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  • Author
    Alyscia Severance
  • Co-author

    Shino Magaki

  • Title

    Microhemorrhages and small vessel disease in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)

  • Abstract

    Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common cause of heritable stroke. However, its low overall prevalence has limited neuropathologic characterization, especially in correlation with imaging findings for microhemorrhages. In this pilot study, we examined autopsy brains from a subset of the genetically characterized Newcastle CADASIL cohort for microhemorrhages and small vessel disease. Histologic sections from the frontal cortex, basal ganglia and cerebellum were stained with hematoxylin & eosin to assess for arteriolosclerosis and macro- and microinfarcts. Sections were also stained with Prussian blue to evaluate for microhemorrhages and smooth muscle actin to assess the degree of medial degeneration in the vessel walls. All cases showed one or more infarcts and moderate to severe arteriolosclerosis. Microhemorrhages were seen in all areas, most pronounced in the basal ganglia, and did not correlate with severity of arteriolosclerosis. This pilot study demonstrates that microhemorrhages are common in the brains of CADASIL patients and suggestive of a complex interplay with small vessel disease.

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