| Congenital Generalized Fibrosis of Extraocular Muscles in a Family. |
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Sang Jin Kim, Kwang Soo Kim, Chung Kyu Suh, Young Choon Park |
1Department of Ophthalmology, Keimyung University School of Medicine, Daegu, Korea.
2Department of Neurology, Keimyung University School of Medicine, Daegu, Korea. |
| 한 가족에 발생한 선천성 전외안근 섬유화증 |
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김상진(Sang Jin Kim),김광수(Kwang Soo Kim),서정규(Chung Kyu Suh),박영춘(Young Choon Park) |
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| Abstract |
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Congenital generalized fibrosis of extraocular muscles is a rare congenital and usually familial anomaly involving fibrosis of most or all of the extraocular muscles and was described by Heuck and by Brown. All muscles, including the levator muscle, tend to be involved in this bilateral syndrome, although the muscles of one eye may be more involved than those of the other. Some muscles may be minimally fibrotic while others are maximally involved. The inferior rectus muscles are usually the most prone to maximal involvement, causing the eyes to be drawn downward and offering resistance to elevation. Because of this and the marked ptosis, a chin-up position is usually assumed to fixate objects straight ahead. Horizontal movements of the eye may be limited to a few degree in one or both directions or there may be no horizontal movements and perverted convergence movements occur on attempted upgaze. This condition may occur in an autosomal dominant inheritance and in a sporadic form. The authors experienced a case of congenital generalized fibrosis of extraocular muscles in a family occurred in an autosomal dominant inheritance. |
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