Myokymia and neuromyotonia in 37 Jack Russell terriers

The clinical and clinicopathological characteristics, treatment and outcome of vermicular muscle contractions (myokymia) and generalized muscle stiffness (neuromyotonia) in 37 Jack Russell terriers were evaluated retrospectively. Thirty dogs were affected by both disorders, whereas seven were presented with myokymia and never developed neuromyotonia. Clinical signs started at the mean age of 8 months. Except for signs of myokymia and neuromyotonia, clinical and neurological examination was normal in all dogs. Thirty dogs demonstrated typical signs of hereditary ataxia.Changes in serum chemistry included increased creatine kinase, aspartate aminotransferase and alanine aminotransferase concentrations. Electromyographic abnormalities, especially in muscles showing macroscopically visible myokymia, consisted of semirhythmic bursts of doublet, triplet, or multiplet discharges of a single motor unit. The amplitudes varied between 80 μV and 1 mV and occurred with an interburst frequency between 10 and 40 Hz and an intraburst frequency between 150 and 280 Hz.Most dogs were treated with a sodium channel blocker with variable results. Seven dogs died (most likely because of hyperthermia) or were euthanased during a neuromyotonic attack; 15 dogs were euthanased due to worsening of clinical signs, or lack of or no long-lasting effect of medication, and three were euthanased for unknown or unrelated reasons. Nine dogs were lost to follow-up and three were still alive 5–10.5 years after the start of clinical signs. In conclusion, young Jack Russell terriers with myokymia and neuromyotonia should undergo a complete blood and electrophysiological examination. Long-term prognosis is not favourable.     

Congenital deafness in Jack Russell terriers: Prevalence and association with phenotype

Congenital hereditary sensorineural deafness is the most common form of deafness in dogs. The objectives of this study were to determine a reliable measure of the prevalence of deafness in Jack Russell terriers, an affected breed, and associations between deafness and phenotypic characteristics. Brainstem auditory evoked response recordings and phenotypic parameters (coat color, coat texture, sex, eye color, sire and dam hearing status) were recorded for 1009 Jack Russell terriers. The prevalence of unilateral and bilateral deafness was 3.57% and 0.50%, respectively, lower by a factor of three to four than in earlier reports based on smaller and closely related kindreds. Significant association with deafness was identified with white coat color and parental hearing status, but not with sex or coat type. Lack of significant sex or coat type associations and the significant association with white coat color are consistent with previous reports. In conclusion the prevalence of deafness in Jack Russell terriers is lower than initially reported. Deafness was associated with white coat color and parental hearing status. The association with parental hearing status supports this form of deafness being a heritable trait in the breed and the association with white coat color supports an inheritance linked to pigmentation genes.     

A KCNJ10 mutation previously identified in the Russell group of terriers also occurs in Smooth-Haired Fox Terriers with hereditary ataxia and in related breeds

Background

Hereditary ataxias with similar phenotypes were reported in the Smooth-Haired Fox Terrier, the Jack Russell Terrier and the Parson Russell Terrier. However, segregation analyses showed differing inheritance modes in these breeds. Recently, molecular genetic studies on the Russell group of terriers found independent mutations in KCNJ10 and CAPN1, each associated with a specific clinical subtype of inherited ataxia. The aim of this study was to clarify whether or not Smooth-Haired Fox Terriers with hereditary ataxia and dogs of other related breeds harbor either of the same mutations. A sub goal was to update the results of KCNJ10 genotyping in Russell group terriers.

Findings

Three Smooth-Haired Fox Terriers with hereditary ataxia and two Toy Fox Terriers with a similar phenotype were all homozygous for the KCNJ10 mutation. The same mutation was also found in a heterozygous state in clinically unaffected Tenterfield Terriers (n = 5) and, in agreement with previous studies, in Jack Russell Terriers, Parson Russell Terriers, and Russell Terriers.

Conclusions

A KCNJ10 mutation, previously associated with an autosomal recessive spinocerebellar ataxia in Jack Russell Terriers, Parson Russell Terriers, and Russell Terriers segregates in at least three more breeds descended from British hunting terriers. Ataxic members of two of these breeds, the Smooth-Haired Fox Terrier and the Toy Fox Terrier, were homozygous for the mutation, strengthening the likelihood that this genetic defect is indeed the causative mutation for the disease known as “hereditary ataxia” in Fox Terriers and “spinocerebellar ataxia with myokymia, seizures or both” in the Russell group of terriers.     

