Retinal Photoreceptor Fine Structure in the Great Blue Heron (Ardea herodias)

The morphology of the retinal photoreceptors of the great blue heron (Ardea herodias) has been investigated by light and electron microscopy. They consist of rods, single cones and double (unequal) cones present in a ratio of about 2:1:1 respectively. The rods are slender elongated cells with outer segments that reach to the retinal epithelial (RPE) cells and are surrounded by pigment-rich apical processes of the RPE cells in the light-adapted state. The rod inner segment displays an ellipsoid of mitochondria, an hyperboloid of glycogen, much rough ER, numerous polysomes, Golgi zones and autophagic vacuoles. The rod nucleus is located deep in the outer nuclear layer and the rod synaptic pedicle displays both invaginated and superficial synaptic sites. Single cones display a slightly tapered outer segment, a large electron lucent oil droplet and an ellipsoid of mitochondria in the apex of the inner segment. Double cones consist of a long thin chief member which shows an electron dense oil droplet and a shorter, stouter accessory cone with no oil droplet but a paraboloid of glycogen below the ellipsoid. As in the single cone, polysomes, RER and Golgi zones are present in the myoid region of both members of the double cone. All photoreceptor types have a connecting cilium joining inner and outer segments. Near the external limiting membrane, the chief and accessory cones show intercellular junctions. All cone photoreceptors are relatively small in diameter and hence tightly packed. While rods are felt to undergo retinomotor movements, cones are felt to move minimally or not at all. Both single and double cones display several invaginated (ribbon) synapses as well as numerous superficial (conventional) synaptic sites.     

Retinal Photoreceptor Fine Structure in the Red-Tailed Hawk (Buteo jamaicensis)

The retinal photoreceptors of the red-tailed hawk (Buteo jamaicensis) consist of rods, single cones and double (unequal) cones present in a ratio of about 2: 11 5. In the light-adapted state, the rods are slender elongated cells with outer segments that reach to the retinal epithelial (RPE) cells. The inner segment displays an ellipsoid of mitochondria, plentiful polysomes, some rough ER and Golgi zones. The rod nucleus is located deep within the outer nuclear layer and the synaptic spherule displays both invaginated (ribbon) and superficial (conventional) synaptic sites. Single cones show a thin tapering outer segment, a large electron lucent oil droplet at the apex of the inner segment and an ellipsoid of mitochondria. Double cones consist of a larger chief member which displays a thin tapering outer segment and an electron dense oil droplet as well as a smaller accessory cone which shows no oil droplet, an ellipsoid and a paraboloid of glycogen. As in the single cone, polysomes, RER and Golgi zones are also noted in the inner segments of both members of the double cone. Near the external limiting membrane the chief and accessory cones show membrane specializations indicative of junctions on their contiguous surfaces. All cone photoreceptors are of a smaller diameter than is normally reported for avian species. Both single and double cones display several invaginated synapses as well as numerous superficial synaptic sites.     

A slowly progressive retinopathy in the Shetland Sheepdog

Objective To describe a slowly progressive retinopathy (SPR) in Shetland Sheepdogs. Animals Forty adult Shetlands Sheepdogs with ophthalmoscopic signs of SPR and six normal Shetland Sheepdogs were included in the study. Procedure Ophthalmic examination including slit‐lamp biomicroscopy and ophthalmoscopy was performed in all dogs. Electroretinograms and obstacle course‐test were performed in 13 affected and 6 normal dogs. The SPR dogs were subdivided into two groups according to their dark‐adapted b‐wave amplitudes. SPR1‐dogs had ophthalmoscopic signs of SPR, but normal dark‐adapted b‐wave amplitudes. Dogs with both ophthalmoscopic signs and subnormal, dark‐adapted b‐wave amplitudes were assigned to group SPR2. Eyes from two SPR2 dogs were obtained for microscopic examination. Results The ophthalmoscopic changes included bilateral, symmetrical, greyish discoloration in the peripheral tapetal fundus with normal or marginally attenuated vessels. Repeated examination showed that the ophthalmoscopic changes slowly spread across the central parts of the tapetal fundus, but did not progress to obvious neuroretinal thinning presenting as tapetal hyper‐reflectivity. The dogs did not appear seriously visually impaired. SPR2 showed significantly reduced b‐wave amplitudes throughout dark‐adaptation. Microscopy showed thinning of the outer nuclear layer and abnormal appearance of rod and cone outer segments. Testing for the progressive rod–cone degeneration ( prcd )‐mutation in three dogs with SPR was negative. Conclusion Slowly progressive retinopathy is a generalized rod–cone degeneration that on ophthalmoscopy looks similar to early stages of progressive retinal atrophy. The ophthalmoscopic findings are slowly progressive without tapetal hyper‐reflectivity. Visual impairment is not obvious and the electroretinogram is more subtly altered than in progressive retinal atrophy. The etiology remains unclear. SPR is not caused by the prcd‐mutation.     

