Cerebrovascular disease in Ehlers-Danlos syndrome type IV
| Autor: | J. W. E. Oorthuys, W. I. Schievink, P. Fleury, F. M. Pope, M. Limburg |
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| Rok vydání: | 1990 |
| Předmět: |
Pathology
medicine.medical_specialty Subarachnoid hemorrhage Adolescent Carotid artery dissection Collagen Type III medicine.artery medicine Humans cardiovascular diseases Skin Advanced and Specialized Nursing Internal carotid artery dissection Aorta medicine.diagnostic_test business.industry Middle Aged medicine.disease Cerebral Angiography Pedigree Cerebrovascular Disorders Ehlers–Danlos syndrome Angiography cardiovascular system Ehlers-Danlos Syndrome Female Neurology (clinical) Collagen Cardiology and Cardiovascular Medicine business Cerebral angiography |
| Zdroj: | Stroke. 21(4) |
| ISSN: | 0039-2499 |
| Popis: | We describe two patients with cerebrovascular complications of Ehlers-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 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 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. |
| Databáze: | OpenAIRE |
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