Gastrointestinal foreign bodies in dogs and cats: a retrospective study of 208 cases

O bjectives : To establish predilection sites of obstruction and to investigate clinical factors associated with a poor outcome.
M ethods : A retrospective study of 208 consecutive cases over a 48-month period from first-opinion practice.
R esults : Overall, 91 per cent of cases recovered with higher survival rates from discrete foreign bodies (94 per cent in dogs and 100 per cent in cats) as opposed to linear foreign bodies (80 per cent in dogs and 63 per cent in cats). English bull terriers, springer spaniels, Staffordshire bull terriers, Border collies and Jack Russell terriers were over-represented. In dogs, 63 per cent of obstructions occurred in the jejunum but foreign objects were encountered at all points along the gastrointestinal tract. A longer duration of clinical signs, the presence of a linear foreign body and multiple intestinal procedures were associated with significantly increased mortality. Neither the degree of obstruction (partial or complete) nor the location of the foreign body was shown to have a significant influence on survival.
C linical S ignificance : Prompt presentation, diagnosis and surgical intervention improve the outcome of gastrointestinal obstruction by foreign bodies. At surgery, the minimal number of intestinal procedures should be performed to restore the integrity of the alimentary tract.     

Normative auditory brainstem response data for hearing threshold and neuro-otological diagnosis in the dog

There Is growing Interest In the application of auditory brainstem response (ABR) audlometry for hearing assessment In dogs. The technique is far from standardised, however, resulting In large discrepancles between studies. This study aimed to obtain normative data, under clearly defined conditions, for two breeds of significantly different size; head size being a potential factor determining ABR latency values. The subjects, 20 daimatians and 20 Jack Russell terriers, were sedated prior to ABR testing, and subcutaneous scalp electrodes used to detect the evoked potential ellclted by a click stimulus presented via Insert earphones. The mean ABR thresholds for the two breeds, 0 and -5 decibels re normal hearing level (dB nHL), respectively, were very simllar to those for humans. The latency values of the main ABR waves and the Interval between them were statistically significantly smaller for the smaller breed, but there was no correlation with head size within either breed. The results provide a baseline to assist with confirmation of hearing Impalrment and neuro-otological diagnosis In the dog.     

Normative auditory brainstem response data for bone conduction in the dog

Auditory brainstem response (ABR) is a valuable tool for the diagnosis of hearing disorders in dogs, but is hampered by the lack of published normative data. The aim of the present study was to obtain normative data for bone conduction, without masking, under clearly defined conditions. Subjects comprised 20 Dalmatians and 20 Jack Russell terriers. Two methods were investigated: holding the bone vibrator against the head by hand or by applying a 500 g weight. The results revealed no difference in hearing threshold between the two breeds or for the two methods of applying the bone vibrator to the head. The mean hearing threshold was close to 0 decibels re normal hearing level (dB nHL), which is the biological norm for humans. Hence, bone conduction thresholds can be used for confirmation of conductive hearing impairment in the dog, in the same way as in humans.     

ADAMTS17 mutation associated with primary lens luxation is widespread among breeds

Primary lens luxation (PLL) is a well-recognized, painful and potentially blinding inherited ocular condition in dogs. We screened PLL-affected dogs of 30 different breeds, to identify those which carried a previously described c.1473+1 G>A mutation in ADAMTS17 that is associated with PLL in Miniature Bull terriers, Lancashire Heelers, and Jack Russell terriers. This ADAMTS17 mutation was identified in PLL-affected dogs from 14 additional breeds. PLL-affected dogs from some breeds (most notably the Shar pei and the Brittany spaniel) did not carry the G1473+1A ADAMTS17 mutation, indicating they must suffer from a genetically distinct form of the condition. We also estimated the frequency of this ADAMTS17 mutation in some of the breeds. Our findings indicate the mutation segregates in a large number of different breeds of dog, many of which are terriers or breeds with terrier co-ancestry, but some of which have more diverse origins. Our results also indicate that the mutation is present at high frequency within most of the breeds in which it segregates. In the miniature bull terrier breed estimates of mutation frequency ranged from 0.27 to 0.39, corresponding to 7.3-15.2% PLL-affected dogs in this breed. We also identified an increased risk of PLL associated with heterozygosity at ADAMTS17, suggesting that carriers carry a low risk of developing PLL.     

