Documenta Ophthalmologica

, Volume 114, Issue 1, pp 27–36 | Cite as

Electroretinographic findings in the Standard Wire Haired Dachshund with inherited early onset cone–rod dystrophy

  • Ernst O. Ropstad
  • Ellen Bjerkås
  • Kristina Narfström
Original Paper



To describe electroretinographic (ERG) findings in a strain of Standard Wire Haired Dachshund (SWHD)-derived dogs at the ages of approximately 5, 8 and 52 weeks selected for inherited early onset cone–rod dystrophy.


Nineteen affected and 13 age-matched control SWHDs were included in the study. All dogs were subjected to standardized bilateral Ganzfeld ERGs and ophthalmoscopic examinations at regular intervals.


Photopic cone-derived ERG amplitudes were significantly lower and never reached similar levels as those recorded in control dogs. In affected dogs there was no increase with age in amplitudes recorded using 30.1 and 50.1 Hz flicker stimuli. In contrast, in the control groups the photopic b-wave amplitude recorded at 50.1 Hz increased significantly from age 5 to 8 and from 5 to 52 weeks. In affected animals, scotopic rod-derived amplitudes were significantly lower for most recordings compared to those of control dogs, although they increased significantly from age 5 to 8 weeks in both affected and controls. Both a- and b-wave implicit times were significantly longer in the youngest affected group when compared to the age-matched control group at 0.6 log cd s/m2 and 5.1 Hz single flash light stimuli. In the control dogs, however, there was a significant shortening in a-wave implicit times from age 5 to 8 weeks, and in a- and b-wave implicit times recorded at 5.1 Hz single flash stimuli from age 5 to 52 weeks.


The described retinal degeneration in the SWHD is an early onset cone–rod dystrophy, initially affecting the cone system most severely. Early functional changes are seen in the rod system as well. Inner retina also appears affected already at a young age with findings indicating postsynaptic functional changes already at the earliest time point studied, at age 5 weeks. The present study further indicates that the canine retina reaches maturity later than previously reported, or that there exist major breed differences.


ERG Dog Cone–rod dystrophy Cone–rod dysplasia Dachshund 



This work is supported by the Norwegian Research Council. We would like to thank Tone Pahle for excellent surveillance and monitoring of the dogs during anesthesia and Richard Madsen for help with the statistics.