The electroretinographic phenotype of dogs with Golden Retriever muscular dystrophy

Objective To characterize the flash electroretinogram (ERG) in the Golden Retriever muscular dystrophy (GRMD) dog and to compare the results with those from a control group of Golden Retrievers. To investigate whether similar abnormalities of the ERG as those found in a majority of human patients with Duchenne muscular dystrophy (DMD) are also observed in the GRMD dog, the canine model for DMD. Animals Five GRMD dogs and five age‐matched clinically normal Golden Retrievers. Procedure An ophthalmic examination was carried out prior to performing electroretinography under general anesthesia. Rod, combined rod–cone and oscillatory potentials responses were recorded after dark adaptation. Responses to 30‐Hz‐flicker were recorded after light adaptation. The ERG responses of the GRMD dogs were compared with those of the control dogs by use of a Wilcoxon signed rank test. Results GRMD dogs had significantly reduced a and b‐wave amplitudes after dim white flash stimuli (rod response) and reduced a‐wave amplitude after bright white flash stimuli (rod–cone response). Conclusion and clinical relevance The ERG abnormalities observed in the GRMD dog suggest a dysfunction in the rod signaling pathway. These ERG alterations are different from those observed in human patients with DMD.     

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

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.     

Cytologic appearance of retinal cells included in a fine‐needle aspirate of a meningioma around the optic nerve of a dog

A 6‐year‐old Wirehair Dachshund had a meningioma around the optic nerve that caused exophthalmos. A benign mesenchymal tumor was suspected based on the cytologic pattern of a fine‐needle aspirate, and a meningioma was diagnosed by histopathologic examination. In addition to the meningioma cells, the cytologic smears included groups of cells from apparently 4 layers of normal retina. In particular, uniform rod‐shaped structures in the cytologic sample could suggest rod‐shaped bacteria, but these structures were identified as cylindrical outer segments of photoreceptor rod cells. Other retinal structures recognized included pigmented epithelial layer cells with their uniquely formed pigment granules, the characteristic bi‐lobed, cleaved nuclei from the outer nuclear layer, and nerve tissue likely from the outer plexiform layer of the retina.     

Progressive retinal atrophy in Tibetan terriers

Progressive retinal atrophy was studied in 17 Tibetan Terriers. The diagnosis was made on the basis of clinical signs of the disease, retinal histopathologic findings, or both. Affected dogs were the progeny of matings of affected or ophthalmoscopically normal dogs. Results of the mating supported a simple autosomal recessive mode of inheritance. The disease initially could be diagnosed by findings of night blindness and ophthalmoscopic signs of tapetal hyperreflectivity in affected dogs that were approximately 1 year old. Electroretinograms recorded from affected dogs, compared with those of clinically normal dogs of the same age, did not reveal appreciable abnormalities until affected dogs were 10 months old, at which time a reduction in the amplitude of the b wave was seen in response to a Ganzfeld white-light stimulus. The peak times of the response were unaffected. With progression of the disease, the electroretinographic b-wave amplitude was gradually reduced, and the electroretinographic response was extinguished in affected dogs by the time they were 30 months old. Early in the disease, rod and cone functions were affected equally, with more rapid loss of rod function developing only later in the disease. Fluorescein angiography of affected dogs did not reveal abnormalities earlier than could be detected by ophthalmoscopy. Despite the electroretinographic findings, histopathologic findings included patchy disorientation and disorganization of the outer segments of rods and cones in affected dogs as young as 9 weeks. With progression of the disease, rods were lost at a faster rate than cones, and atrophy of the inner retinal layer was observed.     

