case_images: PMC9520523_01
case_images: Magnetic resonance imaging (MRI) scan of the brain on admission. T1-weighted gadolinium-enhanced brain MRI revealed enhancement and thickening of dura mater predominantly in the posterior fossa (A,B). Marked reduction of dura thickening and enhancement was evident at the 6-month follow-up (C,D).
case_images: fneur-13-986694-g0001.jpg
case_images: PMC9520523_01_fneur-13-986694-g0001.jpg
case_images: F1
case_images: Gadolinium-enhanced brain magnetic resonance imaging (MRI) revealed enhanced and thickened dura mater, predominantly in the posterior fossa (Figure 1).
case_images: PMC9520523_02
case_images: Physical examination and MRI scan of the brain on admission. Rightward tongue deviation upon protrusion and atrophy of right lingualis were observed on examination (A). T1-weighted gadolinium-enhanced brain MRI showed pachymeningeal enhancement and thickening predominantly in the posterior fossa and bilateral posterior cerebral hemispheres (B-D).
case_images: fneur-13-986694-g0002.jpg
case_images: PMC9520523_02_fneur-13-986694-g0002.jpg
case_images: F2
case_images: The only notable neurological examination result was a right hypoglossal nerve palsy (Figure 2A). The gadolinium-enhanced brain MRI revealed pachymeningeal enhancement and thickening, predominantly in the posterior fossa and bilateral posterior cerebral hemispheres (Figures 2B-D).
case_images: PMC9520523_03
case_images: Magnetic resonance imaging scan of the thoracic spine on admission and pathology findings. Sagittal T2-weighted image (A) showed a low-signal intensity lesion in the anterior and posterior epidural spaces and high enhancement after gadolinium enhancement (B) at the thoracic canal between T7 and T11 vertebral body levels. Axial images showed that the lesion was located in the epidural space and extended to the neural foramen to the right. A fat-suppressed T1-weighted image with gadolinium enhancement demonstrated high enhancement of the mass (C). Pathology findings: Hematoxylin and eosin stain of the epidural mass showed intense lymphoplasmacytic inflammatory cell infiltrate with fibrosis. Plasma cells and lymphocytes were also detected within the wall of a vessel as well as in the perivascular area (D). IgG4 immunohistochemistry showed prominent IgG4+ cells within the inflammatory infiltration. Nearly more than half of the plasma cells exhibited IgG4 reactivity (E).
case_images: fneur-13-986694-g0003.jpg
case_images: PMC9520523_03_fneur-13-986694-g0003.jpg
case_images: F3
case_images: A spinal cord MRI revealed a ribbon-like thickening of the dura mater between vertebral levels T7 and T11 (which was moderately enhanced by gadolinium administration) and a compressed and flattened focal spinal cord (Figures 3A-C). A histopathology analysis showed lymphocyte infiltration, high IgG4+ cell infiltration, and a storiform pattern of fibrosis without granulomatous changes (Figures 3D,E).