Unilateral leukoencephalopathy in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
A 51-year-old female patient with CADASIL due to a pathogenic missense variation in NOTCH3. Brain MRI shows signal abnormalities in the basal ganglia (D) compatible with lacunar infarcts or dilated perivascular spaces, and an extended area of FLAIR hyperintensity in the right frontal and parietal white matter (A) involving the U fibers, appearing hypointense on T1-weighted imaging (C), without gadolinium enhancement (not shown) or additional T2* hypointensities (B). Of note, there was no FLAIR hyperintensity in the temporal poles (E). MR angiography of the intracranial and cervical large arteries (F) was normal.
From: Gollion C, Morel H, Bonneville F. Unilateral Leukoencephalopathy Revealing Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy. Ann Neurol 2022; 91:889.
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