自身免疫性胶质纤维酸性蛋白（GFAP）星形细胞病的磁共振成像特征：中国西南部的儿科病例系列
Magnetic Resonance Imaging Characteristics of Autoimmune Glial Fibrillary Acidic Protein (GFAP) Astrocytopathy: A Pediatric Series in Southwest China.
作者信息
Cheng Weiqin, He Ling, Luo Hechuan, Jiang Yan, Tan Chengbing, Fan Xiao
机构信息
Department of Radiology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, People's Republic of China.
Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, People's Republic of China.
出版信息
Neuropsychiatr Dis Treat. 2023 Jul 29;19:1685-1693. doi: 10.2147/NDT.S417492. eCollection 2023.
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.
目的
探讨并总结儿童自身免疫性胶质纤维酸性蛋白（GFAP）星形细胞病的磁共振成像（MRI）表现。
方法
我们回顾性分析了单中心17例经脑脊液检测GFAP自身抗体确诊的儿童自身免疫性GFAP星形细胞病患者的数据。此外，我们查阅了当前文献并总结了先前关于该疾病儿童MRI特征的研究结果。
结果
在这17例患者中，临床表现和脑脊液分析结果提示自身免疫性疾病，且病情有明显改善。MRI上的病变最常见于双侧基底节（70.6%）、丘脑（64.7%）、脑白质（29.4%）。93.3%的脑部病变相对散在且较小，80%的脊髓病变表现为纵向广泛分布。常见的有脑室周围放射状线性强化（PVRL）（53.8%）以及基底节和丘脑的点状或线性强化（53.8%），其次是软脑膜强化（脑部为46.2%，脊髓为62.5%）。然后我们将当前文献中55例有MRI数据的儿童患者纳入分析（共72例，44例男性）。我们的结果显示，我们的系列研究与先前发表病例的MRI表现相似，但强化模式有所不同，即脑部软脑膜强化分别为46.2%和31.4%，脊髓为62.5%和18.4%，PVRL强化为53.8%和11.2%。关于点状或线性强化没有详细报道。
结论
儿童自身免疫性GFAP星形细胞病的MRI特征具有提示意义。双侧丘脑、基底节和白质中散在的小病变（尤其是点状或线性病变），以及纵向广泛的脊髓病变（如果存在），伴有点状、PVRL和软脑膜强化，可能是该疾病早期诊断的明显指征。
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