20 - MARFAN’S SYNDROME

Summary

Marfan's syndrome is a connective tissue disorder responsible for an extensive and generalized malformation of organs and systems. An estimate of the risk of developing a cerebrovascular event in Marfan's syndrome is entirely elusive, both in general and for a particular patient. Severity of the vascular malformations differs from patient to patient and, in the worst cases, the chance of a disastrous event is largely related to other than neurological causes. Valvular dysfunction and disturbances of cardiac rhythm can produce embolic strokes basically no different from any other embolic stroke. Intracerebral aneurysms and aneurysmal rupture have for a long time been considered frequent complications of Marfan's syndrome. However, there are currently no prophylactic or curative medical treatments for the crucial Marfan's anomalies. Additional progress in understanding genetics and biochemical defects and in the elucidation of the ultimate mechanisms related to malformations in Marfan's syndrome are expected in the near future.