IS MARFAN-SYNDROME ASSOCIATED WITH SYMPTOMATIC INTRACRANIAL ANEURYSMS

Background and Purpose Marfan syndrome is a heritable disorder of conn ective tissue caused by a deficiency of the glycoprotein fibrillin. In several publications and neurological textbooks, a relationship betwe en Marfan syndrome and intracranial aneurysms has been assumed. Method s The records of 135 patients classified as having Marfan syndrome who visited the Amsterdam Marfan clinic or were admitted to the departmen ts of neurology and neurosurgery and the records of all patients with a subarachnoid hemorrhage or intracranial aneurysm who visited or were admitted to the departments of neurology and neurosurgery between Jan uary 1, 1982, and January 1, 1994, were retrieved. The Literature was reviewed regarding Marfan syndrome and intracranial aneurysms. Results No patient visiting the Marfan clinic had a symptomatic intracranial aneurysm. No patient with Marfan syndrome had been admitted with a rup tured intracranial aneurysm at the departments of neurology or neurosu rgery in this period, while during that period 826 patients with sympt omatic intracranial aneurysms had been admitted. During follow-up of 1 29 of the 135 patients with Marfan syndrome (2850 retrospective patien t observation years and 581 prospective patient observation years), no ne presented a symptomatic intracranial aneurysm. The suggested relati onship between Marfan syndrome and intracranial aneurysms is based mai nly on 10 case reports. However, the diagnosis of MarFan syndrome is d oubtful in several of these reports. Several large studies of patients with Marfan syndrome did not mention a ruptured intracranial aneurysm as a clinical manifestation. Conclusions We conclude that there is in sufficient evidence to presume a relationship between symptomatic intr acranial aneurysms and Marfan syndrome on the basis of currently avail able data.