Detection of cone dysfunction induced by digoxin in dogs by multicolor electroretinography

It is difficult to detect discrete cone function with the present conventional electroretinography (ERG) examination. In this study, we developed contact electrodes with a built-in color (red (644 nm), green (525 nm), or blue (470 nm)) light source (color LED-electrode), and evaluated an experimental model of digoxin in the dog. First, 17 normal Beagle dogs were used to determine which electrode works well for color ERG measurement on dogs. Then, color ERG was performed on seven normal Beagle dogs at various points during a 14-day period of digoxin administration. A single daily dose of 0.0125 mg/kg/day, which is within the recommended oral maintenance dosage range for dogs, was administered orally for 2 weeks. Ophthalmic examination, measurement of plasma concentration of digoxin, and color ERG examination were performed. On first examination, amplitudes of all responses were significantly (P < 0.01) lower with the red, than with the blue and green electrodes during ERG recording. In ERG using the red electrode, the standard deviation was large. According to these preliminary results, the red electrode was not used in the experimental dog model with digoxin. In the digoxin administrated animals, no significant change was observed in the ophthalmic examination findings. The digoxin level increased steadily throughout the dosing period but was always within the therapeutic range for dogs. In rod ERG, no abnormalities were detected with any electrode. In standard combined ERG, decreased amplitude of the a-wave was detected with every electrode. In single flash cone ERG, prolongation of implicit time was detected by color ERG with the blue and green electrodes. In 30-Hz flicker ERG, decreased amplitude was detected only by color ERG with the blue electrode. The decreased amplitude and prolonged implicit time recovered after termination of digoxin administration. Cone dysfunction induced by digoxin in the dog was revealed by multicolor ERG using blue and green LED-electrodes. Multi-color ERG was useful for detecting cone type-specific dysfunction in the dog.     

Fine structure of the retinal pigment epithelium, Bruch's membrane and choriocapillaris in the horse

The fine structure of the retinal pigment epithelium (RPE), Bruch's membrane and choriocapillaris was investigated by light and transmission electron microscopy in both the tapetal and non-tapetal fundus of the horse eye. In all locations, the RPE consisted of a single layer of low cuboidal cells. The epithelial cells were joined laterally by apically located tight junctions. These cells displayed numerous basal infoldings and abundant thin apical processes which enclosed the rod outer segments. The epithelial cell nuclei were large and located basally. Within the epithelial cells, smooth endoplasmic reticulum was very abundant, while rough endoplasmic reticulum was scarce, polysomes and mitochondria, which often display a ring-shaped structure, were abundant. Melanosomes were abundant in the non-tapetal area but absent in the tapetal area. Bruch's membrane was pentalaminate throughout the retina. The endothelium of the choriocapillaris was heavily fenestrated.     

Immunohistochemical studies of cone photoreceptors and cells of the inner retina in feline rod-cone degeneration

Previous studies using electroretinography and immunohistochemistry have shown normal cone function and structure in early stages of hereditary rod‐cone degeneration of Abyssinian cats. To further investigate the cone photoreceptors and the inner retina of dystrophic cats, antibodies against green‐ and blue‐sensitive cones and specific cell types of inner retina were used in seven cats with the recessively inherited rod‐cone degeneration, and three normal European short‐haired cats. There was a reduction in number of both types of cones early in the disease. Changes at early stages of disease also occurred among horizontal cells in which there was an extension and a thickening of their lateral processes. The regular configuration of bipolar cells was changed in the more advanced stages of disease and their apical dendrites were lost. Abnormalities were not observed in the amacrine cells and in the ganglion cell layer in any of the present cases. This study shows that the cone system is morphologically abnormal in young cats at an earlier stage of disease than previously shown. The present findings also support the assumption that the inner retina is largely preserved throughout the disease process.     

