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A Case of Syndrome of Sjögren-Larsson Itsuro Saigo 1 1Department of Ophthalmology, Fukushima Medical College pp.487-491
Published Date 1967/4/15
DOI https://doi.org/10.11477/mf.1410203627
  • Abstract
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A 12-year-old male was diagnosed as syn-drome of Sjögren-Larsson. Besides the classical triad of idiocy, congenital ichthyotic erythe-matosis and spastic palsy, peculiar macular degeneration was present consisting of disse-minated drusen-like yellow patches in the ma-cular area surrounded by grayish zone in both eyes. The visual acuity was 0.5 in each eye. ERG findings were unequivocal. The patient was offspring of consanguinous marriage.

The case is the first of its kind to be repor-ted in Japan. Studies of literature indicate that the observed fundus finding is a frequent com ponent of the syndrome.


Copyright © 1967, 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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