Published Online
on November 18, 2004

A more recent version of this article appeared on January 1, 2005

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Submitted on April 22, 2004
Revised on August 10, 2004
Accepted on October 6, 2004

Protease Inhibitors in Spontaneous Cervical Artery Dissections

Carsten Konrad MD^*; C. Langer PhD; G. A. Müller MD; K. Berger MD, MPH; R. Dziewas MD; F. Stögbauer MD; D. G. Nabavi MD; R. Junker MD; E. B. Ringelstein MD; and G. Kuhlenbäumer MD

From the Department of Neurology (C.K., G.A.M., R.D., F.S., D.G.N., E.B.R., G.K.); the Department of Psychiatry (C.K.); the Institute of Clinical Chemistry and Laboratory Medicine (C.L., R.J.); the Institute of Arteriosclerosis Research (C.L., R.J., G.K.); and the Department of Epidemiology (K.B.), University of Münster, Germany.

^* To whom correspondence should be addressed. E-mail: konradc{at}uni-muenster.de.

Background and Purpose--Observations in patients with arterial aneurysms, fibromuscular dysplasia, and spontaneous cervical artery dissection (sCAD) indicate that protease inhibitor deficiency might boost the enzymatic destruction of arterial tissue and increase the risk of these arterial wall diseases. Here we present the first large investigation of the protease inhibitor hypothesis in patients with sCAD.

Methods--Eighty patients with sCAD were compared with 80 age- and sex-matched healthy individuals. ₁-antitrypsin (₁-AT) and ₂-macroglobulin (₂-MG) levels, and ₁-AT genotypes were assessed and compared between groups.

Results--₁-AT and ₂-MG levels as well as ₁-AT genotypes did not differ significantly between patients and controls. The frequency of Z alleles in the patient group was higher than in the control group and than in other cohorts from Europe; however, the difference remained nonsignificant. All patients with Z alleles had internal carotid artery dissections.

Conclusions--Overall, this data does not support the hypothesis that protease inhibitor levels or ₁-AT genotypes play an important role in the etiology of sCAD. The present data does not exclude that the Pi-Z allele might have an influence on subgroups of sCAD, such as internal carotid artery dissections.

Key words: alpha 1-antitryptsin • alpha-macroglobulins • dissection • protease inhibitors • risk factors

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