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1.
An autosomal recessive retinal disease with a late onset in Swedish Papillon dogs has recently been described. A 7-year-old Papillon dog showed no obvious signs of visual impairment and only minor ophthalmoscopic changes. Cone ERG b-wave amplitudes were within normal limits, while rod responses were nonrecordable or severely abnormal. Ultrastructural examination showed a generalized retinal degenerative disease, most prominent in the peripheral areas. The inferior retina was more severely affected than the superior areas. Both rods and cones showed morphological changes. The Papillon dog is another dog breed affected by progressive rod-cone degeneration, with similarities to the canine retinal disease given the gene symbol prcd . 相似文献
2.
Katz ML Sanders DA Sanders DN Hansen EA Johnson GS 《American journal of veterinary research》2002,63(6):890-895
OBJECTIVE: To determine whether the late onset form of inherited ceroid lipofuscinosis (CL) in Tibetan Terriers is accompanied by low plasma carnitine concentrations prior to the appearance of clinical signs. ANIMALS: 129 healthy Tibetan Terriers, 12 Tibetan Terriers with CL, and 95 healthy purebred dogs of other breeds. PROCEDURE: After withholding food, blood samples were collected from all dogs into tubes containing EDTA. Blood samples were analyzed for plasma-free carnitine and acyl-carnitines concentrations. RESULTS: Neither the mean plasma total carnitine concentration nor the mean fraction of carnitine in the free form differed significantly between Tibetan Terriers with CL and healthy Tibetan Terriers. Among Tibetan Terriers and the general dog population, plasma carnitine concentration increased with age. Castrated males had an overall increase in plasma carnitine concentrations and variability, compared with sexually intact males. By comparison, plasma carnitine concentrations were not significantly different between spayed and sexually intact females. The mean plasma carnitine concentration in the Tibetan Terriers was approximately 22% higher than in the general population of healthy dogs of other breeds. CONCLUSIONS AND CLINICAL RELEVANCE: Contrary to what is seen in early onset CL in English Setters and in humans with some forms of CL, plasma carnitine concentrations are not decreased in the late-onset disorder in Tibetan Terriers. Our large-scale study establishes reference range values for plasma carnitine concentrations in dogs as functions of age and sex that will be useful in evaluating potential carnitine deficiencies in other disorders in dogs. 相似文献
3.
A hereditary cerebellar degenerative disorder has emerged in Scottish Terriers. The aims of this study were to describe and quantify polyglucosan body accumulation and quantify Purkinje neurons in the cerebellum of affected and control dogs. The brains of 6 affected Scottish Terriers ranging in age from 8 to 15 years and 8 age-matched control dogs were examined histopathologically. Counts of Purkinje neurons and polyglucosan bodies were performed in control and affected dogs on cerebellar sections stained with periodic acid-Schiff. Affected dogs showed a significant loss of Purkinje neurons compared with control dogs (vermis: P < .0001; hemisphere: P = .0104). The degeneration was significantly more pronounced dorsally than ventrally (P < .0001). There were significantly more polyglucosan bodies in the ventral half of the vermis when compared with the dorsal half (P < .0001) in affected dogs. In addition, there were more polyglucosan bodies in the ventral half of the vermis in affected dogs than in control dogs (P = .0005). Polyglucosan bodies in all affected dogs stained positively with toluidine blue and alcian blue. Immunohistochemically, polyglucosan bodies in affected dogs were positive for neurofilament 200 kD and ubiquitin and negative for glial fibrillary acidic protein, synaptophysin, neurospecific enolase, vimentin, and S100; the bodies were negative for all antigens in control dogs. Ultrastructurally, polyglucosan bodies in 1 affected dog were non-membrane-bound, amorphous structures with a dense core. This study demonstrates significant Purkinje cell loss and increased polyglucosan bodies in the cerebellum of affected Scottish Terriers. 相似文献
4.