  1. 1.
    Narfstrom K, Petersen-Jones S (2006) Diseases of the canine ocular fundus. In: Gelatt KN (ed) Veterinary ophthalmology, 4th edn. Blackwell Publishing (in press)Google Scholar
  2. 2.
    Kijas JW, Miller B, Pearce-Kelling SE, Aguirre GD, Acland GM (2003) Canine models of ocular disease: outcross breedings define a dominant disorder present in the English mastiff and Bull mastiff dog breeds. J Hered 94:27–30PubMedCrossRefGoogle Scholar
  3. 3.
    Phelan JK, Bok D (2000) A brief review of retinitis pigmentosa and the identified retinitis pigmentosa genes. Mol Vis 6:116–124PubMedGoogle Scholar
  4. 4.
    Aguirre DA, Ray K, Acland GM (2002) Candidate gene studies in canine progressive retinal atrophy. Digital J Ophthalmol Scholar
  5. 5.
    Aguirre GD, Rubin LF (1975) Rod-cone dysplasia (progressive retinal atrophy) in Irish setters. J Am Vet Med Ass 166(2):157–164Google Scholar
  6. 6.
    Aguirre GD, Alligood J, O’Brien P, Buyukmichi N (1982) Pathogenesis of progressive rod-cone degeneration in miniature poodles. Invest Ophthalmol Vis Sci 23:610–630PubMedGoogle Scholar
  7. 7.
    Narfström K, Wrigstad A (1999) Clinical, electrophysiological and morphological changes in a case of retinal degeneration in the Papillon dog. Vet Ophthalmol 2:67–74PubMedCrossRefGoogle Scholar
  8. 8.
    Zhang Q, Acland GM et al (2002) Different RPGR exon ORF15 mutations in canids provide insights into photoreceptor cell degeneration. Hum Mol Gen 11(9):993–1003PubMedCrossRefGoogle Scholar
  9. 9.
    Rubin LF (1989) Inherited eye diseases in purebred dogs. William & Wilkins, Baltimore, p 236Google Scholar
  10. 10.
    Rubin LF (1971) Clinical features of hemeralopia in the adult Alaskan malamute. J Am Vet Med Ass 158:1696–1698Google Scholar
  11. 11.
    Rubin LF (1971) Hemeralopia in Alaskan malamute pups. J Am Vet Med Ass 158:1699–1701Google Scholar
  12. 12.
    Koch SA, Rubin LF (1971) Distribution of cones in the hemeralopic dog. J Am Vet Med Ass 159:1257–1259Google Scholar
  13. 13.
    Rubin LF, Bourns TKR, Lord LH (1967) Hemeralopia in dogs: heredity of hemeralopia in Alaskan malamutes. Am J Vet Res 28:355–357PubMedGoogle Scholar
  14. 14.
    Udar N, Yelchits S, Chalukya M, Yellore V, Nusinowitz S, Silva-Garcia R, Vrabec T, Maumenee IH, Donoso L, Small KW (2003) Identification of GUCY2D gene mutations in CORD 5 families and evidence of incomplete penetrance. Hum Mutat 21:1–6Google Scholar
  15. 15.
    Michaelides M, Holder GE, Hunt DM, Fitzke FW, Bird AC, Moore AT (2005) A detailed study of the phenotype of an autosomal dominant cone–rod dystrophy (CORD7) associated with mutation in the gene for RIM1. Br J Ophthalmol 89:198–206PubMedCrossRefGoogle Scholar
  16. 16.
    Ropstad EO, Bjerkås E, Narfström K (2006) Clinical findings in early onset cone–rod dystrophy in the Standard Wire Haired Dachshund. Vet Ophthalmol (in press)Google Scholar
  17. 17.
    Kijas JW, Zangerl B, Miller B, Nelson J, Kirkness EF, Aguirre GD, Acland GM (2004) Cloning of the canine ABCA4 gene and evaluation in canine cone-rod dystrophies and progressive retinal atrophies. Mol Vis 10:223–232PubMedGoogle Scholar
  18. 18.
    Hurn SD, Hardman C, Stanley RG (2003) Day-blindness in three dogs: clinical and electroretinographic findings. Vet Ophthalmol 6:127–130PubMedCrossRefGoogle Scholar
  19. 19.
    Turney C, Chong NHV, Alexander RA, Hogg CR, Flemming L, Flack D, Barnett KC, Bird AC, Holder GE, Luthert PJ (2006) Pathological and electrophysiological features of a canine cone–rod dystrophy in the miniature longhaired dachshund. Invest Ophthalmol Vis Sci (in press)Google Scholar
  20. 20.
    Vaegan, Narfström KN (2005) Amax is the best a-wave measure for classifying Abyssinian cat rod/cone dystrophy. Doc Ophthalmol 111:33–38PubMedCrossRefGoogle Scholar
  21. 21.
    Kondo M, Sieving PA (2001) Primate photopic sine-wave flicker ERG vector modeling analysis of component origins using glutamate analogs. Invest Ophthalmol Vis Sci 42(1):305–312PubMedGoogle Scholar
  22. 22.
    Bush RA, Sieving PA (1996) Inner retinal contributions to the primate photopic fast flicker electroretinogram. J Opt Soc Am 13:557–565CrossRefGoogle Scholar
  23. 23.
    Bush RA, Sieving PA (1994) A proximal retinal component in the primate photopic ERG a-wave. Invest Ophthalmol Vis Sci 35(2):635–645PubMedGoogle Scholar
  24. 24.
    Gum GG, Gelatt KN, Samuelson DA (1984) Maturation of the retina in the canine neonate as determined by electroretinography and histology. Am J Vet Res 45:1166–1171PubMedGoogle Scholar
  25. 25.
    Hansen RM, Fulton AB (2005) Development of the cone ERG in infants. Invest Ophthalmol Vis Sci 46:3458–3462PubMedCrossRefGoogle Scholar
  26. 26.
    Petersen-Jones S, Tuntivanich N, Monttiani-Ferreira F, Khan NW (2006) Electroretinograms of dog and chicken. In: Heckenlively JR, Arden GB (eds) Principles and practice of clinical electrophysiology of vision, 2nd edn. Massachusetts Institute of Technology, Cambridge, MA, pp 912–913Google Scholar
  27. 27.
    Bedford PGC (1999) Diseases and surgery of the canine eyelid. In: Gelatt KN (ed) Veterinary ophthalmology, 3rd edn, ch 14. Lippincott Williams and Wilkins, Baltimore, p 536Google Scholar
  28. 28.
    Tian N (2004) Visual experience and maturation of retinal synaptic pathways. Vis Res 44:3307–3316PubMedCrossRefGoogle Scholar
  29. 29.
    Iijima H, Yamaguchi S, Kogure S, Hosaka O, Shibutani T (1991) Electroretinogram in cone dystrophy. Jpn J Ophthalmol 35:453–466PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, Inc. 2006

Authors and Affiliations

  • Ernst O. Ropstad
    • 1
  • Ellen Bjerkås
    • 1
  • Kristina Narfström
    • 2
  1. 1.Department of Companion Animal Clinical SciencesNorwegian School of Veterinary ScienceOsloNorway
  2. 2.Department of Veterinary Medicine and Surgery, College of Veterinary MedicineUniversity of Missouri-ColumbiaColumbiaUSA

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