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

H Koppen1, J C Vis, D J Gooiker

  • 1Leiden University Medical Centre, The Netherlands. h.koppen@hagaziekenhuis.nl

Abstract

Insights

Migraine with aura is more common in women with Marfan syndrome (MFS). A history of aortic root replacement (AR) surgery is linked to increased migraine with aura risk in MFS patients.

Area of Science:

  • Neurology
  • Cardiology
  • Genetics

Background:

  • Marfan syndrome (MFS) is a genetic disorder affecting connective tissue, potentially impacting cardiovascular and neurological systems.
  • Migraine is a common neurological disorder with a complex pathophysiology.
  • The relationship between MFS, aortic root replacement (AR), and migraine prevalence is not well-established.

Purpose of the Study:

  • To determine the lifetime prevalence of migraine in patients diagnosed with Marfan syndrome.
  • To explore whether a history of aortic root replacement (AR) surgery is a risk factor for migraine in MFS patients.

Main Methods:

  • A multicenter study involving 123 MFS patients (with and without AR), 82 healthy controls, and 51 patients with AR but without MFS.
  • Participants were interviewed using a semi-structured headache questionnaire.
  • Multinomial logistic regression analysis was employed to identify risk factors for migraine with and without aura, adjusting for age and gender.

Main Results:

  • Lifetime migraine prevalence was significantly higher in female MFS patients (51%) compared to female controls (29%).
  • Lifetime prevalence of migraine with aura was elevated in both male (19%) and female (30%) MFS patients compared to controls.
  • A history of aortic root replacement (AR) independently increased the risk of migraine with aura (OR 3.1), with migraine onset preceding AR in most cases.

Conclusions:

  • Patients with Marfan syndrome exhibit an increased lifetime prevalence of migraine with aura, but not migraine without aura.
  • The association between migraine with aura and MFS appears to be driven by a history of aortic root replacement (AR).
  • The findings suggest that underlying vessel wall pathologies, potentially related to MFS and AR, may contribute to migraine with aura.

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