Aortic root pathology in Marfan syndrome increases the risk of migraine with aura

Cephalalgia, 04/16/2012

The lifetime prevalence of migraine with aura, but not migraine without aura, is increased in patients with marfan syndrome (MFS). This association is driven by a history of aortic root replacement (AR). The replacement procedure itself is unlikely to be causally associated with migraine as in nearly all subjects, migraine started before the procedure. However this study adds to the evidence that underlying vessel wall pathology may be involved in migraine with aura.


  • In a multicentre study 123 MFS patients (n=52 with AR, n=71 without AR), 82 age– and sex–matched controls and 51 patients with AR but without MFS, were interviewed using a semi–structured headache questionnaire.
  • A multinomial logistic regression model was used to investigate risk factors for migraine with and without aura, adjusting for age and gender.


  • Lifetime migraine prevalence was increased in female MFS patients (51%) compared to healthy female controls (29%), p=0.017.
  • In males lifetime migraine prevalence among MFS patients was only numerically increased.
  • Lifetime prevalence of migraine with aura was increased among MFS patients compared to healthy controls both in males (19% vs. 3%, p=0.048) and females (30% vs. 14%, p=0.049).
  • A history of AR, independently from MFS, gender and age, increased the lifetime prevalence of migraine with aura (OR 3.1 [1.2–8.0]).
  • In all but one patient migraine started before the AR.

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