Cerebrovascuiar Disease in Ehiers-Danlos Syndrome Type IV
نویسندگان
چکیده
We describe two patients with cerebrovascuiar complications of Ehiers-Danlos syndrome type IV. A 16-year-old girl with spontaneous internal carotid artery dissection and a 46-year-old woman with aneuiysmal subarachnoid hemorrhage and multiple aortic dissections were both deficient in collagen type III, analyzed in cultured skin fibroblasts. To our knowledge, spontaneous carotid artery dissection associated with collagen type III deficiency has not been reported previously. Early clinical recognition of this syndrome is of great importance in view of the hazards of angjography and surgery. Collagen type III deficiency plays a role in the pathogenesis of intracraniai saccular aneurysms and may also be involved in the pathogenesis of carotid cavernous fistulas and dissections of the cervical arteries. (Stroke 1990^1:626-632)
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