Henke D Böttcher P Doherr MG Oechtering G Flegel T 《Journal of veterinary internal medicine / American College of Veterinary Internal Medicine》2008,22(4):969-975
BACKGROUND: Cerebellar cortical degeneration exists in American Staffordshire Terriers. Magnetic resonance imaging (MRI) can be suggestive, but a definitive diagnosis requires histopathology. HYPOTHESIS: Computer-assisted MRI morphometry can be used to distinguish between American Staffordshire Terriers with or without cerebellar cortical degeneration. ANIMALS: Normal American Staffordshire Terriers (n = 17) and those with clinical signs of cerebellar cortical degeneration (n = 14). METHODS: This was a partly retrospective and partly prospective study. Causes of cerebellar disease were ruled out with brain MRI, cerebrospinal fluid (CSF) analysis, CBC, blood biochemistry, and clinical follow-up. On T2-weighted midsagittal MR images, the following parameters were calculated: size of the cerebellum relative to the entire brain, size of the CSF space surrounding the cerebellum relative to the cerebellum, and 2 threshold-dependent cerebellar CSF indices (with and without surrounding CSF). RESULTS: Statistical analyses indicated a significantly lower relative cerebellar size (P < .001) and a larger relative cerebellar CSF space (P < .001) in dogs with cerebellar cortical degeneration. The measurement of relative cerebellar size could distinguish between affected and nonaffected dogs with a sensitivity and a specificity of 93 and 94%, respectively, using a cut-off of 13.3%. Using a cut-off of 12.8%, the measurement of relative CSF space could distinguish between both groups with a sensitivity of 93% and a specificity of 100%. There was a significant difference in 1 of the 2 CSF indices between affected and normal dogs. CONCLUSIONS AND CLINICAL IMPORTANCE: Relative cerebellar size and relative CSF space calculated from MRI are effective in American Staffordshire Terriers to differentiate between normal animals and those with cerebellar cortical degeneration. 相似文献
5.
Hanzlícek D Kathmann I Bley T Srenk P Botteron C Gaillard C Jaggy A 《Schweizer Archiv für Tierheilkunde》2003,145(8):369-375
Three American Staffordshire Terriers were presented with gait abnormalities and loss of balance at the age of 4.5 (female) and 6 years (2 males). The onset varied between 3 and 5 years of age and the clinical signs were slowly progressive. The neurological examination revealed symmetrical generalized cerebellar ataxia with hypermetria, stiffness, and loss of balance with no evidence of paresis. The menace reflex was decreased in one dog and absent in another. A positional nystagmus was found in two dogs. The dogs were euthanized and a histopathological examination of each brain was performed. Pathological changes were confined to the cerebellum. The main finding was loss of Purkinje cells, as well as depletion of granular cell bodies and shrinkage of the granular and molecular cell layer. These findings are consistent with cerebellar cortical abiotrophy. A genetic basis is supposed, but the mode of inheritance is not determined yet. In contrast to some spinocerebellar ataxias in humans, the cause of Purkinje cell degeneration in cerebellar cortical abiotrophy of dogs is not known. 相似文献
6.
7.
N J Millichamp R Curtis K C Barnett 《Journal of the American Veterinary Medical Association》1988,192(6):769-776
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. 相似文献
8.
N J Millichamp 《Veterinary Clinics of North America: Small Animal Practice》1990,20(3):799-835
Retinal degenerations in the dog and cat are an important cause of blindness in these species. Particularly in the dog, many retinal degenerations, collectively called progressive retinal atrophy, seen in clinical practice are inherited. The clinical signs, electrophysiological findings, pathology, and underlying biochemical defects in the retina vary from breed to breed. Specific categories of inherited retinal degeneration are now recognized, and classified into early onset photoreceptor dysplasias, late-onset retinal degenerations, or retinal degenerations secondary to primary RPE dystrophy. As new inherited retinal degenerations are reported in different breeds they can generally be assigned to one these categories. Other causes of retinal degeneration include nutritional deficiencies, glaucoma, inflammation, ischemia, and toxins. Idiopathic retinal degeneration occurs in the dog with some frequency. 相似文献
9.
