Clinical, electrophysiological, and morphological

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Nov 1, 2011 - pearance of distinct phthisis bulbi. Four of the phthisic eyes showed severe corneal scarring, blurring all deeper parts in two eyes. The anterior ...
Veterinary Quarterly

ISSN: 0165-2176 (Print) 1875-5941 (Online) Journal homepage: http://www.tandfonline.com/loi/tveq20

Clinical, electrophysiological, and morphological findings in a case of neuronal ceroid lipofuscinosis in The Polish Owczarek Nizinny (PON) dog K. Narfström & A. Wrigstad To cite this article: K. Narfström & A. Wrigstad (1995) Clinical, electrophysiological, and morphological findings in a case of neuronal ceroid lipofuscinosis in The Polish Owczarek Nizinny (PON) dog, Veterinary Quarterly, 17:sup1, 46-46, DOI: 10.1080/01652176.1995.9694592 To link to this article: http://dx.doi.org/10.1080/01652176.1995.9694592

Published online: 01 Nov 2011.

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Date: 31 October 2016, At: 14:15

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OPHTHALMOLOGY

pearance of distinct phthisis bulbi. Four of the phthisic eyes showed severe corneal scarring, blurring all deeper parts in two eyes. The anterior chambers in 10 eyes were free of abnormalities, but in four there were post-inflammatory scars, synechia, and clots. Five eyes had mature cataracts, four had local cataracts, in two eyes there was sclerosis, and in one eye no abnormalities were found. The fundus of nine eyes could not be inspected, due to either unclear anterior media (n=7) or lack of anesthesia (n=2). In three eyes the fundus showed no abnormalities; in two there were signs of local, and in two generalized, retinal degeneration.

with circular adhesions of the iris to the anterior capsule of the lens. After her transportation to a zoo in the Netherlands, lens extraction could be performed in one eye. After removal of the lens and its posterior capsule, white sheets of scar-tissue and patent vessels were found, filling the vitreous space of the eye completely. CONCLUSION

In conclusion, the practice of using a 'correction' stick to keep the bear on its hind limbs during 'dancing' and to prevent it from attacking its trainer, the amount of scar-tissue, the cataracts and the retinal degeneration found, and the lack of signs of penetrating wounds, all point to post-traumatic, blunt lesions induced by deliberate beating with the stick on the eyes of the dancing bears by its 'trainer'.

DISCUSSION Three bears were completely blind and two were unilaterally

blind. Only two bears had no apperent visual deficiency. In two eyes of one bear the lenses appeared to be subluxated,

CLINICAL, ELECTROPHYSIOLOGICAL, AND MORPHOLOGICAL FINDINGS IN A CASE OF NEURONAL CEROID LIPOFUSCINOSIS IN THE POLISH OWCZAREK NIZINNY (PON) DOG. '"'kr K. NarfstrOm1, and A. Wrigstad2

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PURPOSE

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of varying sizes were observed in the retinal pigment epithe-

This study describes clinical and laboratory findings in a 2year-old Polish Owczarek Nizinni (PON) dog with visual dysfunction and neurological symptoms. Methods: A clinical examination of visual and neurological

lium (RPE). Membranous 'finger print' or tubular material was seen in some of the inclusions. Such inclusions were also observed in ganglion cells and granular, round or oval inclusions were seen in the cytoplasm of photoreceptors, functions was made in the patient as well as in an age- " Muller cells, and astrocytes. Degenerative changes were obmatched normal, unrelated PON dog. Ophthalmoscopy, bioserved in the photoreceptor layer, more obvious in the cenmicroscopy, and DC-electroretinography (ERG) were pertral area than the peripheral, corresponding to the severity of formed, after which the affected dog was euthanized and the changes in the RPE. eyes were obtained for light and electron microscopy. DISCUSSION AND CONCLUSION

RESULTS

The clinical examination indicated some impaired visual capacity and the owner said that the dog sporadically had a vicious behavior. Ophthalmoscopy showed grayish to brown pigmented spots and a changed reflectivity in the tapetal fundus and there was severe vascular attenuation. Scotopic aand b-wave responses were not detected. A 30 Hz flicker response was obtained, however, that was about 30% of the response in the normal dog. Partly membrane-bound inclusions

Cases of reduced vision or blindness together with retinal changes and neurological aberrations have been observed previously in Sweden in the PON. This is the first PON dog that has been obtained and followed clinically and through laboratory studies at a rather early stage of the disease. The inclusions observed mainly in the RPE but also in other retinal cells have a structure similar to the lipopigments described in human cases and animals models of neuronal ceroid lipofuscinosis.

I Department of Medicine and Surgery. Faculty of Veterinary Medicine, Swedish University of Agricultural Sciences, 75007 Uppsala, Sweden and 2 Department of Ophthalmology. University Hospital o fLinkiiping, 581 85 Linkilping, Sweden. Supported by Swedish MRC projects no.: 12X-734 and 19X-09938.

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THE VETERINARY QUARTERLY, VOL. 17, SUPPLEMENT I, APRIL 1995

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