Characteristics of 234 dog bite incidents in Ireland during 2004 and 2005

Information was obtained by telephone interview from 100 dog owners whose dog had bitten a person, and from 134 victims of bites by a dog not owned by the victim. Three-quarters of the victims were female and aged from 21 to 60 years. The majority of the dogs were owned, male, two to six years old, over 10 kg in bodyweight and belonged to the popular breeds: collies, cocker/springer spaniels, terrier breeds, Jack Russell terriers, German shepherd dogs, golden retrievers and crossbreeds. The numbers of bites by the different breeds indicated that those that inflicted the most bites were the popular breeds rather than the breeds with any greater propensity to bite. Most attacks were rapid single bites and in 50 per cent of the cases, neither the owner nor the victim was able to identify any signal of the dog's intention to bite. Overall, 21 per cent of the incidents were rated as 'serious' and 2 per cent as 'life threatening'. One fifth of the dogs were euthanased as a result of the incident. Half the incidents required professional medical assistance for the victim. Almost half the incidents took place while the victim was walking or passing close to the dog's territory, or while the victim was interacting with the dog at home.     

Contrast-enhanced CT features of pyloric lesions in 17 dogs: Case series

Pyloric outflow obstructions can be caused by several types of lesions. When a thickened gastric wall and pyloric mass are detected, malignant neoplasia must be differentiated from chronic hypertrophic pyloric gastropathy. CT can characterize gastric tumors. However, based on the authors’ review of the literature, there is limited information about the CT findings of pyloric lesions. The purpose of this retrospective case series study was to assess the CT findings of canine pyloric lesions. The following CT parameters were recorded: anatomical area, involved area, lesion shape, growth patterns of wall thickening lesions, enhancement pattern of the lesion in the early and delayed phases, lymphomegaly, and pulmonary metastasis. Seventeen dogs were included in this study and had the following final diagnoses: hyperplasia (five dogs), adenoma (five dogs), adenocarcinoma (three dogs), gastrointestinal stromal tumor (GIST; two dogs), polyposis (one dog), and pyogenic granuloma (one dog). Hyperplasia, adenoma, and polyposis formed mass lesions that involved the mucosal layer. Lymphomegaly was detected in two Jack Russell terriers with hyperplasia; however, the causes were unknown because we did not perform biopsies. All adenocarcinomas formed wall-thickened lesion that involved the outer layer, with lymphomegaly. All GISTs formed mass lesion that involved the outer layer. The pyogenic granulomas formed symmetric wall-thickened lesion that involved the mucosal and outer layers. CT facilitated the characterization of canine pyloric lesions using contrast enhancement, based on the involved area and lesion shape. However, polyposis may require caution in diagnosis based on CT findings alone.     

Inheritance of cataracts and primary lens luxation in Jack Russell Terriers

OBJECTIVE: To characterize heritability and mode of inheritance of cataracts and primary lens luxation in Jack Russell Terriers. ANIMALS: 872 Jack Russell Terriers from which buccal epithelial cells were collected and phenotypes for cataracts and lens luxation were determined and an additional 1,898 Jack Russell Terriers without phenotypic information used to complete pedigree relationships and that were included in the analyses. PROCEDURES: Narrow-sense heritabilities and genetic correlation for cataracts and lens luxation were modeled by use of threshold analysis, whereas complex segregation analysis was used to characterize mode of inheritance. For the analyses, dogs < 6 years old, unless confirmed as having cataracts or lens luxation, were classified as an unknown phenotype. The possible involvement of an HSF4 mutation in cataracts was determined by DNA sequencing. RESULTS: Cataracts and primary lens luxation were highly heritable and genetically correlated, and neither was controlled by a single gene. Cataracts were not associated with an HSF4 mutation. CONCLUSIONS AND CLINICAL RELEVANCE: Analysis of the data indicated that concerted selection against both cataracts and primary lens luxation when choosing breeding animals can be used to improve ocular health in Jack Russell Terriers.     

Factor X deficiency in a Jack Russell terrier

Deficiency in factor X (Stuart-Prower factor) was identified in a 7-month-old spayed female Jack Russell Terrier following recurrent bleeding episodes. Various relatives were screened for factor X deficiency and low and subnormal levels were identified in the father and paternal grandmother, respectively. Factor X deficiency has been previously documented in a family of American Cocker Spaniels, in which the inheritance pattern appeared to be an autosomal dominant trait with variable expression. This is the first report describing this coagulopathy in the Jack Russell Terrier.     

