PMC3917527_01
Brain magnetic resonance images before (A-C) and after (D-F) treatment. (A) T2-weighted FLAIR imaging demonstrates bilateral, diffuse hyperintense lesions in the white matter with sparing of the cortical U fibers. T1-weighted images following intravenous gadolinium administration in the (B) axial and (C) coronal planes show bilateral, symmetrical, linear contrast enhancement in a radial distribution throughout the white matter of the cortex and brainstem (arrows). (D) Following treatment with steroids and cyclophosphamide, T2-weighted FLAIR imaging demonstrates a dramatic improvement of the hyperintense abnormalities and almost complete resolution of gadolinium enhancement on the T1-weighted images in the (E) axial and (F) coronal planes.
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F1
T2-weighted fluid attenuated inversion recovery (FLAIR) brain MRI revealed diffuse, symmetrical, increased intensity throughout the white matter in his centrum semiovale and corona radiata (Figure 1A) concurrent with bilateral linear enhancement in a radial distribution on the gadolinium-contrasted study (Figure 1B,C). Follow-up brain and spine MRI scans showed improvement in the white matter hyperintensities on FLAIR imaging (Figure 1D) as well as on the gadolinium-enhanced images (Figure 1E,F).
Pathology of the brain biopsy. (A) Hematoxylin and eosin staining shows the development of a non-caseating granuloma with giant cells (arrow). (B) Staining with anti-CD68 antibodies demonstrates the presence of macrophages within a developing granuloma with giant cells (arrow).
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F2
A right frontal brain biopsy showed numerous perivascular non-caseating granulomas consistent with the granulomatous pattern of PACNS (Figure 2).