Retinal Photoreceptor Fine Structure in the American Crow (Corvus brachyrhynchos)

The morphology of the light-adapted retinal photoreceptors of the American crow (Corvus brachyrhynchos) has been investigated by light and electron microscopy. They consist of rods, single cones and double (unequal) cones present in a ratio of about 4:3:3 respectively. The rods are stout cells with a long inner segment and an outer segment that reaches to the RPE cell body. In the light-adapted state, the pigment-laden apical processes of the RPE cells surround cell photoreceptor types for most of their length. The rod inner segment displays an ellipsoid of mitochondria, a large hyperboloid of glycogen, much RER, numerous polysomes, Golgi zones and autophagic vacuoles. Single cones show a slightly tapered outer segment, a large and usually heterogeneous oil droplet and an ellipsoid of mitochondria at the apex of the inner segment. Double cones consist of a longer, stouter chief member which displays a more homogeneous oil droplet and a prominent paraboloid of glycogen and a slightly shorter and thinner accessory member with no oil droplet or paraboloid. Both members of the double cone as well as the single cones show a prominent ellipsoid and plentiful polysomes, RER and Golgi zones in the inner segment. Along the length of the contiguous membranes of the two members of the double cone are presumed interreceptor junctions. All cone photoreceptors are relatively small in diameter and hence are tightly packed. Judging by their morphology in the light-adapted state neither rods nor cones are felt to undergo photomechanical movements in this species. Rods and cones (both types) display both invaginated (ribbon) synapses as well as numerous flat (conventional) synaptic sites.     

Photoreceptor cell dysplasia in two Tippler pigeons

Two 12-week-old Tippler pigeons were evaluated for ocular abnormalities associated with congenital blindness. The pigeons were emaciated and blind. Biomicroscopy and direct and indirect ophthalmoscopy findings of the Tippler pigeons were normal with the exception of partially dilated pupils at rest. Scotopic (blue stimuli) and photopic monocular electroretinograms were extinguished in the blind Tippler pigeons. Histological and electron microscopy studies revealed reduced numbers of rods and cones, and an absence of the double cone complex. The photoreceptor cells' outer segments were absent, and the inner segments were short and broad. The number of cell nuclei in the outer and inner nuclear layers was decreased, and the internal and external plexiform layers were reduced in width. Photoreceptor cell endfeet with developing synaptic ribbons were present in the external plexiform layer. Inflammatory cell and subretinal debris was not seen. The electroretinographic, histopathological, and ultrastructural findings of the blind Tippler pigeons support the diagnosis of a photoreceptor cell dysplasia.     

The normal electroretinogram in adult healthy Shih Tzu dogs using the HMsERG

Electroretinography (ERG) is a reliable diagnostic tool for the diagnosis of retinal disease. It measures electric potentials occurring in the retina in response to light stimulation. In this study, we examined the normal electroretinogram using the Handheld Multispecies ERG (HMsERG) in Shih Tzu dogs. ERG recordings were performed in twelve eyes of six healthy Shih Tzu dogs. Dogs were anesthetized with a combination of medetomidine and ketamine. Proparacaine eye drops were also applied as a topical anesthetic. Tropicamide eye drops were applied for mydriasis. After 20 min of dark adaptation, we recorded the amplitudes and implicit times of the b-waves of the rod, standard rod and cone (Std R&C), high-intensity rod and cone (Hi-int R&C), and cone systems, and responses of the cones and inner retina by flicker light stimulation (cone flicker). Results showed that mean the amplitudes of a-waves of Std R&C, Hi-int R&C, and the cone responses were 141.25 µV, 173.00 µV, and 12.92 µV, respectively. The b-waves of the rod responses ranged from 141.58 to 155.25 µV; the Std R&C was 314.75 µV, the Hi-int R&C was 329.42 µV, the cones were 37.75 µV, and the flicker responses were 64.08 µV. The b/a ratios for the Std R&C, Hi-int R&C, and the cone response were 2.29, 1.94, and 3.71, respectively. Mean implicit time of the a-wave of the Std R&C was 15.12 ms, of Hi-int R&C was 13.42 ms, and of the cone response was 7.22 ms. The b-wave of the rod responses ranged from 68.12 to 72.68 ms, of Std R&C were 37.28 ms, of Hi-int R&C were 41.90, of the cone responses were 38.12 ms, and of the cone flicker responses were 22.80 ms. We believe that these parameters can be used as reference "normal" ERGs ranges for Shih Tzu dogs using the HMsERG under medetomidine and ketamine anesthesia.     

