What is the role of MRI in the diagnosis 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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Hyperintensities on T2-weighted imaging or FLAIR are seen in the periventricular and deep white matter. [21] These white matter hyperintensities on MRI can be visualized in those aged 21 years and older. [22] MRI lesion volume correlates with the level of disability associated with CADASIL. [23] A characteristic finding on the MRI in patients with CADASIL is the presence of isolated T2 hyperintensities involving the temporal poles (see image below), a feature that can differentiate the condition from chronic microvascular ischemia due to hypertension. This finding is associated with a sensitivity and specificity of 95% and 80% respectively. [24]

FLAIR MRI of the brain showing hyperintensities in FLAIR MRI of the brain showing hyperintensities involving the temporal poles in a patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). (Reprinted with permission from Mayo Clin Proc, Meschia, 2005.)

Another relatively conspicuous MRI feature is involvement of the external capsules (see image below), which has a sensitivity of 93% and a specificity of 45%. [25] In association with age, volume of lacunar lesions is a strong and independent MRI predictor of cognitive and motor disability in CADASIL. [26] MRI studies in patients with CADASIL have also shown an age-related increased risk of intracerebral microbleeds. [27]

FLAIR MRI of the brain showing hyperintensities in FLAIR MRI of the brain showing hyperintensities involving bilateral external capsules in a patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). (Reprinted with permission from Mayo Clin Proc, Meschia, 2005.)

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