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Neuronal ceroid lipofuscinosis in the Polish Owczarek Nizinny (PON) dog

A retinal study

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Abstract

Visual dysfunction and neurological symptoms were found in Polish Owczarek Nizinny (PON) dogs. Two dogs were examined, one at 2 years of age and the other one at 4 years. The oldest dog was totally blind. The 2-year-old dog developed mental disturbances and the 4-year-old dog became severely ataxic. Ophthalmoscopical findings were retinal hyper-reflectivity, attenuation of the retinal vessels and the presence of greyish to brown spots in the fundus. Electrophysiological and ultrastructural studies were performed in the 2-year-old dog. Scotopic ERG responses were absent, whereas 30 Hz cone flicker responses were recordable, although with an amplitude reduced to about 30% of the normal level. A slow negative potential replaced the c-wave, indicating a dysfunction of the RPE. Intracellular inclusions with a granular appearance or containing membranous fingerprint-like or curvilinear profiles, resembling ceroid, were found in different retinal cells. The RPE cells in the central areas were charged with autofluorescent material having similar structure, Photoreceptor degeneration was most severe in the central areas, corresponding to the RPE changes. It appears than the PON dog may provide a new animal model for neuronal ceroid lipofuscinosis.

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Authors and Affiliations

  1. Department of Ophthalmology, University of Linköping, S-581 85, Linköping, Sweden

    Anders Wrigstad & Sven Erik G. Nilsson

  2. Department of Medicine and Surgery, Faculty of Veterinary Medicine, Swedish University of Agricultural Sciences, Uppsala, Sweden

    Kristina Narfström

  3. Department of Pathobiological Sciences, School of Veterinary Medicine, University of Wisconsin, Madison, USA

    Richard Dubielzig

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  1. Anders Wrigstad
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  2. Sven Erik G. Nilsson
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  3. Richard Dubielzig
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  4. Kristina Narfström
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Wrigstad, A., Nilsson, S.E.G., Dubielzig, R. et al. Neuronal ceroid lipofuscinosis in the Polish Owczarek Nizinny (PON) dog. Doc Ophthalmol 91, 33–47 (1995). https://doi.org/10.1007/BF01204622

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