The objective of the study was to describe a form of early retinopathy in the Bernese Mountain Dog in France. Sixty-two Bernese Mountain Dogs (38 males and 24 females), whose ages ranged from 2 months to 9 years, were examined over a period of 3 years. Visual behavior, pupillary light reflexes, menace responses and ocular fundi were evaluated in all animals. Electroretinography (ERG) was performed on six of the affected dogs after dark adaptation. Fluorescein angiography (FA) was performed on one affected dog. Whenever possible, the pedigrees of the affected dogs were evaluated. A histological examination of the retina was performed on one of the affected dogs. Eight dogs (seven males and one female) were diagnosed with retinopathy with an early onset of clinical signs. (Four dogs were aged between 3 months and 1 year, two dogs were aged 2 and 3.5 years, and one dog was 7 years old.) Night vision was impaired in most of the dogs. Retinopathy was characterized ophthalmoscopically by a bilateral, symmetrical horizontal zone of tapetal hyper-reflectivity adjacent to and above the optic disc, and sometimes by peri-papillary hyper-reflectivity. ERG changes included a reduction in b-wave amplitude varying from one case to another. Fluoroscein angiography demonstrated an ischemic-type alteration with epitheliopathy opposite the hyper-reflective zone. Pedigree examinations suggested a familial predisposition. The histological examination indicated photoreceptor degeneration that was more pronounced in the central tapetal zone. In France, retinopathy in the Bernese Mountain Dog involves an early retinal degeneration that produces specific manifestations of the ocular fundus, night visual impairment or blindness, and has familial transmission. 相似文献
10.
Severe, unilateral, unresponsive keratoconjunctivitis sicca in 16 juvenile Yorkshire Terriers 总被引：1，自引：1，他引：0
OBJECTIVE: To present ophthalmic findings, clinical data, and treatment outcomes of 16 juvenile Yorkshire Terriers with severe unilateral keratoconjunctivitis sicca. RESULTS: Each of the 16 dogs exhibited extreme unilateral dryness associated with blepharospasm, mucoid discharge, and corneal vascularization. Ages of affected dogs at presentation ranged from 5 months to 4 years. Mean Schirmer tear test (STT) result for affected eyes was 1 mm/min. Topical application of 0.2% cyclosporine to the affected eye was not associated with improvement in STT values in any dog. Clinical signs subjectively improved with topical application of 20% chondroitin sulfate ophthalmic solution in some dogs, and transposition of the parotid duct was performed in three dogs. Histopathologic examination in one dog failed to show evidence of orbital lacrimal gland tissue. Clinical signs, age of presentation, disease severity, and lack of response to treatment are consistent with breed-related unilateral aplasia or hypoplasia of the lacrimal gland. CONCLUSION: Lacrimal gland aplasia or hypoplasia should be considered in young dogs with severe unilateral ocular dryness, especially female Yorkshire Terriers. 相似文献
11.
Ketteritzsch K Hamann H Brahm R Grussendorf H Rosenhagen CU Distl O 《Veterinary journal (London, England : 1997)》2004,168(2):151-159
We analysed the systematic environmental influences and the additive genetic variation for the presumed inherited eye diseases (PIED), membrana pupillaris persistens, distichiasis, primary lens luxation, non-congenital cataract, and progressive retinal atrophy, in Tibetan Terriers. Data were obtained from the International Kennel Club for Tibetan dog breeds in Germany. PIED were recorded in the years 1987 to 2001 by standardised protocols of the Dortmunder Kreis, the association for diagnosis of inherited eye diseases in animals (DOK). The material included 849 Tibetan Terriers from 596 litters in 203 different kennels. The multivariate linear animal model using residual maximum likelihood methods regarded the fixed effects of sex, birth year, experience of the veterinary ophthalmologist, litter size, percentage of examined dogs per litter, inbreeding coefficient, and age at examination. The common environment of the litter and the additive genetic effect of the animal were taken into account as randomly distributed effects. The heritability estimates for PIED in Tibetan Terriers were h2=0.17+/-0.04 (membrana pupillaris persistens), h2=0.04+/-0.03 (distichiasis), h2=0.13+/-0.04 (primary lens luxation), h2=0.13+/-0.04 (non-congenital cataract), and h2=0.49+/-0.08 (progressive retinal atrophy). The additive genetic correlation between non-congenital cataract and progressive retinal atrophy was highly positive rg=0.76+/-0.11, while that between membrana pupillaris persistens and progressive retinal atrophy rg=-0.43+/-0.14 was highly negative. The number of examinations performed by the veterinary ophthalmologists was associated with higher heritabilities for non-congenital cataract and progressive retinal atrophy. We concluded from our analysis that all investigated PIED in Tibetan Terriers are genetically influenced. 相似文献
12.
