Intracranial aneurysms in Marfan's syndrome: An autopsy study

OBJECTIVE: Marfan's syndrome is a heritable connective tissue disorder that has been associated with intracranial aneurysms. However, the prevalence of intracranial aneurysms in Marfan's syndrome is unknown and pathological studies of affected vessels have not been reported. We therefore examined the neuropathological findings in a group of patients with Marfan's syndrome. METHODS: We identified all patients with Marfan's syndrome in whom postmortem examination had been performed at the Mayo Clinic between 1969 and 1993. RESULTS: Autopsy included examination of the brain in seven patients with Marfan's syndrome (five men and two women with a mean age of 28 yr). Each of two patients had one or more intracranial aneurysms. The first patient, a 32-year-old man who died as a result of aortic dissection, was observed to have an incidental saccular supraclinoid carotid artery aneurysm (7 mm). Microscopic examination of the remainder of the cerebral arteries revealed duplication and fragmentation of the internal elastic lamina, The second patient, a 20-year-old man who died as a result of a subarachnoid hemorrhage, had ruptured saccular supraclinoid carotid artery (3 mm) and anterior cerebral artery (20 mm) aneurysms as well as unruptured fusiform middle cerebral artery (18 mm) and posterior cerebral artery (13 mm) aneurysms. Microscopic examination of the cerebral arteries revealed widespread changes consisting of intimal proliferation, medial degeneration, and fragmentation of the internal elastic lamina. CONCLUSION: These findings confirm an association between Marfan's syndrome and intracranial aneurysms. Microscopic involvement of cerebral arteries in Marfan's syndrome may be variable, even among those with intracranial aneurysms.