Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL): MRI Findings

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare, inherited cause of early stroke and dementia.

MRI will show multiple areas of white matter T2-hyperintensity and lacunar infarctions. The highest load of T2-hyperintensity are found in the anterior temporal lobes and in the deep white matter of the frontal and parietal lobes, with relative sparing of the occipital lobes. Anterior temporal pole and external capsule lesions have higher sensitivity and specificity for CADASIL. Digital subtraction angiography is normal in CADASIL.

Differential considerations include:

• Sporadic subcortical arteriosclerotic encephalopathy: Associated with hypertension. CADASIL has more extensive bilateral involvement of anterior temporal and superior frontal white matter, as well as signal intensity reductions in the dentate nuclei, deep cerebellar white matter, crus cerebri, and thalamus
• Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes
• Primary angiitis of the CNS: Unlike CADASIL, digital subtraction angiogram shows lumen irregularities in distal cerebral arteries.
• Hypercoagulable states

References

• Liem MK, Lesnik Oberstein SA, Haan J, van der Neut IL, van den Boom R, Ferrari MD, van Buchem MA, van der Grond J. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy: progression of MR abnormalities in prospective 7-year follow-up study. Radiology. 2008 Dec;249(3):964-71.
• Provenzale J. CADASIL. StatDx. 2008-06-05.