Documenta Ophthalmologica

, Volume 116, Issue 1, pp 41–47 | Cite as

Gradient of deficit in cone responses in the incomplete form of congenital stationary night blindness revealed by multifocal electroretinography

  • Francois Tremblay
  • Joan Parkinson
Case Report


Multifocal electroretinograms were recorded in one case of incomplete form of congenital stationary night blindness. First order kernel revealed reduced cone macular P1 responses with normal implicit time (22.7 nV, 33.3 ms; normal 43.3 ± 8.2 nV, 32.7 ± 0.6 ms) whereas more peripheral responses exhibited low responses of extremely delayed implicit time (5.1 nV, 47.5 ms; normal 7.4 ± 2.1 nV, 32.3 ± 0.8 ms). Responses from the first slice of the second kernel were present in the macular area but absent from the more peripheral areas. In comparison, mfERGs in the complete form of CSNB showed normal amplitude but slightly delayed responses at all eccentricities and normal second kernel responses. Results are discussed in terms of the dichotomy in synaptic transmission between macular and peripheral cones.


Multifocal electroretinogram Incomplete congenital stationary night blindness Cone Retinal diseases 


  1. 1.
    Miyake Y. (2006) Electrodiagnosis of retinal diseases. Springer, TokyoGoogle Scholar
  2. 2.
    Tremblay F, Laroche RG, De Becker I (1995) The electroretinographic diagnosis of the incomplete form of congenital stationary night blindness. Vision Res 35(16):2383–2393PubMedCrossRefGoogle Scholar
  3. 3.
    Miyake Y, Yagasaki K, Horiguchi M, Kawase Y, Kanda T (1986) Congenital stationary night blindness with negative electroretinogram. A new classification. Arch Ophthalmol 104(7):1013–1020PubMedGoogle Scholar
  4. 4.
    Bradshaw K, Allen L, Trump D, Hardcastle A, George N, Moore A (2004) A comparison of ERG abnormalities in XLRS and XLCSNB. Doc Ophthalmol 108(2):135–135PubMedCrossRefGoogle Scholar
  5. 5.
    Cibis GW, Fitzgerald KM (2001) The negative ERG is not synonymous with night blindness. Trans Am Ophthalmol Soc 99:171–175 discussion 175–176Google Scholar
  6. 6.
    Miyake Y, Horiguchi M, Terasaki H, Kondo M (1994) Scotopic threshold response in complete and incomplete types of congenital stationary night blindness. Invest Ophthalmol Vis Sci 35(10):3770–3775PubMedGoogle Scholar
  7. 7.
    Terasaki H, Miyake Y, Nomura R, Horiguchi M, Suzuki S, Kondo M (1999) Blue-on-yellow perimetry in the complete type of congenital stationary night blindness. Invest Ophthalmol Vis Sci 40(11):2761–2764PubMedGoogle Scholar
  8. 8.
    Miyake Y, Yagasaki K, Horiguchi M, Kawase Y (1987) On- and off-responses in photopic electroretinogram in complete and incomplete types of congenital stationary night blindness. Jpn J Ophthalmol 31(1):81–87PubMedGoogle Scholar
  9. 9.
    Bech-Hansen NT, Naylor MJ, Maybaum TA, Pearce WG, Koop B, Fishman GA et al (1998) Loss-of-function mutations in a calcium-channel alpha1-subunit gene in Xp11.23 cause incomplete X-linked congenital stationary night blindness. Nat Genet 19(3):264–267PubMedCrossRefGoogle Scholar
  10. 10.
    Strom TM, Nyakatura G, Apfelstedt-Sylla E, Hellebrand H, Lorenz B, Weber BH et al (1998) An L-type calcium-channel gene mutated in incomplete X-linked congenital stationary night blindness. Nat Genet 19(3):260–263PubMedCrossRefGoogle Scholar
  11. 11.
    Morgans CW (2001) Localization of the alpha(1F) calcium channel subunit in the rat retina. Invest Ophthalmol Vis Sci 42(10):2414–2418PubMedGoogle Scholar
  12. 12.
    Chang B, Heckenlively JR, Bayley PR, Brecha NC, Davisson MT, Hawes NL et al (2006) The nob2 mouse, a null mutation in Cacna1f: anatomical and functional abnormalities in the outer retina and their consequences on ganglion cell visual responses. Vis Neurosci 23(1):11–24PubMedGoogle Scholar
  13. 13.
    Lachapelle P, Little JM, Polomeno RC (1983) The photopic electroretinogram in congenital stationary night blindness with myopia. Invest Ophthalmol Vis Sci 24(4):442–450PubMedGoogle Scholar
  14. 14.
    Tremblay F, Parkinson JE (2003) Alteration of electroretinographic recordings when performed under sedation or halogenate anesthesia in a pediatric population. Doc Ophthalmol 107(3):271–279PubMedCrossRefGoogle Scholar
  15. 15.
    Schuster A, Pusch CM, Gamer D, Apfelstedt-Sylla E, Zrenner E, Kurtenbach A (2005) Multifocal oscillatory potentials in CSNB1 and CSNB2 type congenital stationary night blindness. Int J Mol Med 15(1):159–167PubMedGoogle Scholar
  16. 16.
    Mansergh F, Orton NC, Vessey JP, Lalonde MR, Stell WK, Tremblay F et al. (2005) Mutation of the calcium channel gene Cacna1f disrupts calcium signaling, synaptic transmission and cellular organization in mouse retina. Hum Mol Genet 14(20):3035–3046PubMedCrossRefGoogle Scholar
  17. 17.
    Hood DC, Frishman LJ, Saszik S, Viswanathan S (2002) Retinal origins of the primate multifocal ERG: implications for the human response. Invest Ophthalmol Vis Sci 43(5):1673–1685PubMedGoogle Scholar
  18. 18.
    Kondo M, Miyake Y, Kondo N, Tanikawa A, Suzuki S, Horiguchi M et al (2001) Multifocal ERG findings in complete type congenital stationary night blindness. Invest Ophthalmol Vis Sci 42(6):1342–1348PubMedGoogle Scholar
  19. 19.
    Dryja TP, McGee TL, Berson EL, Fishman GA, Sandberg MA, Alexander KR et al (2005) Night blindness and abnormal cone electroretinogram ON responses in patients with mutations in the GRM6 gene encoding mGluR6. Proc Natl Acad Sci USA 102(13):4884–4889PubMedCrossRefGoogle Scholar
  20. 20.
    Leifert D, Todorova MG, Prunte C, Palmowski-Wolfe AM (2005) LED-generated multifocal ERG on- and off-responses in complete congenital stationary night blindness––a case report. Doc Ophthalmol 111(1):1–6PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2007

Authors and Affiliations

  1. 1.Department of Ophthalmology & Visual Sciences, IWK Health CenterDalhousie UniversityHalifaxCanada
  2. 2.Clinical Vision Science Program, IWK Health CenterDalhousie UniversityHalifaxCanada

Personalised recommendations