The authors report on an Italian family with eight affected members who sho
w autosomal dominant migraine with prolonged visual, sensory, motor, and ap
hasic aura. These symptoms are associated with white matter abnormalities o
n brain MRI. All living affected members carry a Notch3 mutation (Arg153Cys
) previously reported in cerebral autosomal dominant arteriopathy with subc
ortical infarcts and leukoencephalopathy (CADASIL). White matter abnormalit
ies occur in a variable percentage of the general migraine population; CADA
SIL should be suspected in migraineurs with prolonged atypical aura and whi
te matter abnormalities.