PMC6908508_01
Initial MRI brain without and with gadolinium. Axial fluid-attenuated inversion recovery (FLAIR) sequences (A,B) illustrate scattered T2 FLAIR hyperintensities in the right temporal and parietal lobes involving the cortex and subcortical white matter. The lesions did not demonstrate contrast enhancement, and no leptomeningeal enhancement was noted either (C). Susceptibility-weighted imaging (SWI) sequence shows numerous bihemispheric microhemorrhages, in the cortical and subcortical white matter (D).
fneur-10-01283-g0001.jpg
PMC6908508_01_fneur-10-01283-g0001.jpg
F1
Brain MRI with contrast demonstrated abnormal T2-weighted fluid-attenuated inversion recovery signal hyperintensity in the cortical and subcortical right temporal, parietal, and occipital lobes without contrast enhancement (Figure 1).
MRI brain without and with contrast approximately 3 weeks after the initial scan. Axial FLAIR sequence demonstrates significant progression of the hyperintense lesions in the right temporal, occipital, and fronto-parietal areas, with mild mass effect on the right lateral ventricle and midline shift (A,B). No contrast enhancement is appreciated (C). SWI sequence shows stable, diffuse, bihemispheric microhemorrhages (D).
fneur-10-01283-g0002.jpg
PMC6908508_01_fneur-10-01283-g0002.jpg
F2
Repeat neuroimaging was significant for more extensive, confluent, non-enhancing right hemispheric white matter hyperintensities; and stable diffuse microhemorrhages (Figure 2).
Brain biopsy stained with hematoxylin and eosin discloses thickened microvasculature with amorphous hyaline-like material replacing the media and associated with mononuclear as well as granulomatous infiltrates (arrows on A). Congo Red stain without (B) and with polarization (C) highlights congophilic (B) bi-refringent (C) amyloid deposits, immunoreactive with antibody to Abeta (D). Immunophenotyping of the inflammatory cells shows staining for macrophage marker CD163 (E-G), T lymphocyte marker CD8 (H), and cytotoxic granule marker T-cell intracellular antigen (TIA) (I). Bars: 100 mu.
fneur-10-01283-g0003.jpg
PMC6908508_01_fneur-10-01283-g0003.jpg
F3
The vasculature showed presence of congophilic polarizable birefringent deposits, which stained strongly with anti-Abeta 6F/3D monoclonal antibody, and intramural and perivascular collections of histiocytes and lymphocytes (Figure 3).
MRI brain without and with contrast 1 month after the brain biopsy and treatment with high-dose corticosteroids. The right cerebral T2-weighted fluid-attenuated inversion recovery (T2/FLAIR) hyperintensities have decreased in size, and the midline shift has resolved (A,B). No contrast enhancement is noted (C).
fneur-10-01283-g0004.jpg
PMC6908508_01_fneur-10-01283-g0004.jpg
F4
Steady clinical improvement followed shortly, and significant radiographic improvement was noted on repeat neuroimaging a month later (Figure 4).