An autopsied case of Williams syndrome complicated by moyamoya disease
| Autor: | Takeshi Kasajima, Mizuo Kagawa, Mie Kawai, Takashi Higa, Tatsuya Tanikawa, Kazuo Momma, Masato Tanaka, Toshio Nishikawa, Akiko Ando |
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| Rok vydání: | 1993 |
| Předmět: |
medicine.medical_specialty
Adolescent Cerebral arteries Subclavian Artery Autopsy Internal medicine medicine.artery Intellectual Disability Medicine Humans Abnormalities Multiple Moyamoya disease Cerebral Hemorrhage Intracerebral hemorrhage business.industry Anatomy Aortic Valve Stenosis Syndrome Cerebral Arteries medicine.disease Internal elastic lamina Pediatrics Perinatology and Child Health Cardiology Hypercalcemia Circle of Willis Female Williams syndrome Moyamoya Disease business Supravalvular aortic stenosis |
| Zdroj: | Acta paediatrica Japonica : Overseas edition. 35(1) |
| ISSN: | 0374-5600 |
| Popis: | An 18 year old girl with typical clinical features of Williams syndrome suddenly died of intracerebral hemorrhage due to moyamoya disease. Autopsy revealed vascular abnormalities, such as supravalvular aortic stenosis (SAS) and an abnormal complicated cerebrovascular network in the cerebral arteries. The arterial wall of the SAS lesion consisted of thickened medial tissue showing elastic disorganization with prominence of the smooth muscle cells. The narrowed vessels of the circle of Willis showed intimal thickening with an extremely wavy internal elastic lamina and marked thinning of the media. To our knowledge, this is the first report of moyamoya disease associated with Williams syndrome. |
| Databáze: | OpenAIRE |
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