Cerebral autosomal dominant arteriopathy with subcortical infarcts and 
leukoencephalopathy (CADASIL) is caused by mutations in the NOTCH3 gene and is 
clinically characterized by recurrent stroke, cognitive decline, psychiatric 
disturbances and migraine. The prevalence of migraine in CADASIL is slightly 
higher than in the general population, and the proportion of migraine with aura 
is much higher. The pathophysiological mechanism that leads to increased aura 
prevalence in CADASIL is unknown. Possible mechanisms of the excess of migraine 
with aura are an increased susceptibility to cortical spreading depression (CSD) 
or a different expression of CSD. It is also possible that the brainstem 
migraine area is involved in CADASIL. Last, it is possible that the NOTCH3 
mutation acts as a migraine aura susceptibility gene by itself. In this 
narrative review we summarize the literature about migraine in CADASIL, with a 
special focus on what CADASIL might teach us about the pathophysiology of 
migraine.