However, it has recently been reported that a subset of patients with NOTCH3 gene mutations show atypical clinical symptoms of CADASIL, such as absence of temporal pole lesions (Ueda et al., 2015), elderly onset (Watanabe et al., 2012), and cerebellar atrophy (Sari et al., 2019). This evidence concerns the gene NOTCH3 and CADASIL.