FAMILIAL INTRACRANIAL ANEURYSMS - AN AUTOPSY STUDY

OBJECTIVE: Familial intracranial aneurysms are more common than has be en appreciated, but systematic autopsy studies of affected individuals have not been reported. We reviewed the autopsy findings of a group o f patients with familial aneurysms to elucidate the nature of the puta tive underlying arteriopathy. METHODS: Using a computerized diagnostic index, we identified all patients with intracranial aneurysms in whom postmortem examination had been performed at the Mayo Clinic between January 1, 1992, and December 31, 1994. The medical records, radiograp hic studies, and autopsy findings of these patients were reviewed. RES ULTS: Among the 28 patients with intracranial aneurysms, 3 (11%) had o ne or more first-degree relatives with documented intracranial aneurys ms. The mean age of the three patients (two women and one man) was 54 years. Microscopic examination of the vascular system revealed medial changes, consisting of degeneration of elastic fibers and increased gr ound substance, in the systemic arteries of 2 of the 3 patients with f amilial aneurysms but in none of the 25 patients with sporadic aneurys ms. These nonspecific medial changes involved both common and extracra nial internal carotid arteries in one patient and the entire aorta as well as intracranial and common carotid arteries in the other patient. CONCLUSION: These observations suggest that an underlying arteriopath y in patients with familial intracranial aneurysms involves the tunica media and commonly may affect systemic (extracranial) arteries.