Clinical Features of Patients With Cervical Artery Dissection and Fibromuscular Dysplasia
Authors: Bonacina Sonia , Grassi Mario , Zedde Marialuisa , Zini Andrea , Bersano Anna , Gandolfo Carlo , Silvestrelli Giorgio , Baracchini Claudio , Cerrato Paolo , Lodigiani Corrado , Marcheselli Simona , Paciaroni Maurizio , Rasura Maurizia , Cappellari Manuel , Del Sette Massimo , Cavallini Anna , Morotti Andrea , Micieli Giuseppe , Lotti Enrico Maria , DeLodovici Maria Luisa , Gentile Mauro , Magoni Mauro , Azzini Cristiano , Calloni Maria Vittoria , Giorli Elisa , Braga Massimiliano , La Spina Paolo , Melis Fabio , Tassi Rossana , Terruso Valeria , Calabrò Rocco Salvatore , Piras Valeria , Giossi Alessia , Locatelli Martina , Mazzoleni Valentina , Pezzini Debora , Sanguigni Sandro , Zanferrari Carla , Mannino Marina , Colombo Irene , Dallocchio Carlo , Nencini Patrizia , Bignamini Valeria , Adami Alessandro , Magni Eugenio , Bella Rita , Padovani Alessandro , Pezzini Alessandro , Pascarella Rosario , Sessa Maria , Scelzo Emma , Bandettini di Poggio Monica Laura , Boscain Francesca , Naldi Andrea , Caso Valeria , Gamba Massimo , Casetta Ilaria , Forlivesi Stefano , Tomelleri Giampaolo , Schirinzi Elena , Verrengia Elena , Nuzzaco Graziamaria , Beretta Sandro , Musolino Rossella , Imperiale Daniele , Acampa Maurizio , Gasparro Antonio , Melis Maurizio , Fisicaro Francesco , Santilli Ignazio , Corato Manuel , Padroni Marina , Leuci Eleonora , Mazzacane Federico , Gaiani Alessandra , Assenza Federica , Cariddi Lucia Princiotta , Sarti Cristina , Monaco Serena , Puca Emanuele , Ciolli Ludovico 
Published: January 2021

Background and Purpose:
Observational studies have suggested a link between fibromuscular dysplasia and spontaneous cervical artery dissection (sCeAD). However, whether patients with coexistence of the two conditions have distinctive clinical characteristics has not been extensively investigated.

Methods:
In a cohort of consecutive patients with first-ever sCeAD, enrolled in the setting of the multicenter IPSYS CeAD study (Italian Project on Stroke in Young Adults Cervical Artery Dissection) between January 2000 and June 2019, we compared demographic and clinical characteristics, risk factor profile, vascular pathology, and midterm outcome of patients with coexistent cerebrovascular fibromuscular dysplasia (cFMD; cFMD+) with those of patients without cFMD (cFMD–).

Results:
A total of 1283 sCeAD patients (mean age, 47.8±11.4 years; women, 545 [42.5%]) qualified for the analysis, of whom 103 (8.0%) were diagnosed with cFMD+. In multivariable analysis, history of migraine (odds ratio, 1.78 [95% CI, 1.13–2.79]), the presence of intracranial aneurysms (odds ratio, 8.71 [95% CI, 4.06–18.68]), and the occurrence of minor traumas before the event (odds ratio, 0.48 [95% CI, 0.26–0.89]) were associated with cFMD. After a median follow-up of 34.0 months (25th to 75th percentile, 60.0), 39 (3.3%) patients had recurrent sCeAD events. cFMD+ and history of migraine predicted independently the risk of recurrent sCeAD (hazard ratio, 3.40 [95% CI, 1.58–7.31] and 2.07 [95% CI, 1.06–4.03], respectively) in multivariable Cox proportional hazards analysis.

Conclusions:
Risk factor profile of sCeAD patients with cFMD differs from that of patients without cFMD. cFMD and migraine are independent predictors of midterm risk of sCeAD recurrence.