Which histologic findings are characteristic of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)?

Updated: Oct 09, 2018
  • Author: Reza Behrouz, DO, FACP; Chief Editor: Helmi L Lutsep, MD  more...
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Answer

Pathologically, CADASIL is characterized by degeneration and thickening of the arterial walls (especially cerebral vasculature) with deposition in the media of a nonatheromatous, nonamyloidotic substance that is characteristically periodic acid-Schiff (PAS) positive. Under the electron microscope (EM), this substance appears as a granular osmiophilic material (GOM), pathognomonic for the disease. [29] Skin biopsy typically shows ultrastructural alterations of skin vessels similar to those of brain arteries. [30] Recent reports indicate that these morphologic abnormalities may also involve the kidney. [31]


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