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. 2021 Jan 25:8:626564.
doi: 10.3389/fped.2020.626564. eCollection 2020.

Glial Fibrillary Acidic Protein Astrocytopathy in Pediatric Patients: A Retrospective Study

Haixia Huang  1   2 , Ke Bai  1   2 , Yueqiang Fu  1   2 , Siwei Lu  1   2 , Yunni Ran  1   2 , Hongxing Dang  1   2 , Jing Li  1   2
Affiliations

Glial Fibrillary Acidic Protein Astrocytopathy in Pediatric Patients: A Retrospective Study

Haixia Huang et al. Front Pediatr. .

Abstract

Autoimmune glial fibrillary acidic protein astrocytopathy is a novel form of autoimmune meningoencephalitis related to GFAP autoantibodies. This condition is still being characterized, and few pediatric patients have been identified. Here, we report three patients presenting with fever, nausea, and headache, following progressive disturbance of consciousness, limb weakness, dyspnea, or urine retention. MRI analysis revealed that T2-hyperintense lesions, or enhancement of the meninges and spinal cord. CSF and serum analyses revealed they were positive for GFAP antibody, confirming GFAP astrocytopathy diagnosis. Treating the patients with IVIG, with or without intravenous steroids, gradually improved their clinical symptoms. Our findings indicate that GFAP astrocytopathy should be considered in children who are clinically diagnosed with meningoencephalitis, whether or not myelitis is present, and if the MRI reveals enhancement of meninges or spinal cord, T2-hyperintense lesions, or a pattern of linear perivascular gadolinium enhancement. Suspected cases should be tested for GFAP antibody as soon as possible because these patients may benefit from immunotherapy.

Keywords: child; glial fibrillary acidic protein astrocytopathy; meningoencephalitis; myelitis; steroids.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Axial fluid attenuation inversion recovery (FLAIR) MRI brain (A) upon admission and (B) 2 months later. Image (A) showed T2-hyperintense lesions in in bilateral thalamus and basal ganglia. Follow-up image (B) showed improved T2 lesions. MRI of the spine (C) 10 days after admission showed leptomeningeal enhancement in the cervical cord.
Figure 2
Figure 2
Axial fluid attenuation inversion recovery (FLAIR) MRI brain (A) 7 days after admission and (B) 3 weeks later. Image (A) showed the enhancement in the surface of the large and small brain as well as brainstem (not shown). Image (B) showed meningeal enhancement was reduced. Spine MRI (C) 10 days after admission and (D) 3 weeks later. Image (C) showed the enhancement of spine. And Image (D) showed the enhancement was reduced after the immunotherapy treatment.
Figure 3
Figure 3
Axial fluid attenuation inversion recovery (FLAIR) MRI brain (A,C) 12 days after admission and (B,D) 1 month later. Image (A) showed T2-hyperintense lesions in internal capsule, hind limbs and external capsule. Image (C) showed T2-hyperintense lesions in brain stem. Follow-up images (B,D) showed improved T2 lesions.
Figure 4
Figure 4
The glial fibrillary acidic protein autoantibodies in the cerebrospinal fluid of the patients were transfected into cell-indirect immunofluorescence detection: human embryonic kidney 293T (HEK293T), after cell fixation and permeabilization, immunofluorescence detection of GFAP autoantibodies in the sample. In the picture, green is the cells transfected to express GFAP, and red is human IgG that binds to the corresponding antigen.
See this image and copyright information in PMC

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