Concept: Jack Russell Terrier
A CAPN1 missense mutation in Parson Russell Terrier dogs is associated with spinocerebellar ataxia. We now report that homozygous or heterozygous CAPN1-null mutations in humans result in cerebellar ataxia and limb spasticity in four independent pedigrees. Calpain-1 knockout (KO) mice also exhibit a mild form of ataxia due to abnormal cerebellar development, including enhanced neuronal apoptosis, decreased number of cerebellar granule cells, and altered synaptic transmission. Enhanced apoptosis is due to absence of calpain-1-mediated cleavage of PH domain and leucine-rich repeat protein phosphatase 1 (PHLPP1), which results in inhibition of the Akt pro-survival pathway in developing granule cells. Injection of neonatal mice with the indirect Akt activator, bisperoxovanadium, or crossing calpain-1 KO mice with PHLPP1 KO mice prevented increased postnatal cerebellar granule cell apoptosis and restored granule cell density and motor coordination in adult mice. Thus, mutations in CAPN1 are an additional cause of ataxia in mammals, including humans.
A two-year-old Jack Russell terrier was presented for evaluation of chronic cough and exercise intolerance. Previous treatment with antibiotics and glucocorticoids had only partially ameliorated the clinical signs. During investigation, hypoxaemia, peripheral eosinophilia and an eosinophilic bronchoalveolar lavage fluid were noted. Thoracic radiographs revealed two ovoid clearly delineated soft-tissue opacities, one in the caudal segment of the left cranial lung lobe (diameter 26 mm) and the other in the right cranial lung lobe (diameter 20 mm). These findings were verified by computed tomography, which identified an additional smaller lesion (diameter 16 mm) dorsally in the right caudal lobe. Ultrasound-guided fine-needle aspiration samples confirmed the diagnosis of eosinophilic pulmonary granulomatosis and treatment with prednisolone and azathioprine was initiated. Within 1 month, granulomas were no longer detectable radiographically. All medication was discontinued after 7 months and currently, after 2·5 years, the dog remains free of clinical signs. To the authors' knowledge this is the first case report to describe prolonged remission from idiopathic canine eosinophilic pulmonary granulomatosis.
The age associated decline in immune function is preceded in mammals by a reduction in thymic output. Furthermore, there is increasing evidence of a link between immune competence and lifespan. One approach to determining thymic output is to quantify signal joint T cell receptor excision circles (sj-TRECs), a method which has been developed and used in several mammalian species. Life expectancy and the rate of aging vary in dogs depending upon their breed. In this study, we quantified sj-TRECs in blood samples from dogs of selected breeds to determine whether there was a relationship between longevity and thymic output. In Labrador retrievers, a breed with a median expected lifespan of 11 years, there was an age-associated decline in sj-TREC values, with the greatest decline occurring before 5 years of age, but with sj-TREC still detectable in some geriatric animals, over 13 years of age. In large short-lived breeds (Burnese mountain dogs, Great Danes and Dogue de Bordeaux), the decline in sj-TREC values began earlier in life, compared with small long-lived breeds (Jack Russell terriers and Yorkshire terriers), and the presence of animals with undetectable sj-TRECs occurred at a younger age in the short-lived breeds. The study findings suggest that age-associated changes in canine sj-TRECs are related to breed differences in longevity, and this research highlights the use of dogs as a potential model of immunosenescence.
