A Rare Case of Oguchi Disease

  • Siddharam S Janti Tamil Nadu, India
  • D P Shinisha Kelambakkam, Tamil Nadu, India
  • K Stephen Sudhakar Kelambakkam, Tamil Nadu, India
Keywords: Dark adaptation, Mizuo-Nakamura, Oguchi

Abstract

Oguchi disease is a rare autosomal recessive disorder and the patients present with congenital stationary night blindness with slow dark adaptation. Two causative genes have been reported till date. The fundus shows typical golden sheen pattern which disappears when they remain in a darkened environment for few hours. We report a case of young girl who presented with non-progressive night blindness for 10 years and on examination typical fundus finding of Oguchi disease was revealed. The typical golden sheen fundus disappeared after 3 h of dark adaptation. This case is reported for its rarity as only <50 cases have been reported till date.

Author Biographies

Siddharam S Janti, Tamil Nadu, India

1Professor, Department of Ophthalmology, Chettinad Hospital and Research Institute, Kelambakkam,

D P Shinisha, Kelambakkam, Tamil Nadu, India

2Assistant Professor, Department of Ophthalmology, Chettinad Hospital and Research Institute,

K Stephen Sudhakar, Kelambakkam, Tamil Nadu, India

3Head and Professor, Department of Ophthalmology, Chettinad Hospital and Research Institute, 

References

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Published
2020-06-19