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Peer-reviewed veterinary case report

Retinal function loss and brain changes in Tibetan Terriers

By Katz, Martin L et al.·Published in American journal of veterinary research·2005·Mason Eye Institute, United States·View original on PubMed

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Original publication title: Assessment of retinal function and characterization of lysosomal storage body accumulation in the retinas and brains of Tibetan Terriers with ceroid-lipofuscinosis.

Species:
dog
Canine GlaucomaBrain & nervesDogs

Plain-English summary

Three Tibetan Terriers, aged 7 to 10 years, were evaluated for vision problems due to a condition called ceroid-lipofuscinosis, which affects the retina and brain. Owners reported that the dogs had moderate difficulty seeing in low light but could see well in bright light. Tests showed that the dogs had reduced function in the cells responsible for vision, and some had signs of retinal degeneration. Unfortunately, the disease led to significant loss of photoreceptor cells, which are crucial for vision. While there is no cure, understanding this condition can help in managing the symptoms and improving the quality of life for affected dogs.

People also search for: Tibetan Terrier vision problems · ceroid-lipofuscinosis symptoms in dogs · dog retinal degeneration treatment

Abstract

OBJECTIVE: To characterize lysosomal storage body accumulation in the retina and brain of Tibetan Terriers with ceroid-lipofuscinosis and determine whether the disease in these dogs is accompanied by impaired retinal function and retinal degeneration. ANIMALS: Three 7- to 10-year-old Tibetan Terriers with ceroid-lipofuscinosis and 1 healthy 5-year-old Tibetan Terrier. PROCEDURE: Owners completed a questionnaire to identify behavioral and physical signs indicative of ceroid-lipofuscinosis. Neurologic, behavioral, and ophthalmologic evaluations, including full-field electroretinograms, were performed on each dog. Fluorescence, light, and electron microscopy were performed on specimens of retina, cerebral cortex, and cerebellum of all dogs postmortem. RESULTS: Behavioral assessments of the affected dogs revealed moderate visual impairment in low-light conditions but good vision in bright light. On funduscopic evaluation of these dogs, abnormalities detected ranged from none to signs of moderately advanced retinal degeneration. Compared with findings in the control dog, electroretinography revealed depressed rod cell function with some impairment of cone cell function in the affected dogs. Morphologically, disease-specific storage bodies were detected in retinal Müller cells and neurons, particularly in ganglion cells, and in cells of the cerebral cortex and cerebellum in affected dogs. Substantial photoreceptor cell loss and disruption of photoreceptor outer segment morphology appeared to develop late in the disease. IMPLICATIONS FOR HUMAN MEDICINE: The similarities between ceroid-lipofuscinosis in Tibetan Terriers and some forms of ceroid-lipofuscinosis in humans suggest that the canine disease may have a genetic and biochemical basis similar to that of one of the ceroid-lipofuscinosis disorders in humans.

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Original publication on PubMed: https://pubmed.ncbi.nlm.nih.gov/15691038/