PMC9136286_01
MRI at time of first relapse: (A) Axial T2 FLAIR sequence of the brain showing diffuse corpus callosum involvement. (B) Axial T1 with gadolinum showing patchy enhancement of the lesion in the right splenium of the corpus callosum. (C) Axial DWI sequence of the brain showing diffusion restriction. (D) T2 STIR sequence of the lower cervical spine and thoracic spine showing a central hyperintensity extending from the level of T1-T3. MRI after treatment of first relapse with corticosteroids: (E) Axial T2 FLAIR sequence of the brain showing an improvement of the corpus callosum lesions after the steroid course. (F) Axial T1 with gadolinium showing a resolution of the patchy enhancement after the steroid course. MRI immediately prior to eculizumab: (G) Axial T2 FLAIR sequence of the brain showing an increased T2 signal in the right optic nerve in the mid intraorbital course with focal atrophy of the left optic nerve. (H) Axial T1 with Gadolinium sequence of the orbits before starting eculizumab showing mild enhancement of both optic nerves. MRI after eculizumab: (I) Axial T2 FLAIR sequence of the brain showing stable deep periventricular lesions without any new lesions identified. (J) Axial T1 with gadolinium showing no enhancing lesions. (K) Axial T2 FLAIR sequence of the brain showing a decrease in the intensity of the T2 signal in the right optic nerve. (L) Axial T1 with gadolinium shows resolution of the previously enhancing optic nerve lesions.
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F1
She had an MRI of the brain that showed diffuse involvement of the corpus callosum, particularly the splenium, with diffusion restriction and patchy enhancement (Figures 1A-C), and a lesion in the right superior cerebellar peduncle. She also had an MRI of the spinal cord, which showed a lesion in the high thoracic cord centered on the left side from T1 through T3 (Figure 1D). MRI of the brain showed an improvement in the lesions with a resolution of the enhancement (Figures 1E,F). After 2 cycles of rituximab she was found to have worsening vision in her right eye from 20/80 to 20/160 along with new enhancement along the intraorbital portions of the optic nerves consistent with a bilateral optic neuritis (Figures 1G,H). Repeat MRI brain showed no new lesion and resolution of the enhancement in the optic nerves (Figures 1I-L).
Brain biopsy with (A) Luxol Fast Blue staining of myelin shows a sharply demarcated area of myelin loss. (B) This area is infiltrated by lipid-laden macrophages, labeled by CD163. (C) The area with the green star demonstrates partial loss of aquaporin-4 astrocytes. (D) There is also depletion of GFAP positive astrocytes in this area. (E) Under high power (yellow star area on first slide), we observed the edge of the demyelinated lesion. (F) There are sparse GFAP positive astrocytes and (G) aquaporin-4 positive astrocytes present around blood vessels. (H) Olig-2 stain shows loss of oligodendrocytes.
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F2
Her former biopsy slides were obtained and reviewed again; there was extensive loss of AQP4+ and GFAP+ astrocytes supporting the diagnosis of NMOSD (Figures 2A-D). In addition, sparse AQP4+ and GFAP+ astrocytes were observed in clusters around blood vessels, perhaps suggesting an early repair phenomenon (Figures 2E-H).
PMC9136286_02
MRI at time of first relapse: (A-C) Axial T2 FLAIR sequence of the brain showing involvement of the corpus callosum, bilateral mesial temporal lobes, bilateral inferior frontal lobes, and bilateral pons and midbrain. (D) Axial sequence of the brain with gadolinium showing discrete enhancing lesions in the corpus callosum. MRI after treatment of first relapse with corticosteroids and prior to eculizumab: (E-G) Axial T2 FLAIR sequences of the brain showing an improvement in the mesial temporal lobe and corpus callosum lesions after a course of steroids. (H) Coronal T1 sequences with gadolinium showing a new small right inferomedial frontal lesion with patchy enhancement. MRI after eculizumab: (I-K) Axial T2 FLAIR sequences of the brain showing stable inferior frontal and temporal lobe and corpus callosum lesions after starting eculizumab. (L) Axial T1 sequences with gadolinium showing no enhancement.
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F3
MRI of the brain revealed T2 hyperintensities in the left frontal lobe, left insula, bilateral basal ganglia, corpus callosum, bilateral mesial temporal lobes, bilateral inferior frontal lobes, and bilateral pons and midbrain (Figures 3A-C). There were discrete enhancing lesions visualized in several places in the corpus callosum (Figure 3D). On follow-up, repeat MRI showed a small, 7 mm, new right inferomedial frontal lesion with patchy enhancement, although she did not complain of any new cognitive issues (Figures 3E-H). MRI of the brain showed stable lesions with a resolution of the enhancement (Figures 3I-L).