Congenital dilatation of the bile ducts (Caroli's disease) in young dogs

We describe 8 young dogs with congenital dilatation of the intra- and extrahepatic bile ducts and diffuse cystic kidney disease, compatible with Caroli's disease in humans. The dogs were referred between 1980 and 2000 because of chronic disease at an age of 6 months to 3 years. These dogs included 3 Collies, 2 Frisian Stabyhouns, 2 Jack Russell Terriers, and 1 mixed-breed dog. The most common signs were vomiting (6/6), polyuria and polydipsia (4/6), and anorexia (4/6). Ascites was a common finding (4/6). Clinicopathologic abnormalities were available for 6 dogs. All had increased plasma alkaline phosphatase activity and fasting bile acids: increased alanine aminotransferase activity and urea and creatinine concentrations were present in 50% of dogs. Ultrasound examination of the liver showed severely dilated bile ducts without evidence of obstruction, and calcification in all cases but 1. Postmortem examination revealed severe dilatation of the larger intra- and extrahepatic bile ducts. The common bile duct and gall bladder were normal, and the bile system was patent. The ducts contained a clear viscid fluid often with calcified material. Microscopically, marked portal fibrosis was present, often with abnormally structured dilated bile ducts lined with columnar or cuboid epithelium and regularly small calcifications. The lesion was complicated by ascending cholangitis in 1 dog. The kidneys showed marked cortical and medullary fibrosis with a diffuse radial cystic pattern; only slight renal fibrosis was found in the oldest dog. Seven dogs were euthanized without treatment; the oldest dog was alive and well 5 months after diagnosis and was maintained on a protein-restricted diet.     

Variation in position of the medial fabella in West Highland white terriers and other dogs

O bjectives : To investigate whether West Highland white terriers exhibit significant variation in the position of the medial fabella compared to both small and large breed dogs.
M ethods : Criteria for the normal location of the medial fabella on a caudocranial radiograph were established. A retrospective analysis was undertaken of a consecutive series of bilateral caudocranial stifle joint radiographs from 70 West Highland white terriers, 100 small and 100 large control dogs by three reviewers. Medial fabellar location, cruciate ligament disease and medial patellar luxation were examined within and between groups.
R esults : Abnormal medial fabellar location was identified in 70 per cent, 9 per cent and 0 per cent of West Highland white terriers, small dogs and large dogs, respectively. In the vast majority of the affected dogs, the fabella was found in a mediodistal location. Presence of concurrent cranial cruciate ligament disease or medial patellar luxation and body weight was excluded as confounding factors.
C linical S ignificance : West Highland white terriers appear predisposed to, and have a high prevalence of, an abnormal mediodistal location of the medial fabella. The authors suggest this is an incidental finding and should not be confused with true pathological fabellar displacement.     

Plasma amino acid concentrations in 36 dogs with histologically confirmed superficial necrolytic dermatitis

Plasma amino acid concentrations were measured in 36 dogs diagnosed with superficial necrolytic dermatitis (SND) via skin biopsy. The median age of the dogs was 10 years, and 27 out of 36 (75%) were male. Twenty-two out of 36 (61%) of the dogs were accounted for by six breeds; West Highland white terriers (six), Shetland sheepdogs (five), cocker spaniels (four), Scottish terriers (three), Lhasa apsos (two) and Border collies (two). The mean concentration (+/- standard deviation) was calculated for each measured plasma amino acid and compared to previously documented concentrations of plasma amino acids measured in dogs with acute and chronic hepatitis. The ratio of branched chain amino acids to aromatic amino acids in the dogs with SND was 2.6, slightly lower than that in normal dogs. The mean plasma amino acid concentrations for dogs with SND were significantly lower than for dogs with acute and chronic hepatitis. A metabolic hepatopathy in which there is increased hepatic catabolism of amino acids is hypothesized to explain the hypoaminoacidaemia seen in SND.     

