Migraine is often the first manifestation of CADASIL, the commonest monogenic cause of stroke. We aimed to determine the pattern of migraine, and its relationship with other features of CADASIL. Of 314 patients seen in a national referral clinic, 71.6% reported migraines. Migraine was the first feature of CADASIL in 67.6% of symptomatic individuals. Females (81.7%) were more likely to develop migraine than males (66.9%), and suffered migraine at an earlier age (27.0±12.7 vs 32.3±13.2 years, p=0.004).
89.8% of migraneurs experienced aura. Where aura occurred it was visual in 69.7% sensory 58.7%, dysphasic 30.3%, confusional 17.9%, motor 15.9% and sensorimotor 9.95%.
A self-limiting encephalopathy occurred in 33 patients (10.8%); 72.7% of these evolved from a migraine episode. Patients with confusional migraines were more likely to develop encephalopathy (OR=3.93, 95% CI=1.51–9.73, p=0.0024).
Non-migraneurs had a higher cumulative risk of stroke than those migraneurs (HR 2.13, 95% CI 1.53–2.96, p=6.8×10−6).
In conclusion, the spectrum of migraine in CADASIL differs from that in the general population. Acute encephalopathy is a common feature of CADASIL and usually evolves from a migraine episode. Non-migraneurs have a higher risk of developing stroke, however the reasons for this are not well understood.