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Cerebrovascular Disease in Ehlers-Danlos Syndrome Type IV
Stroke 21:626-632, Schievink,W.I.,et al, 1990
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Article Abstract
We describe two patients with cerebrovascular complications of Ehler- Danlos syndrome type IV.A 16-year-old girl with spontaneous internal carotid artery dissection and a 46-year-old woman with aneurysmal 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 association with collagen type III deficiency had not been reported previously.Early clinical recognition of this syndrome is of great importance in view of the hazards of angiography and surgery.Collagen type III deficiency plays a role in the pathogenesis of intracranial saccular aneurysms and may also be involved in the pathogenesis of carotid cavernous fistulas and dissections of the cervical arteries.
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arterial dissection
arterial dissection,aorta
arterial dissection,carotid
arterial dissection,ruptured
cerebrovascular accident
cerebrovascular disease
cultured skin fibroblasts
Ehlers-Danlos syndrome
genetic neurologic disorders
mitral valve prolapse
subarachnoid hemorrhage
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