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(Investigative Ophthalmology and Visual Science. 2007;48:4240-4249.)
© 2007 by The Association for Research in Vision and Ophthalmology, Inc.
DOI:  10.1167/iovs.04-0737
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Pathological and Electrophysiological Features of a Canine Cone–Rod Dystrophy in the Miniature Longhaired Dachshund

Clare Turney,1 N. H. Victor Chong,1,2 Robert A. Alexander,1 Chris R. Hogg,3 Lorraine Fleming,4 Deborah Flack,4 Keith C. Barnett,4 Alan C. Bird,3 Graham E. Holder,3 and Philip J. Luthert1

1From the UCL Institute of Ophthalmology, London, United Kingdom; 2King's College Hospital, London, United Kingdom; 3Moorfields Eye Hospital, London, United Kingdom; and the 4Animal Health Trust, Newmarket, United Kingdom.

PURPOSE. To characterize the electrophysiological and histopathological features of a retinal degenerative disease in a colony of miniature longhaired dachshunds known to have a form of progressive retinal atrophy (PRA).

METHODS. Serial electroretinograms were recorded from affected homozygous (n = 36) and heterozygous (n = 15) dogs. Morphologic investigations including immunohistochemistry and lectin histochemistry were performed on selected homozygous animals (n = 15).

RESULTS. Clinical findings included loss of tapetal hyperreflectivity. The mode of inheritance was autosomal recessive. An early dramatic reduction of cone-specific ERG amplitude with a more modest reduction in rod b-wave amplitude was demonstrated. Progressively, rod specific responses diminished until there were no recordable responses to the ERG stimuli at 40 weeks of age. Morphologic changes confirmed early cone inner and outer segment loss. Other abnormalities included opsin mislocalization and outer nuclear layer thinning due to the subsequent loss of rod photoreceptors.

CONCLUSIONS. A novel canine cone–rod dystrophy has been identified.






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