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Cerebrovascular Complications in Ehlers-Danlos Syndrome Type IV Ann Neurol 38:960-964, North,K.N.,et al, 1995 See this aricle in Pubmed Article Abstract We identified 19 individuals with cerebrovascular complications,which included intracranial aneurysms with secondary hemorrhage,spontaneous carotid cavernous sinus fistula,and cervical artery dissection.The mean age at presentation with these events was 28.3 years(range 17-48 years). Although uncommon,EDS type IV is an important potential cause of stroke in young people.The disorder is readily identifiable clinically and the diagnosis has important implications of acute and long-term management and,potentially,for other family members.Because conventional angiography may exacerbate severe complications,noninvasive procedures such as Doppler and magnetic resonance angiography are the investigations of choice. anticoagulation therapy may result in increased bruising or bleeding and should be used with caution.

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