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

H. Koppen, J. C. Vis, D. J. Gooiker, S. Knudsen, B. J. Bouma, J. G.P. Tijssen, B. A.J.M. De Mol, B. J.M. Mulder, M. B. Russell, M. D. Ferrari

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Aim: To assess the lifetime prevalence of migraine in patients with Marfan syndrome (MFS) and to investigate a history of aortic root replacement (AR) as a possible risk factor.Methods: 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.Results: 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.Conclusions: The lifetime prevalence of migraine with aura, but not migraine without aura, is increased in patients with MFS. This association is driven by a history of 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.

Original languageEnglish
Pages (from-to)467-472
Number of pages6
Issue number6
Publication statusPublished - Apr 2012


  • aortic root replacement
  • Marfan syndrome
  • Migraine
  • migraine with aura

ASJC Scopus subject areas

  • Clinical Neurology

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