Whiteman AL Klauss G Miller PE Dubielzig RR 《American journal of veterinary research》2002,63(2):257-261
OBJECTIVE: To investigate cellular death in the neurosensory portion of the retina during the first 7 days after onset of clinical signs of overt primary angleclosure glaucoma (PACG) in dogs. SAMPLE POPULATION: 14 globes from dogs with PACG and 2 normotensive globes from dogs with PACG in the opposite eye. PROCEDURES: Retinas were examined via light microscopy and terminal deoxynucleotidyl transferase-mediated biotin-dUTP nick end-labeling. RESULTS: Necrosis of ganglion cells and segmental degeneration of the nerve fiber layer rapidly progressed to scattered full-thickness retinal attenuation and disorganization. Apoptosis was detectable within 1 day after onset of PACG and was prominent by 3 days. Necrosis of ganglion cells was significantly greater in retinas affected for < or = 1 day, compared with retinas affected for > 1 day. In contrast, apoptosis in the ganglion cell layer was significantly greater in retinas affected for > 1 day, compared with retinas affected for < or = 1 day. End-stage retinal atrophy was seen by day 7. CONCLUSIONS AND CLINICAL RELEVANCE: The presence of necrotic ganglion cells within 1 day after onset of clinical signs suggests a narrow window of opportunity to initiate effective therapy in overt PACG. Photoreceptor death is an important and striking aspect of neurosensory retinal degeneration after acute onset of PACG. 相似文献
13.
Djajadiningrat-Laanen SC Boevé MH Stades FC van Oost BA 《The Journal of small animal practice》2003,44(3):113-116
Four Irish setters were diagnosed with bilateral retinal degeneration and cataracts at an age ranging from six to 11 years. In three of these dogs, progressive night blindness was reported from an age of eight to 11 years. In the fourth dog, aged six, no signs of visual impairment had been noticed. In all four dogs, the rod-cone dysplasia type 1 (rcd1) mutation was excluded as a cause, using an allele-specific PCR. From their three-generation pedigrees, a familial relationship was detected in three out of four dogs, which were also related to four additional Irish setter dogs with a history and clinical signs suggestive of late-onset progressive retinal degeneration. These results suggest the existence of a possibly hereditary, late-onset, progressive retinal atrophy in the Irish setter breed, that is distinct from rcd1. 相似文献
14.
Eva Hertil Tomas Bergstrm Ülle Kell Lena Karlstam Stina Ekman Bjrn Ekesten 《Veterinary ophthalmology》2010,13(2):110-116
The Jämthund is the fourth most common breed in Sweden with approximately 1600 pups registered each year. Although it has been known that some adult dogs go blind, so they cannot hunt, the Jämthund dog has historically not been screened for hereditary eye diseases. This report describes nine Swedish Jämthund dogs with retinal degeneration. These dogs represent all Jämthund dogs diagnosed with progressive retinal atrophy (PRA) by the Swedish Eye Panel and registered with the Swedish Kennel Club from January 1998 to September 2008. The dogs were examined with indirect opthalmoscopy and slitlamp biomicroscopy. Additionally, electroretinograms (ERGs) following ECVO guidelines were performed in two dogs (one affected and one normal) and the eyes from three affected dogs were examined by light‐microscopy postmortem. Typical findings were bilateral symmetric generalized retinal degeneration with tapetal hyper‐reflectivity, attenuation of blood vessels and pigment clumping in the nontapetal fundus. These retinal findings progressed with time in two dogs after re‐examination. Visual impairment, especially under dim light conditions, was observed in the affected dogs. ERG from one affected dog showed profoundly reduced rod responses, whereas cone responses were better preserved. Microscopic changes in the eyes from three dogs were characterized by a severe diffuse predominantly outer retinal degeneration and atrophy. Re‐sequencing of the prcd‐gene for eight of the nine investigated dogs revealed that none of the individuals carried disease allele that has been associated with prcd‐PRA in other breeds. In conclusion, ophthalmoscopic, electroretinographic, and light‐microscopic alterations observed in nine Jämthund dogs were compatible with PRA. The prcd mutation was excluded as a cause of this retinopathy. 相似文献
15.
