Familial hemiplegic migraine is an autosomal dominant disorder of unkn
own pathogenesis in which the migrainous attacks are marked by the occ
urrence of a transient hemiplegia during the aura, While investigating
CADASIL, mapped previously to chromosome 19, we observed that some pa
tients had recurrent attacks of migraine with aura. Although the clini
cal and neuroimaging features of familial hemiplegic migraine differ m
arkedly from CADASIL, we hypothesized that the same gene could be invo
lved in the pathogenesis of both conditions. We chose two large pedigr
ees for linkage analysis of familial hemiplegic migraine. A maximum lo
d score > 8 was found with two markers that are also strongly linked t
o CADASIL. Multilocus linkage analysis suggested that the loci respons
ible for the two diseases reside within an interval of about 30 cM on
chromosome 19.