A CADASIL-Like Case with a Novel Noncysteine Mutation of the NOTCH3 Gene and Granular Deposits in the Renal Arterioles

Figure 3

The light microscopic findings and electron microscopic (EM) findings suggestive of MPGN (a–d) or of CADASIL (e, f) in the renal biopsy. (a) A glomerulus showed severe mesangial expansion with mesangiolysis and segmental endocapillary proliferation. Tuft adhesion (arrow) was observed. Periodic acid-silver methenamine (PAM) stain, ×200. (b) Double contours of the glomerular basement membrane with mesangial interposition were also observed. PAM stain, ×400. (c) The glomerulus showed prominent deposition of C3 along the capillary loop. Immunofluorescence stain, ×200. (d) Dilatation of the subendothelial spaces with mesangial interposition was observed. Electron-dense deposits were also observed in subendothelial space (arrow). EM, ×2,500. (e) Severe hyaline changes of the arterioles (arrow) were observed. PAM staining, ×100. (f) Granular deposits were found in the thickened endothelial basement membrane or the basement membrane-like material of arterioles, similar to the findings of GOM. EM, ×2,500 (left panel) and ×12,000 (right panel). Abbreviations: EC: vascular endothelial cell; VSMC: vascular smooth muscle cell; L: vascular lumen; GOM: granular osmiophilic material.

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