It is worthy of note that ischemic strokes typically occur in the absence of traditional cardiovascular risk factors.

Autosomal dominant mutations in the NOTCH3 gene (on the long arm of chromosome 19) cause an abnormal accumulation of Notch 3 protein at the cytoplasmic membrane of vascular smooth muscle cells both in cerebral and extracerebral vessels,[8] seen as granular osmiophilic deposits on electron microscopy.

Depending on the nature and position of each mutation, a consensus significant loss of beta sheet structure of the Notch3 protein has been predicted using in silico analysis.

These lesions are concentrated around the basal ganglia, peri-ventricular white matter and the pons and are similar to those seen in Binswanger disease.

[15] Short-term use of atorvastatin, a statin-type cholesterol-lowering medication, has not been shown to be beneficial in CADASIL patients' cerebral hemodynamic parameters,[17] although treatment of comorbidities such as high cholesterol is recommended.

[18] In this regard, the advent of the "Ditans" such as Lasmiditan, lacking vasoconstrictive effect and the "Gepants" such as Ubrogepant and Rimegepant, are attractive alternatives, albeit not yet field-tested in this condition.

[21] In one small study, around 1/3 of patients with CADASIL were found to have cerebral microhemorrhages (tiny areas of old blood) on MRI.

[16] L-arginine, a naturally occurring amino acid, has been proposed as a potential therapy for CADASIL,[22] but as of 2017 there are no clinical studies supporting its use.

[24] Ruskin reported in his diaries having visual disturbances consistent with the disease and it has also been suggested that it might have been a factor in causing him to describe James Whistler's Nocturne in Black and Gold – The Falling Rocket as "ask[ing] two hundred guineas for throwing a pot of paint in the public's face".