Clinical characteristics of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children: A case series of 16 patients

作者全名："Li, Tianyi; Li, Xiujuan; Hong, Siqi; Jiang, Li; Ma, Jiannan"

作者地址："[Li, Tianyi; Li, Xiujuan; Hong, Siqi; Jiang, Li; Ma, Jiannan] Chongqing Med Univ, Natl Clin Res Ctr Child Hlth & Disorders, Chongqing Key Lab Pediat, Dept Neurol,Minist Educ,Key Lab Child Dev & Disord, Chongqing, Peoples R China; [Ma, Jiannan] Chongqing Med Univ, Dept Neurol, Childrens Hosp, 136 Zhongshan 2nd Rd, Chongqing 400014, Peoples R China"

通信作者："Ma, JN (通讯作者)，Chongqing Med Univ, Dept Neurol, Childrens Hosp, 136 Zhongshan 2nd Rd, Chongqing 400014, Peoples R China."

来源：JOURNAL OF NEUROIMMUNOLOGY

ESI学科分类：NEUROSCIENCE & BEHAVIOR

WOS号：WOS:001076657000001

JCR分区：Q2

影响因子：2.9

年份：2023

卷号：382

期号：　

开始页：　

结束页：　

文献类型：Article

关键词：Glial fibrillary acidic protein; GFAP-IgG; Encephalitis; Autoimmune encephalitis; Children

摘要："Purpose: To investigate the clinical characteristics of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy in children. Methods: We reviewed the medical records of Children's Hospital of Chongqing Medical University from January 2020 to September 2021 and retrospectively analysed the clinical features, magnetic resonance imaging (MRI) findings, laboratory findings, treatment and outcome of children with autoimmune GFAP astrocytopathy. Results: Sixteen patients were included: 6 and 10 tested positive for GFAP-IgG in cerebrospinal fluid (CSF) and both CSF and serum, respectively. The median patient age was 115 months (range: 36-180 months), and 7 patients (43.8%) were male. All patients had the clinical syndrome of encephalitis/meningoencephalitis with or without myelitis: encephalitis (8), meningoencephalitis (3), encephalomyelitis (1) and meningoencephalomyelitis (4). The most common clinical symptoms were fever (11), altered consciousness (11), headache (10) and seizure (9). Four patients developed central respiratory failure for which mechanical ventilation was needed. All patients showed hyperintense T2-weighted lesions on brain MRI in the cerebral white matter (13), brainstem (11), basal ganglia (11), thalamus (9), and cerebellum (3). Nine patients (56%) had abnormal hyperintense lesions in the bilateral basal ganglia and thalamus. Six of 12 patients who underwent gadolinium-enhanced brain MRI showed abnormal enhancement images, and five of them showed linear perivascular radial enhancement. The modified Rankin scale (mRS) score decreased significantly in most patients after immunotherapy. Two patients with coexisting neural autoantibodies relapsed; however, 15 patients who were followed up successfully had favorable outcomes at the last follow-up.Conclusion: Children with autoimmune GFAP astrocytopathy usually have a clinical syndrome of encephalitis/ meningoencephalitis with or without myelitis. Except for the linear perivascular radial gadolinium enhancement pattern, hyperintense lesions in the bilateral basal ganglia and thalamus might be another characteristic brain MRI finding of autoimmune GFAP astrocytopathy in children. Although a few patients with coexisting neural autoantibodies might relapse, children with autoimmune GFAP astrocytopathy usually have favorable outcomes after immunotherapy."

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