Fine structure of the retinal pigment epithelium, Bruch's membrane and choriocapillaris in the camel

The morphology of the retinal pigment epithelium (RPE) and closely associated Bruch's membrane and choriocapillaris was investigated by light and transmission electron microscopy in the camel (Camelus dromedarius). The study showed that RPE is composed of a single layer of hexanocuboidal cells that were joined laterally by a series of apically located tight junctions. In addition, adjacent from internal side of cell membrane at the level of tight junctions, an undefined structure which resembled the myofibrillar organization of skeletal muscles in appearance was located. These cells displayed numerous short basal infoldings and abundant thin apical processes which enclosed the rod outer segments. The epithelial cell nuclei were large, vesicular and eccentrically located. Within the epithelial cells, smooth endoplasmic reticulum was very abundant, while rough endoplasmic reticulum was present only in small amounts. Polysomes were also numerous and the mitochondria often displayed a ring-shaped structure. Lipofuscin granules were plentiful in all locations. Bruch's membrane (complexus basalis) was typically pentalaminate throughout the retina. The endothelium of the choriocapillaris facing Bruch's membrane was extremely thin and heavily fenestrated. These fenestrations displayed typical single-layered diaphragm as noted in most species.     

Functional disorder of the retina in manganese-deficient Japanese quail revealed by electroretinography using a contact lens electrode with built-in light source

Manganese deficiency results in neurological and skeletal defects, together with ultrastructural disarrangement of the retina in rats. Wild birds show a range of Mn concentrations in their tissues, including the liver, raising the possibility of Mn-related disorders in the wild. Electroretinography (ERG) provides a useful noninvasive approach to evaluate visual function. This method is especially useful in birds, as objective analysis of them is very difficult, while they have well-developed vision. In this study, we carried out a convenient and reliable ERG recording using a contact lens electrode with a built-in light source (LED electrode) of Japanese quail (Coturnix coturnix japonica) fed a Mn-deficient diet. After 10 min light adaptation, single-flash and flicker cone responses were reproducibly recorded to cause an intensity-dependent increase in amplitude of both a-wave and b-wave in single-flash ERG. Mn-deficient feeding markedly decreased the Mn concentration in the liver by almost half in 3 to 6 weeks, followed by body weight loss in 13 to 15 weeks. Implicit time of a-wave and b-wave cone response by single-flash stimulation was significantly delayed in quail with a Mn depletion from 3 to 6 weeks. Every cone response of the Mn-deprived quail had a tendency to decrease amplitude. The ultrastructure of cone photoreceptor cells was disorganized by Mn deficiency, including changes in outer segment discs of photoreceptor cells. These results suggest the essential role of Mn in the integrity of the retinal function of birds.     

Anaplastic ependymoma in the cervical spinal cord of a maltese dog

An 8-year and 6-month-old female Maltese dog showed a stoop with rigidity of her cervix and back. Neurologic examination showed loss of proprioception, and deficiency of pain response. Postmortem examination revealed the neoplastic mass replacing the central area in the cervical spinal cord at the level from 4th to 5th segments. Histologically, the mass was composed of neoplastic ependymal cells. The neoplastic cells showed marked atypism, and occasionally formed ependymal rosettes. Based on the morphologic features, the tumor was diagnosed as anaplastic ependymoma. Immunohistochemistry showed that the neoplastic cells were negative for glial fibrillary acid protein, and slightly positive for vimentin and cytokeratin.     

