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Wouter I. Schievink, David G. Piepgras, Franklin Earnest IV, and Hymie Gordon

previous cavernous sinus thrombosis. Ehlers-Danlos syndrome is a group of hereditary connective-tissue disorders first described in 1668 50 and is currently divided into nine types. 7 Ehlers-Danlos syndrome Type IV, the so-called “vascular type,” was first described as a distinct clinical entity in 1967. 3 The basic molecular defect of this condition is an abnormality of Type III collagen. 39, 41 This type of collagen is one of the three major fibrillar collagens and normally constitutes approximately 40% of blood vessel walls. 32 The gene for the alpha-1 chain of

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Wouter I. Schievink, David G. Piepgras, and Douglas A. Nichols

by spontaneous dissection. However, rupture of such extracranial aneurysms rarely if ever occurs. 36 Our patient has a previously undescribed constellation of findings including spontaneous carotid—jugular fistula, carotid dissection, multiple intracranial arachnoid cysts, hemifacial atrophy, mitral valve prolapse, easy bruisibility, and abnormal scarring, suggesting the presence of a generalized connective tissue disorder. Although this complex of findings appears unique there is some overlap with previously described syndromes. Ehlers—Danlos syndrome type IV

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Wouter I. Schievink, Bahram Mokri, and David G. Piepgras

population was due to the high incidence in the female patients, who harbored intracranial aneurysms in 8.8%; only 1.4% of the male patients were found to have an intracranial aneurysm, a figure similar to that reported by Atkinson, et al. Evidence for a Common Underlying Vasculopathy Various disorders affecting the extracellular matrix have been associated with both intracranial aneurysms and cervical artery dissections, including Ehlers-Danlos syndrome type IV, 7, 37, 39 Marfan's syndrome, 3, 6, 9, 13, 17, 30, 33, 41, 42, 47 and fibromuscular dysplasia. 12, 22