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. 2014 Oct 16;24(5):525–544. doi: 10.1111/bpa.12181

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© 2014 The Authors. Brain Pathology published by John Wiley & Sons Ltd on behalf of International Society of Neuropathology.

This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

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Compared with a control person's cerebral WM arteriole (A), the wall of a CADASIL patient's arteriole (B) is markedly thickened and fibrotic, and its rigidity renders the arteriole exceptionally circular (C). Tunica media is slightly basophilic in hematoxylin and eosin (H&E) (B) and prominently positive in periodic acid Schiff (PAS) staining (C). In advanced disease, the fibrosis has almost completely obliterated the lumen (D). Similarly, as in dermal arteries, NOTCH3 extracellular domain (N3ECD) immunopositivity accumulates in the tunica media of the thickened WM arterioles (E). The irregularly decreased immunoreactivity for α‐smooth muscle actin (α‐SMA) in the wall of a WM arteriole reflects the degeneration of VSMCs (G). The thickened adventitia harbors extracellular matrix proteins, here shown by collagen type I immunohistochemistry (F). vG = van Gieson.