancement pattern between our series and previously published cases, that is, leptomeningeal enhancement in the brain 46.2% vs 31.4%, in spinal cord 62.5% vs 18.4%, and PVRL enhancement 53.8% vs 11.2%. There were no detailed reports on punctate or linear enhancement.Conclusion: The MRI characteristics of autoimmune GFAP astrocytopathy in children could be suggestive. Scattered and small lesions (especially punctate or linear) in the bilateral thalamus, basal ganglia, and white matter, as well as longitudinally extensive spinal cord lesions (if present), with punctate, PVRL and leptomeningeal enhancement might be a distinct indication for the early diagnosis of this disorder."

基金机构：clinical new technology project in the Children's Hospital of Chongqing Medical University [202026]

基金资助正文：Funding This work was supported by the clinical new technology project in the Children's Hospital of Chongqing Medical University [grant number 202026] .