{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T04:19:28Z","timestamp":1791001168010,"version":"4.1.0"},"reference-count":24,"publisher":"American Veterinary Medical Association (AVMA)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["ajvr"],"published-print":{"date-parts":[[2005,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n\t\t\t\t<jats:p>\n\t\t\t\t\t<jats:bold>Objective<\/jats:bold>\u2014To characterize lysosomal storage body\naccumulation in the retina and brain of Tibetan Terriers\nwith ceroid-lipofuscinosis and determine whether the\ndisease in these dogs is accompanied by impaired\nretinal function and retinal degeneration.<\/jats:p>\n\t\t\t\t<jats:p>\n\t\t\t\t\t<jats:bold>Animals<\/jats:bold>\u2014Three 7- to 10-year-old Tibetan Terriers with\nceroid-lipofuscinosis and 1 healthy 5-year-old Tibetan\nTerrier.<\/jats:p>\n\t\t\t\t<jats:p>\n\t\t\t\t\t<jats:bold>Procedure<\/jats:bold>\u2014Owners completed a questionnaire to\nidentify behavioral and physical signs indicative of\nceroid-lipofuscinosis. Neurologic, behavioral, and\nophthalmologic evaluations, including full-field electroretinograms,\nwere performed on each dog.\nFluorescence, light, and electron microscopy were\nperformed on specimens of retina, cerebral cortex,\nand cerebellum of all dogs postmortem.<\/jats:p>\n\t\t\t\t<jats:p>\n\t\t\t\t\t<jats:bold>Results<\/jats:bold>\u2014Behavioral assessments of the affected\ndogs revealed moderate visual impairment in lowlight\nconditions but good vision in bright light. On\nfunduscopic evaluation of these dogs, abnormalities\ndetected ranged from none to signs of moderately\nadvanced retinal degeneration. Compared with findings\nin the control dog, electroretinography revealed\ndepressed rod cell function with some impairment\nof cone cell function in the affected dogs.\nMorphologically, disease-specific storage bodies\nwere detected in retinal M\u00fcller cells and neurons,\nparticularly in ganglion cells, and in cells of the cerebral\ncortex and cerebellum in affected dogs.\nSubstantial photoreceptor cell loss and disruption of\nphotoreceptor outer segment morphology appeared\nto develop late in the disease.<\/jats:p>\n\t\t\t\t<jats:p>\n\t\t\t\t\t<jats:bold>Conclusions and Clinical Relevance<\/jats:bold>\u2014The similarities\nbetween ceroid-lipofuscinosis in Tibetan Terriers and\nsome forms of ceroid-lipofuscinosis in humans suggest\nthat the canine disease may have a genetic and\nbiochemical basis similar to that of one of the ceroidlipofuscinosis\ndisorders in humans. 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