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Typical neurovascular complications of Ehlers-Danlos syndrome are carotid- cavernous fistulae,intracranial aneurysms,and cervical artery dissections. Arterial dissections and intracranial aneurysms cause the majority of neurovascular symptoms in Marfan's syndrome.Neurovascular disease is uncommon in osteogenesis imperfecta,although carotid-cavernous fistulae and vertebral artery dissections have been reported.Neurovascular disease in pseudoxanthoma elasticum is characterized by intracranial aneurysms and cerebral ischemia caused by premature arterial occlusive disease. Intracranial occlusive arterial disease is the most common neurovascular manifestation of neurofibromatosis,followed by cervical arteriovenous fistulae and aneurysms and intracranial aneurysms.Intracranial aneurysms are the hallmark of polycystic kidney disease.Recognition of an underlying generalized connective tissue disorder may be of considerable importance, although marked phenotypic heterogeneity often complicates the diagnosis of these disorders.Conversely,the association of certain neurovascular anomalies with generalized connective tissue disorders and recognition of their basic molecular defect may offer clues to the etiology and pathogenesis of these neurovascular diseases in general. |
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aneurysm aneurysm,intracranial arterial dissection arterial dissection,carotid arterial dissection,ruptured arterial dissection,vertebral cerebral ischemia cerebrovascular accident cerebrovascular accident,familial occurrence cerebrovascular accident,non atherosclerotic cause of cerebrovascular disease collagen vascular disease Ehlers-Danlos syndrome epidemiology of neurology familial fistula,arterio-venous fistula,arterio-venous,carotid-cavernous genetic neurologic disorders Marfan syndrome osteogenesis imperfecta pseudoxanthoma elasticum review article skin,lesions in neurologic disorders subarachnoid hemorrhage
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