The aims of this observational, analytical, retrospective study were to (i) obtain computed tomographic (CT) cricoid dimensions (height, width, and transverse-sectional area), (ii) compare the cricoid dimensions between brachycephalic and mesaticephalic breeds, and (iii) compare cricoid cartilage dimensions between dogs without and affected with brachycephalic airway syndrome. The study is important to help to further evaluate and understand the anatomical components of brachycephalic airway syndrome. Measurements were performed in 147 brachycephalic and 59 mesaticephalic dogs. The cricoid cartilage was found to be significantly more oval in Pugs and French Bulldogs compared to mesaticephalic breeds. The cricoid cartilage transverse-sectional area was smallest for the Pug and, after adjusting for weight, significantly smaller for Pugs (P < 0.001), Boston Terriers (P = 0.001), and French Bulldogs (P < 0.001) compared to Jack Russell Terriers. The tracheal transverse-sectional area at C4 of English Bulldogs was significantly smaller than for Jack Russell Terriers (P = 0.005) and Labradors (P < 0.001). The cricoid cartilage transverse-sectional area:weight ratio was significantly lower in brachycephalic breeds compared to mesaticephalic breeds (P < 0.001). The cricoid cartilage:trachea at C4 transverse-sectional area for brachycephalic dogs was significantly larger than for mesaticephalic dogs (<0.001), demonstrating that the trachea was the narrowest part of the airway. No significant differences were found for cricoid dimensions between dogs affected with and without brachycephalic airway syndrome. However, large individual variation was found among the brachycephalic breeds and further studies investigating the relationship between cricoid cartilage size, laryngeal collapse, concurrent tracheal hypoplasia, and/or severity of brachycephalic airway syndrome are warranted.
Anatomic variations in skull morphology have been previously described for brachycephalic dogs; however there is little published information on interbreed variations in tympanic bulla morphology. This retrospective observational study aimed to (1) provide detailed descriptions of the computed tomographic (CT) morphology of tympanic bullae in a sample of dogs representing four brachycephalic breeds (Pugs, French Bulldogs, English Bulldog, and Cavalier King Charles Spaniels) versus two mesaticephalic breeds (Labrador retrievers and Jack Russell Terriers); and (2) test associations between tympanic bulla morphology and presence of middle ear effusion. Archived head CT scans for the above dog breeds were retrieved and a single observer measured tympanic bulla shape (width:height ratio), wall thickness, position relative to the temporomandibular joint, and relative volume (volume:body weight ratio). A total of 127 dogs were sampled. Cavalier King Charles Spaniels had significantly flatter tympanic bullae (greater width:height ratios) versus Pugs, English Bulldogs, Labrador retrievers, and Jack Russell terriers. French Bulldogs and Pugs had significantly more overlap between tympanic bullae and temporomandibular joints versus other breeds. All brachycephalic breeds had significantly lower tympanic bulla volume:weight ratios versus Labrador retrievers. Soft tissue attenuating material (middle ear effusion) was present in the middle ear of 48/100 (48%) of brachycephalic breeds, but no significant association was found between tympanic bulla CT measurements and presence of this material. Findings indicated that there are significant interbreed variations in tympanic bulla morphology, however no significant relationship between tympanic bulla morphology and presence of middle ear effusion could be identified.
A 7-year-old Jack Russell Terrier with a history of minor trauma was presented for lameness of the left forelimb. Radiography and computed tomography demonstrated a localized radioulnar osteolytic lesion with cortical bone loss and enthesiophytes. Based on results of diagnostic imaging and histopathological examination, the final diagnosis was radioulnar ischemic necrosis (RUIN), complicated by pathologic fracture. A rare disorder of unknown etiology, RUIN may be secondary to tearing of the interosseous ligament and potential ischemia. It should be differentiated from neoplastic or fungal disease. To the authors´ knowledge, this is the first canine case report describing RUIN.
Spinocerebellar ataxia also referred to as hereditary ataxia comprises different forms of progressive neurodegenerative diseases. A complex mode of inheritance was most likely in Parson Russell Terriers (PRT) and in Jack Russell Terriers (JRT). Recently, the missense mutation KCNJ10:c.627C > G was shown to be associated with the spinocerebellar ataxia (SCA) in JRT and related Russell group of terriers, whereas the missense mutation CAPN1:c.344G > A was associated with late onset ataxia (LOA) in PRT.