Tuddow Thaiwong Sarah Corner Stacey La Forge Matti Kiupel 《Journal of veterinary diagnostic investigation》2021,33(4):740
Canine pituitary dwarfism in German Shepherd and related dog breeds has been reported to be associated with a 7-bp deletion mutation in intron 5 of the LHX3 gene. This mutation is transmitted as an autosomal recessive trait that results in dwarf dogs with significantly smaller stature and abnormal haircoat, and potentially early death. Phenotypically, affected adult dogs are proportionally dwarfs. These dwarfs also have a soft, woolly puppy coat that fails to transition into the typical adult hair coat, and marked hair loss occurs in some dogs. We report a similar manifestation of dwarfism in Tibetan Terriers with the same LHX3 mutation. Dwarf Tibetan Terrier puppies were born physically normal but failed to gain weight or to grow at the same rate as their normal littermates. The 7-bp deletion mutation of the LHX3 gene was identified in both alleles of 3 Tibetan Terrier dwarfs from 3 litters, which were biologically related. All parents of these dogs are carriers, confirming transmission of dwarfism in an autosomal recessive manner. Recognition and detection of this mutation will help in guiding future breeding plans to eventually eliminate this trait from Tibetan Terriers. 相似文献
16.
Five Devon cattle with suspected ceroid-lipofuscinosis and aged between 19 and 39 months of age were humanely slaughtered and subjected to post-mortem examination. There was severe atrophy of the cerebrum, particularly of the occipital cortex. Microscopy also showed severe atrophy of the retina with complete loss of photoreceptor cells, even in the youngest animal examined. Histopathologically the disease was characterised by accumulation of a fluorescent lipopigment in neurones, including those of the retina and a severe astrocytosis. The disease, which is characterised by the accumulation of subunit c of mitochondrial ATP synthase, is similar to that extensively described in South Hampshire sheep except that the retinal lesions were more severe. In contrast, tremors were not noted in the cattle. The clinical history and similarity to the disease in sheep and other species indicated inheritance was as an autosomal recessive trait. 相似文献
17.
The effect of copper on thiobarbituric acid (TBA) reaction values, an index of lipid peroxidation, was examined in Bedlington Terriers, healthy dogs, and rats. High hepatic concentrations of copper appeared to lower TBA values in the inherited, chronic, progressive hepatic degeneration of Bedlington Terriers, a disease associated with copper toxicosis. The suspected TBA inhibition was confirmed when Cu2+ was added to homogenates of healthy dog or rat liver or a malondialdehyde standard. The amount of copper added approximated that in diseased Bedlington Terriers. Because of the interference by copper, the TBA test was judged to be an inappropriate test for the evaluation of lipid peroxidation in samples containing high copper concentrations such as those in diseased Bedlington Terriers. 相似文献
18.
