INTRACRANIAL ANEURYSMS IN MARFANS-SYNDROME - AN AUTOPSY STUDY

OBJECTIVE: Marfan's syndrome is a heritable connective tissue disorder that has been associated with intracranial aneurysms. However, the pr evalence of intracranial aneurysms in Marfan's syndrome is unknown and pathological studies of affected vessels have not been reported. We t herefore examined the neuropathological findings in a group of patient s with Marfan's syndrome. METHODS: We identified all patients with Mar fan's syndrome in whom postmortem examination had been performed at th e Mayo Clinic between 1969 and 1993. RESULTS: Autopsy included examina tion of the brain in seven patients with Marfan's syndrome (five men a nd two women with a mean age of 28 yr). Each of two patients had one o r more intracranial aneurysms. The first patient, a 32-year-old man wh o died as a result of aortic dissection, was observed to have an incid ental saccular supraclinoid carotid artery aneurysm (7 mm). Microscopi c examination of the remainder of the cerebral arteries revealed dupli cation and fragmentation of the internal elastic lamina, The second pa tient, a 20-year-old man who died as a result of a subarachnoid hemorr hage, had ruptured saccular supraclinoid carotid artery (3 mm) and ant erior 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 w idespread changes consisting of intimal proliferation, medial degenera tion, and fragmentation of the internal elastic lamina. CONCLUSION: Th ese findings confirm an association between Marfan's syndrome and intr acranial aneurysms. Microscopic involvement of cerebral arteries in Ma rfan's syndrome may be variable, even among those with intracranial an eurysms.