case_images: PMC10904644_01
case_images: Imaging findings in the case report 1. Sagittal T2-weighted (A) and axial FLAIR MRI scans (B) at the time of symptom onset showing multiple hyperintense small lesions in the white matter, predominantly affecting corpus callosum in the form of "spokes." Axial black blood MRI scans at the time of symptom onset (C.I), showing microangiopathy as multiple small, hypersignal nodules in small vessel walls combined with discreet leptomemingeal enhancement (marked with arrows); and after 6 months (C.II), showing regression of the pathology. Fluorescein angiogram (left eye) at the time of symptom onset (D.I), where the areas of hyperfluorescence mark leakage from inflamed retinal arterioles (red arrows) and the area of fluorescence decrease corresponds to the lower nasal arteriolar occlusion (precapillary plaque) with segmentation (black arrows). Fluorescein angiogram (left eye) after 8 months (D.II) shows the absence of leakage and reperfusion of the previously occluded retinal arterioles (black arrows).
case_images: fneur-15-1339438-g001.jpg
case_images: PMC10904644_01_fneur-15-1339438-g001.jpg
case_images: fig1
case_images: Magnetic resonance (MRI) scans of the head showed several lesions (Figures 1A,B) in the corpus callosum and in the left precuneus with diffusion restriction; black blood MRI (Figure 1C.I) detected inflammatory microangiopathy, with gadolinium enhancement of the small vessel walls, dot-like infarctions, and focal leptomeningeal enhancement. SuS, fundus fluorescein angiography (FAG) was performed, detecting regional hyperfluorescence and branch retinal artery occlusion with segmentation (Figure 1D). Since the findings were consistent with suspected SuS, the patient was treated with a pulse of methylprednisolone (total dose of 5 g) followed by high-dose oral prednisone (1 mg/kg) with a slow taper. Black blood MRI (Figure 1C.II) revealed regression of leptomeningeal and vessel wall enhancement.
case_images: PMC10904644_02
case_images: Imaging findings in the case report 2. Axial FLAIR MRI scans (A) at the time of symptom onset showing multiple hyperintense "snowball" lesions in the white matter. Axial average diffusion-weighted scans (B.I) at the time of symptom onset and apparent diffusion coefficient maps (B.II), with small areas of restriction of diffusion (hypersignal in B.I and hyposignal in B.II), corresponding to small infarctions. Black blood MRI scans at the time of symptom onset (C.I), detecting microangiopathy (multiple small hypersignal nodules of small vessel walls) in both supra- and infratentorial region; and after 6 months (C.II), with regression of the initial pathology, but development of substantial atrophy.
case_images: fneur-15-1339438-g002.jpg
case_images: PMC10904644_02_fneur-15-1339438-g002.jpg
case_images: fig2
case_images: Brain MRI detected multiple areas of restriction of diffusion predominantly in the corpus callosum, in bilateral subcortical white matter and in the cerebellum (Figure 2). Black blood MRI showed gadolinium enhancement of small vessel walls (Figure 2C.I), similarly to the first patient. FLAIR hyperintensities and no enhancement of vessel wall (Figure 3C) or leptomeningeal enhancement in black blood sequences (Figure 2C.II), confirming the remission of the disease. FAG performed after 6 months showed revascularization of small vessel occlusions present in the previous examination; no areas of hyperfluorescence were detected (Figure 4B).
case_images: Imaging findings in the case report 2. Axial FLAIR MRI scans (A) about 1.5 months after the symptom onset, showing cystoid degeneration of previous hypersignal areas and several new lesions. Axial average diffusion-weighted scans (B.I) and apparent diffusion coefficient maps (B.II) about 1.5 months after the symptom onset, with several new lesions causing diffusion restriction, and 6 months after the symptom onset (C.I,C.II), where no new lesions are apparent.
case_images: fneur-15-1339438-g003.jpg
case_images: PMC10904644_02_fneur-15-1339438-g003.jpg
case_images: fig3
case_images: Brain MRI scan showed multiple new dot-like areas of diffusion restriction (Figures 3A,B), vessel wall, and leptomeningeal enhancement. FLAIR hyperintensities and no enhancement of vessel wall (Figure 3C) or leptomeningeal enhancement in black blood sequences (Figure 2C.II), confirming the remission of the disease. FAG performed after 6 months showed revascularization of small vessel occlusions present in the previous examination; no areas of hyperfluorescence were detected (Figure 4B).
case_images: Imaging findings in the case report 2 - Fluorescein angiogram of the left (A.I) and right eye (A.II) 1.5 months after symptom onset, where the areas of hyperfluorescence mark leakage from inflamed retinal arterioles (red arrow) and the areas of fluorescence decrease correspond to arteriolar occlusion (precapillary plaque) (black arrows). Follow-up fluorescein angiogram (left eye) after 6 months (B) showing revascularization of small vessel occlusions present in the previous examination (black arrow); no areas of hyperfluorescence were detected.
case_images: fneur-15-1339438-g004.jpg
case_images: PMC10904644_02_fneur-15-1339438-g004.jpg
case_images: fig4
case_images: FAG performed at this stage showed multiple branch retinal artery occlusions and some areas of hyperfluorescence (Figure 4A). FLAIR hyperintensities and no enhancement of vessel wall (Figure 3C) or leptomeningeal enhancement in black blood sequences (Figure 2C.II), confirming the remission of the disease. FAG performed after 6 months showed revascularization of small vessel occlusions present in the previous examination; no areas of hyperfluorescence were detected (Figure 4B).