OBJECTIVES: To identify the prevalence of Borzoi chorioretinopathy in western Canada, characterize lesions with fluorescein angiography, determine if lesions were progressive, clarify the association of progressive retinal atrophy and investigate the etiology. MATERIALS AND METHODS: Serial ophthalmic examination, fundus photography, electroretinography, and fluorescein angiography were used to evaluate Borzoi dogs with lesions of Borzoi chorioretinopathy. Pedigree analysis and test breeding of two affected dogs were completed to determine the heritability of Borzoi chorioretinopathy. RESULTS: One hundred three Borzoi dogs were examined between 1998 and 2003. Focal, peripheral, tapetal, hyper-reflective and pigmented areas consistent with focal retinal degeneration and RPE pigmentation were identified in 12 dogs between 7 months and 7 years of age. Seven males and five female dogs were affected. Ophthalmoscopy and fundus photography over 5 years revealed individual lesions that did not progress or coalesce in 12 affected dogs. Electroretinography of affected and normal Borzoi dogs confirmed that retinal function was similar in normal and affected dogs up to 7 years of age. Fluorescein angiography was performed in three affected dogs and confirmed intact blood-ocular barriers, focal retinal pigment epithelium hypertrophy, and focal absence of choroiocapillaris corresponding to chronic, focal lesions. Pedigree analysis precluded simple dominant, X-linked dominant, or X-linked recessive inheritance. One male dog from the test-bred litter developed bilateral lesions at 14 months of age. Simple recessive, polygenetic, and acquired etiologies of these lesions cannot be ruled out at this time. CONCLUSIONS: Borzoi chorioretinopathy is an acquired condition that initially manifests as focal retinal edema and loss of choriocapillaris and tapetum. With time the retina degenerates becoming hyper-reflective and with RPE hyper-pigmentation and clumping within the borders of the tapetal lesions. Choriocapillaris remains hypofluorescent on fluorescein angiography. Progressive retinal atrophy was excluded as an etiology of multifocal chorioretinopathy in Borzois dogs. This condition is not inherited by simple autosomal dominant or sex-linked modes of inheritance. 相似文献
19.
A.E. Vanhaesebrouck I. Van Soens L. Poncelet L. Duchateau S. Bhatti I. Polis S. Diels L. Van Ham 《Journal of veterinary internal medicine / American College of Veterinary Internal Medicine》2010,24(4):882-889
Background: Generalized myokymia and neuromyotonia (M/NM) in Jack Russell Terriers (JRTs) is related to peripheral nerve hyperexcitability syndrome in humans, a symptom complex resulting from diverse etiologies. Objective: Clinical and electrodiagnostic evaluation is used to narrow the list of possible etiological diagnoses in JRTs with M/NM. Animals: Nine healthy JRTs and 8 affected JRTs. Methods: A prospective study was conducted comparing clinical and electrophysiological characteristics in 8 JRTs affected by M/NM with 9 healthy JRT controls. Results: All affected dogs except 1 had clinical signs typical of hereditary ataxia (HA). In 6 dogs, neuromyotonic discharges were recorded during electromyogram. Motor nerve conduction studies showed an axonal neuropathy in only 1 affected dog. Compared with controls, brainstem auditory‐evoked potentials (BAEP) showed prolonged latencies (P < .05) accompanied by the disappearance of wave components in 3 dogs. Onset latencies of tibial sensory‐evoked potentials (SEP) recorded at the lumbar intervertebral level were delayed in the affected group (P < .001). The BAEP and SEP results of the only neuromyotonic dog without ataxia were normal. Conclusions and Clinical Importance: The BAEP and spinal SEP abnormalities observed in JRTs with M/NM were associated with the presence of HA. Therefore, these electrophysiological findings presumably arise from the neurodegenerative changes characterizing HA and do not directly elucidate the pathogenesis of M/NM. An underlying neuronal ion channel dysfunction is thought to be the cause of M/NM in JRTs. 相似文献
20.
Advanced diffuse bilateral retinal degeneration was identified in a 13-week old female domestic short-haired kitten. The animal was in good physical health but had steady deterioration in vision over a period of a month. Clinical signs were mydriasis, tapetal hyper-reflectivity, marked loss of retinal vasculature and almost total blindness. Histological examination of the retina showed degeneration of the photoreceptor and outer nuclear layers. The clinical and pathological features are similar to those described in inherited retinal degeneration in Persian kittens. 相似文献