case_images: PMC10726202_01
case_images: Brain and spinal MRI of a subject with autoimmune GFAP astrocytopathy. (A, B) The cranial MRI T2 FLAIR sequences: multiple patchy abnormal signals beside the anterior and posterior horns of the bilateral lateral ventricles and in the center of the semiovals(white arrow). (C) Diffusion Weighted Imaging(no high signal seen). (D-F) The gadolinium-enhanced T1-weighted image shows patchy enhancement of thoracic spine 11- lumbar spine 1 (white arrows). (G) Spinal digital subtraction angiography: the third lumbar artery on the right side of the vertebral canal to the supply of the anterior spinal artery in the spinal canal about the thoracic 10 position(white arrows).
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case_images: PMC10726202_01_fimmu-14-1293425-g001.jpg
case_images: f1
case_images: MRI showed multiple patchy abnormal signals beside the anterior and posterior horns of the bilateral lateral ventricles and in the center of the semiovals, with isosignal in T1Flair, high signal in T2WI, and no high signal in DWI (Figures 1A-C). MRI/Magnetic Resonance Angiography (MRA) of thoracolumbar segment showed striated long T2 signal foci from T11-L1 spinal cord, enhancement was visible; striated T2WI sequence slightly high signal shadow was seen in the spinal cord at the level of T11-12 vertebral body, enhancement scan was visible as striated enhancement, and vascular malformation was considered (Figures 1D-F). DSA, confirmed that the third lumbar artery on the right side of the vertebral canal to the supply of the anterior spinal artery in the spinal canal about the thoracic 10 position (Figure 1G). Due to the very small fistulae of PMAVFs, as well as the improvement of the patient's symptoms with out-of-hospital dexamethasone, after a comprehensive evaluation, the patient was considered to have this symptom due to autoimmune GFAP astrocytopathy, and was given methylprednisolone 500 mg (x3d) intravenously, which was changed to 250 mg (x3d) after 3 days, and then changed to prednisone acetate 60 mg/d orally after 3 days.
case_images: Glial fibrillary acidic protein (GFAP-IgG) by GFAP-transfected cell-based immunofluorescence assay. Cells were expressing green fluorescent protein-tagged GFAP (green) and immunostained (red if positive). (A-C) Examination of cerebrospinal fluid (CSF) in first admission. Merged images revealed the colocalization of the GFAP antibody and astrocyte (white arrows).
case_images: fimmu-14-1293425-g002.jpg
case_images: PMC10726202_01_fimmu-14-1293425-g002.jpg
case_images: f2
case_images: Serum/CSF was positive for anti-GFAP antibodies (1:32) (Figures 2A-C) and negative for anti-AQP4, anti-MOG and anti-NMDAR.