Clinical features of autoimmune glial fibrillary acidic protein astrocytopathy in children: an analysis of 34 cases
WANG Wen-Wen, LI Mei
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/Chongqing Key Laboratory of Pedatrics, Chongqing 400014, China
Abstract Objective To study the clinical features of children with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A). Methods A retrospective analysis was performed on the medical data of 34 children with GFAP-A who attended the Department of Neurology, Children's Hospital of Chongqing Medical University, from January 2020 to February 2022. The medical data included clinical manifestations, cerebrospinal fluid features, imaging examination results, treatment, and prognosis. Results The median age of onset was 8.4 (range 1.9-14.9) years for the 34 children with GFAP-A. The main clinical manifestations included headache (50%, 17/34), fever (47%, 16/34), visual impairment (47%, 16/34), and disturbance of consciousness (44%, 15/34). Abnormal cerebrospinal fluid results were observed in 19 children (56%, 19/34), among whom 8 children had positive autoantibody. The children with overlap syndrome had significantly higher recurrence rate and rate of use of immunosuppressant than those without overlap syndrome (P<0.05). About 77% (24/31) of the children had good response to immunotherapy, and only 1 child had poor prognosis. Conclusions Children with GFAP-A often have non-specific clinical symptoms and show good response to immunotherapy. Children with overlap syndrome have a high recurrence rate, and early application of immunosuppressants may help to prevent recurrence and alleviate symptoms.
WANG Wen-Wen,LI Mei. Clinical features of autoimmune glial fibrillary acidic protein astrocytopathy in children: an analysis of 34 cases[J]. CJCP, 2023, 25(1): 67-72.
WANG Wen-Wen,LI Mei. Clinical features of autoimmune glial fibrillary acidic protein astrocytopathy in children: an analysis of 34 cases[J]. CJCP, 2023, 25(1): 67-72.
Martinez-Hernandez E, Guasp M, García-Serra A, et al. Clinical significance of anti-NMDAR concurrent with glial or neuronal surface antibodies[J]. Neurology, 2020, 94(22): e2302-e2310. PMID: 32161029. DOI: 10.1212/WNL.0000000000009239.
Iorio R, Damato V, Evoli A, et al. Clinical and immunological characteristics of the spectrum of GFAP autoimmunity: a case series of 22 patients[J]. J Neurol Neurosurg Psychiatry, 2018, 89(2): 138-146. PMID: 28951498. DOI: 10.1136/jnnp-2017-316583.
Flanagan EP, Hinson SR, Lennon VA, et al. Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: analysis of 102 patients[J]. Ann Neurol, 2017, 81(2): 298-309. PMID: 28120349. DOI: 10.1002/ana.24881.
Yang X, Liang J, Huang Q, et al. Treatment of autoimmune glial fibrillary acidic protein astrocytopathy: follow-up in 7 cases[J]. Neuroimmunomodulation, 2017, 24(2): 113-119. PMID: 28922662. DOI: 10.1159/000479948.
Long Y, Liang J, Xu H, et al. Autoimmune glial fibrillary acidic protein astrocytopathy in Chinese patients: a retrospective study[J]. Eur J Neurol, 2018, 25(3): 477-483. PMID: 29193473. DOI: 10.1111/ene.13531.
Huang Q, Yang H, Liu T, et al. Patients with suspected benign tumors and glial fibrillary acidic protein autoantibody: an analysis of five cases[J]. Int J Neurosci, 2019, 129(12): 1183-1188. PMID: 31327295. DOI: 10.1080/00207454.2019.1645140.
Dubey D, Hinson SR, Jolliffe EA, et al. Autoimmune GFAP astrocytopathy: prospective evaluation of 90 patients in 1 year[J]. J Neuroimmunol, 2018, 321: 157-163. PMID: 29793728. DOI: 10.1016/j.jneuroim.2018.04.016.
Handoko M, Hong W, Espineli E, et al. Autoimmune glial fibrillary acidic protein astrocytopathy following herpes simplex virus encephalitis in a pediatric patient[J]. Pediatr Neurol, 2019, 98: 85-86. PMID: 31248671. DOI: 10.1016/j.pediatrneurol.2019.05.010.