A post hoc analysis of the metabolizable energy (ME) intake of privately owned pet dogs from the authors' nutrition consultation practice (Years 2007-2011) was carried out to identify if current ME recommendations are suitable for pet dogs. Data on 586 adult dogs were available (median age 5.5, median deviation from ideal weight 0.0), 55 of them were healthy; the others had various diseases. For ration calculation, a standardized questionnaire and the software diet-check Munich(™) was used. ME was predicted according to NRC (2006). Data were evaluated for the factors disease, breed, size, age, gender and type of feeding. The mean ME intake of all adult dogs amounted to 0.410 ± 0.121 MJ/kg metabolic body weight (BW(0.75) ) (n = 586). There was no effect of size and disease. Overweight dogs ate 0.360 ± 0.121 MJ/kg BW(0.75) , and underweight dogs ate 0.494 ± 0.159 MJ/kg BW(0.75) . Older dogs (>7 years, n = 149, 0.389 ± 0.105 MJ/kg BW(0.75) ) had a lower ME intake than younger ones (n = 313, 0.419 ± 0.121 MJ/kg BW(0.75) ), and intact males had a higher ME intake than the others (p < 0.001). Some breeds were above average: Jack Russell Terrier, Dalmatian, small Munsterlander and Magyar Viszla, Bearded Collies, Sight Hounds, German Boxers, English foxhounds, Rhodesian Ridgebacks and Flat-Coated Retrievers with a mean ME intake of 0.473 ± 0.121 MJ/kg BW(0.75) . The following breeds were below average: Dachshunds, Bichons, West highland White Terrier, Collies except Bearded Collies, Airedale Terriers, American Staffordshire terriers and Golden Retrievers with a mean ME intake of 0.343 ± 0.096 MJ/kg BW(0.75) . The mean maintenance energy requirements of pet dogs are similar to that of kennel dogs which do not exercise very much. These results suggest that opportunity and stimulus to exercise provided for pet dogs are lower than for kennel dogs. Lower activity in pet dogs may reduce part of potential effects of breed, medical history and age groups.
Delineation of the typical disease progression in canine paroxysmal dyskinesia (PD) may assist in evaluating therapeutic agents during clinical trials. Our objective was to establish the natural disease course in a group of dogs diagnosed with PD that received no medication. Fifty-nine dogs (36 Labradors, 23 JRTs) with clinically confirmed PD and a follow-up of ≥3 years were retrospectively reviewed. Dogs with PD had a young onset, were triggered by startle or sudden movements, and had a male bias (75%) with the majority being entire sample population. Twenty-one dogs (36%) had at least one event comprising cluster episodes. Episode duration and frequency varied dramatically, even within an individual. Median follow-up was 7 years. No concurrent disease was identified in any dog that was investigated. The natural history was self-limiting with 32% entering remission and an improvement in 75%. Episodes reduced in terms of frequency and duration in Labradors and JRTs respectively. Remission was lower in dogs with cluster episodes than those without. These findings suggest that the diagnostic yield of advanced neuroimaging techniques in dogs with video footage and historical data supporting PD, without neurological deficits, is low. The presence of cluster episodes is of predictive value for the prognosis of canine PD. Future research should be cautious in reporting treatment response for PD without first considering the spontaneous remission rate and improvements in untreated dogs documented in this study.
Myasthenia gravis (MG) is a disorder of neuromuscular transmission in which muscle weakness results from an autoantibody mediated depletion of acetylcholine receptors (AChRs) at the neuromuscular junction. Myasthenia gravis occurs spontaneously in dogs and cats, and as in human MG, an autoimmune response against nicotinic AChRs has been demonstrated and autoantibodies against AChRs implicated in the pathogenesis. While both species are affected with MG, there are distinct differences in clinical presentations and frequency of spontaneous remission. Congenital myasthenic syndromes (CMSs) are hereditary disorders of neuromuscular transmission resulting in structural or functional defects of the neuromuscular junction. The clinical presentation and pathogenesis of a CMS in Jack Russell terriers was first described in the 1970’s and 1980s and has since been reported in a few other breeds. Mutations have been reported in CHRNE, COLQ and CHAT in canine CMS. A form of COLQ deficient CMS has recently been reported in cats.