Microvascularization of the canine hepatic ducts

The angioarchitecture of the proximal and distal segments of the hepatic duct in the dog was investigated by means of vascular corrosion casts under a scanning electron microscope. The results of observations indicated a change of the pattern of vascularization of the hepatic duct along with the increasing distance of the hepatic duct from the liver and increasing diameter of the duct. In the proximal hepatic duct, the main blood vessels run along the duct as a pair of supplying arteriole and voluminous collecting venule, while in the distal segments of the hepatic duct on the opposite margin of the duct two vascular triads were observed, composed of two venules and one medial arteriole. On the surface of both segments of the hepatic duct, there are well-anastomosed outer venous plexuses. In the distal segments of the hepatic duct, the outer venous plexus accompanies a fine outer arterial rete. Observations of the intramural network indicate the presence of single terminal arterioles running to mucosa and supplying a subepithelial capillary network. Differences were observed in the blood drainage from the mucosa, as in the proximal segment of the hepatic duct single post-capillary venules are found, while in the distal segment in the mucosa a well-developed mucosal venous plexus is formed. In the well-developed venous system of the hepatic duct no valves were observed.     

Photoreceptor density of the domestic pig retina

The spatial distribution and densities of photoreceptors in seven whole-mounted porcine retinas were studied and maps illustrating photoreceptor topography were constructed. Total photoreceptor densities ranged from to 83 000 to 200 000 cells/mm², with a mean of 138 500 cells/mm². Cone densities ranged from 39 000 (area centralis) to 8500 cones/mm² (peripherally), with a mean of 16 400 cones/mm². Rod:cone ratios ranged from 3:1 centrally to 16:1 peripherally, with a mean ratio of 8:1. Averaged photoreceptor densities are greatest (166 000 cells/mm²) within the central inferior retina, and regional differences in rod:cone ratios were found. Cone densities are increased in a broad region dorsal to the optic disk, extending both nasally and temporally. This region is believed to represent the area centralis. Cone densities gradually decrease and taper towards the periphery and inferior retina as rod:cone ratios increase. In addition to the many anatomic and ultrastructural similarities to the human eye, this study illustrates similarities within the photoreceptor mosaic of these two species and supports the use of the pig retina as a model for human/animal research.     

Clinical findings in early onset cone-rod dystrophy in the Standard Wire-haired Dachshund

OBJECTIVE: To describe the clinical findings and the age of onset of cone-rod dystrophy (crd) in the Standard Wire-haired Dachshund (SWHD) and to evaluate which clinical tests could be used to obtain a reliable diagnosis. ANIMALS: Sixty-eight SWHD and SWHD-derived dogs were used, including 23 affected with crd and 45 controls, respectively. PROCEDURES: The dogs were subjected to behavioral testing, examination of pupillary light reflexes (PLRs), indirect ophthalmoscopy and bilateral full field electroretinography (ERG). RESULTS: The majority of affected puppies (5-10 weeks) displayed pin-point sized pupils upon examination with focal light. All dogs in the control group, except one, displayed normal PLRs upon examination. In all crd-affected dogs there was a great variation both in age of onset and in clinical appearance of retinal changes upon fundoscopy. Two siblings displayed panretinal degeneration at the age of 10 months while other affected dogs showed early changes at the age of 3 years. Generalized bilateral retinal atrophy was the end stage of the disease. The maze test revealed no obvious differences among affected and unaffected groups. ERG recordings showed only slightly reduced rod, and mixed rod-cone responses, but severely reduced cone single flash a- and b-wave amplitudes, and cone flicker amplitudes were observed in all affected dogs. CONCLUSION: Presence of pin-point sized pupils in young SWHDs was found to be an important indicator of early onset crd. Fundoscopic changes and progression of disease at later stages resembled those previously described in the majority of progressive retinal atrophies in dog. ERG was found to be the most reliable diagnostic procedure to clinically diagnose crd in the SWHD.