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A case of Kearns-Sayre syndrome Yoko Shimizu 1 , Machiko Shimakawa 1 , Yoka Sannomiya 1 , Kazuko Kameyama 1 , Sadao Hori 1 1Dept of Ophthalmol,Tokyo Women's Med Univ pp.1529-1532
Published Date 2004/8/15
DOI https://doi.org/10.11477/mf.1410100701
  • Abstract
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 A 40-year-old female presented with blepharoptosis and external ophthalmoplegia. She had a history of diabetes mellitus,impaired cardiac conduction,and sensorineural hearing difficulty. She had had an attack of complete atrioventicular block at the age of 25 years,necessitating implantation of artificial pacemaker. Biopsy led to the diagnosis of mitochondrial encephalomyopathy. Her corrected visual acuity was 0.9 in either eye. Both eyes showed marked ophthalmoplegia and blepharoptosis. Both fundus showed findings of retinitis pigmentosa without pigment granules. Electroretinogram showed extinguished pattern. Dynamic perimetry showed normal findings except enlarged blind spot. We diagnosed her with Kearns-Sayre syndrome manifesting retinitis pigmentosa,blepharoptosis and impaired cardiac conduction and characterized by late onset at 25 years.


Copyright © 2004, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1308 印刷版ISSN 0370-5579 医学書院

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