Page 89 - Migraine, the heart and the brain

introduCtion
Migraine is a disabling neurovascular disorder with a lifetime prevalence of 13% in
men and 33% in women (1). Approximately 70% of migraineurs suffer from migraine
without aura and 30% from migraine with aura (1). The aetiology of migraine with
and without aura are considered to be multifactorial due to a combination of genetic
and environmental factors (2,3). Migraine with aura has been associated with cardiac
shunts (4,5), non-shunting congenital heart defects (6,7), congenital abnormalities
of the aorta (8), pulmonary arteriovenous malformation (9) and connective tissue
disorders such as Ehlers–Danlos and Marfan syndrome (10,11). Marfan syndrome
(MFS) is an autosomal-dominant multisystem disorder with speci c cardiovascular,
ocular and skeletal symptoms caused by a mutation in the brillin-1 gene (12). Aortic
root dilatation or aortic dissection are considered major criteria for the diagnosis
of MFS (13) and can be found in approximately half of patients (14). Aortic root
replacement (AR) is a common procedure in severe MFS. In the present study we 5 investigated (i) whether migraine prevalence is increased in patients with MFS, (ii)
whether the effect is stronger in MFS patients who had severe aorta root dilatation requiring AR as a measure of disease severity, (iii) whether migraine prevalence is increased in non-MFS patients who underwent AR.
materials and methods
Patients and procedures
MFS patients were recruited from two sites. MFS diagnosis on both sites was made according to the Ghent nosology (13). Danish recruitment took place in 2000 among members of the Danish Marfan patients’ organisation (Landsforeningen of Marfan’s syndrome) with re-interviewing in 2009 to register the speci c history of AR.
The second site was the cardiology outpatient clinic of the Academic Medical Centre in 2008 (Amsterdam, the Netherlands). All eligible MFS patients were invited to participate. History of aortic root pathology was obtained from the database of the cardiology outpatient clinic. When AR had been performed, the indication was registered, which could either be prophylactic because of progressive aortic root dilatation, or acute following acute type-A aortic dissection. Two types of AR could have been performed and were registered, a Bentall procedure or a valve-sparing AR (David procedure).
Marfan syndrome and migraine
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