ALPHA-1-ANTITRYPSIN DEFICIENCY IN SPONTANEOUS INTRACRANIAL ARTERIAL DISSECTIONS

alpha(1)-Antitrypsin deficiency has been associated with a variety of vascular disorders including arterial aneurysms, spontaneous extracran ial arterial dissections, and arterial fibromuscular dysplasia. We det ermined the distribution of alpha(1)-antitrypsin phenotypes in patient s with intracranial arterial dissections, a rare cause of subarachnoid hemorrhage. The study population consisted of 4 consecutive patients with subarachnoid hemorrhage due to spontaneous intracranial arterial dissections. The vertebral artery was involved in 3 patients and the p osterior inferior cerebellar artery in 1 patient. Three of these 4 pat ients were found to have a heterozygous alpha(1)-antitrypsin deficienc y (PiMZ or PiMS phenotypes). These data support previous studies sugge sting that patients with alpha(1)-antitrypsin deficiency may be at an increased risk of developing spontaneous arterial dissections.