PMC7270931_01
The immunoreactivity of the patient's glial fibrillary acidic protein-immunoglobulin G
(GFAP-IgG) in cerebrospinal fluid (CSF) and aquaporin 4 (AQP4)-IgG in the serum. Using
transfected cell-based immunofluorescence assays, GFAP-IgG was detected in the CSF (a)
and AQP4-IgG was detected in the serum (b).
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GFAP-IgG in the CSF (Figure 1a) and aquaporin 4 (AQP4)-IgG in serum (Figure 1b). GFAP and AQP4 antibodies in the CSF and serum were assessed by an immunofluorescence assay using rat hippocampus tissue, and by a cell-based assay using HEK293 cells transfected with GFAP and AQP4, as reported previously.
Brain and spinal cord magnetic resonance imaging (MRI) performed before systemic
treatment. Contrast enhanced brain MRI was normal (a). Spinal cord MRI sagittal and
coronal sequences show longitudinally extensive T2-hyperintense lesions with poorly
defined margins [medulla oblongata (b, arrow), cervical (b, arrowhead), thoracic (c,
arrow), lumbar (c, arrowhead) spinal cord] accompanied by spinal cord central canal,
patchy, punctate, and pia gadolinium enhancement on the medulla oblongata (d, e; arrow)
and the cervical (d, e; arrowhead), thoracic (f, arrow) and lumbar (f, arrowhead) spinal
cord.
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Interestingly, spinal cord MRIs showed longitudinally extensive spinal cord lesions involving the cervical, thoracic, and lumbar spinal cord in T2-weighted images, with spinal cord central canal, patchy, punctate, and pia enhancement lesions extending up to the medulla oblongata in T1-weighted images (Figure 2). The most common characteristic of autoimmune GFAP astrocytopathy (linear, radial perivascular patterns of enhancement throughout the cerebral white matter) was not found in this patient (Figure 2a).
Spinal cord magnetic resonance imaging (MRI) performed 3 months after onset. Spinal
cord MRI sagittal sequence show residual T2 hyperintensities [medulla oblongata (a,
arrow), cervical (a, arrowhead), thoracic (b, arrow), lumbar (c) spinal cord] and
T1-gadolinium enhancement [medulla oblongata (d, arrow), cervical (d, arrows), thoracic
(e, arrows), lumbar (f, arrow) spinal cord], without evidence of new lesions.
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Repeated MRI scans were performed, showing residual T2 hyperintensities and T1-gadolinium enhancement in the spinal cord and medulla oblongata with no evidence of new lesions (Figure 3).