CADASIL

Discussion:

The important finding is the involvement of both temporal lobes. These are commonly spared in non-CADASIL cases.

Case Discussion

Congenital Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL)

  • hereditary condition due to mutation in the NOTCH-3 Gene on chromosome 19
  • results in small vessel disease and stroke in young patients
  • imaging findings of leukoaraiosis and small infarcts
  • involves external capsules, paramedian frontal lobes and anterior temporal lobes, the latter considered relatively specific for the disease

Other imaging features that may be seen in CADASIL include:

  • micro-hemorrhages, which occur more frequently than in patients without the disease, but occur in many locations (white matter, deep grey nuclei, brainstem, cortex) making them a non-specific finding
  • iron accumulation in putamen and caudate nuclei
Loading...