[Clinical features of congenital stationary night blindness]

Zhonghua Yi Xue Za Zhi. 2012 Oct 23;92(39):2756-9.
[Article in Chinese]


Objective: To explore the clinical features of Chinese patients with congenital stationary night blindness (CSNB).

Methods: An observational serial case study was conducted for 22 patients diagnosed as CSNB at Department of Ophthalmology, Peking Union Medical College Hospital from December 2003 through December 2011. Sixteen patients (72.7%) were males and 6 patients (27.3%) were females. Their age range was 4-73 years old. All of them underwent detailed ocular examinations including electroretinogram (ERG). Thirteen patients underwent optical coherence tomography (OCT) while 9 had visual field (VF) testing. Medical and family history was recorded.

Results: Seven patients had a positive family history. Thirteen patients (59.1%) complained of a poor night vision. Best corrected visual acuity was < 0.8 in both eyes for 16 patients (72.7%). There were nystagmus (n = 4) and strabismus (n = 4). Except for 1 complicated with background diabetic retinopathy, all other patients showed normal or myopic fundi. Nine patients revealed peripheral visual field defects. Twenty-one (95.5%) patients had Schubert-Bornstein type negative ERG while another one had Riggs type ERG. Among 21 Schubert-Bornstein type patients, 13 (61.9%) patients showed complete type CSNB and 8 (38.1%) were of incomplete CSNB. Before referral to our tertiary hospital, only 2 patients were suspected as CSNB. The common primary diagnoses included pathogenic myopia, amblyopia and retinitis pigmentosa.

Conclusion: CSNB is frequently misdiagnosed in China. Poor visual acuity is one of the major complaints. And around 50% CSNB patients experience night vision problem. Complete CSNB is more common than incomplete type in China.

Publication types

  • English Abstract
  • Observational Study
  • Research Support, U.S. Gov't, Non-P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Child, Preschool
  • Electroretinography
  • Eye Diseases, Hereditary / diagnosis*
  • Female
  • Genetic Diseases, X-Linked / diagnosis*
  • Humans
  • Male
  • Middle Aged
  • Myopia / diagnosis*
  • Night Blindness / diagnosis*
  • Nystagmus, Pathologic / diagnosis
  • Visual Acuity
  • Young Adult

Supplementary concepts

  • Night blindness, congenital stationary