Case Report Cutaneous vasculitis in five Jack Russell Terriers

Abstract A new, possibly breed associated, vasculitis of Jack Russell Terriers is described. Lesions include alopecia and focal crusted ulcers of the distal extremities and bony prominences. Histopathological lesions include single cell necrosis of the epithelium, pigmentary incontinence, leucocytoclastic vasculitis and ischaemic degeneration of hair follicles. Dermal oedema and an infiltrate of lymphocytes and/or macrophages are often seen. Clinically and histopathologically the disease most closely resembles dermatomyositis of Collies and Shetland sheepdogs but the obvious breed discrepancy makes systemic lupus erythematosus the most likely differential diagnosis. Dapsone and anti-inflammatory doses of prednisone have proved to be satisfactory treatments. Résumé— Une nouvelle dermatose à prédisposition raciale, de type vascularite est décrite chez des jack russel terriers. Les lésions cliniques incluent une alopécie, des ulcèrations focales et croûteuses des extrémités distales et des points de pression. Les lésions histolopathologiques montrent des nécroses isolées des kératinocytes, une incontinence pigmentaire, une vascularite leucocytoclasique et une dégénérescence ischémique des follicules pileux. Un oedème dermique et une infiltration lymphocytaire et/ou macrophagique sont également observés. Sur les plans clinique et histopathologique, cette maladie ressemble plus aux dermatomyosites du Colley et du Shetland, mais la discordance raciale évidente fait du lupus érythémateux systémique le diagnostic différentiel le plus vraisemblable. La dapsone et la prednisone à dose antiinflammatoire sont des traitements satisfaisants. [Parker, W.M., Foster, R.A. Cutaneous vasculitis in five Jack Russell Terriers (Vascularite cutanée chez 5 jack russel terriers). Veterinary Dermatology 1996; 7 : 109–115.] Resumen Se describe una vasculitis nueva, posiblemente asociada a la raza en el terrier Jack Russell. Las lesiones incluyen alopecia y ulceraciones costrosas en las extremidades distales y en prominencias óseas. Las lesiones histopatológicas incluyen necrosis celular individual del epitelio, incontinencia pigmentaria, vasculitis leucocitoclástica y degeneración isquémica de los foliculos pilosos. Con frecuencia se observa edema dérmico y una infiltración por linfocitos y/o macrófagos. Este cuadro se asemeja clinica e histopatológicamente a la dermatomiositis de los perros Collie y Shetland pero la distancia obvia entre estas razas hace que el diagnóstico diferencial más probable sca el de lupus eritematoso sistémico. Se consiguieron tratamientos satisfactorios con Dapsona y dosis antiinflamatorias de prednisona. [Parker, W.M., Foster, R.A. Cutaneous vasculitis in five Jack Russell Terriers (Vasculitis cutánea en cinco Terriers Jack Russell). Veterinary Dermatology 1996; 7 : 109–115.] Zusammenfassung— Es wird eine neue, möglicherweise rasseabhängige Vaskulitis bei Jack Russell Terriern beschrieben. Die Veränderungen bestehen in Alopezie, fokalen verkrusteten Geschwüren der distalen Extremitäten und Knochevorsprünge. Histopathologische Veränderungen bestehen in Einezelzellnekrose des Epithels, Pigmentinkontinenz, leukozytoklastischer Vaskulitis und ischämischer Degeneration der Haarfollikel. Dermales ödem und Lymphozyten- und/oder Makrophageninfiltrate werden häufig beobachtet. Klinisch und histopathologisch ähnelt die Erkrankung sehr der Dermatomyositis von Collie und Sheltie, aber die offensichtliche Rassendiskrepanz läßt den systemischen Lupus erythematosus als wahrscheinlichste Differentialdiagnose erscheinen. Dapson und entzündungshemmende Dosen von Prednison zeigten sich als ausreichende Behandlung. [Parker, W. M., Foster, R. A. Cutaneous vasculitis in five Jack Russell Terriers (Kutane Vaskulitis bei fünf Jack Russell Terriern). Veterinary Dermatology 1996; 7 : 109–115.]     

Proteinuria as an indicator of early renal disease in bull terriers with hereditary nephritis

A group of non-azotaemic bull terriers from families with hereditary nephritis had significant subclinical renal disease. Of the renal function tests carried out, proteinuria, almost exclusively albumin, was a reliable and early indicator of glomerular abnormality. While 24-hour urinary protein excretion (24 UPE) in the bull terriers was significantly higher than in a group of normal dogs of other breeds, urinary protein excretion (UPE) was also significantly higher when measured by simple single sample tests such as the urinary protein to urinary creatinine ratio (UP/C). UPE in bull terriers was correlated with increasing age, in contrast to lack of correlation in the normal dogs. The degree of proteinuria in affected bull terriers from which renal biopsies were taken correlated with the severity of histopathological changes which mainly affected glomeruli. Light microscopic examination revealed segmentally thickened glomerular and tubular basement membranes, thickened Bowman's capsules and adhesions between glomerular capillaries and Bowman's capsules. Other renal function tests were performed but failed to detect subclinical disease. It is suggested that hereditary nephritis (HN) in bull terriers is similar to that seen in samoyeds, dober-mann pinschers and humans.     

Study of 253 dogs in the United Kingdom with diabetes mellitus

Clinical information and blood samples were collected from 253 dogs with naturally occurring diabetes mellitus. Over half of them were labrador retrievers, collies, Yorkshire terriers or crossbred dogs, and approximately 80 per cent of them were diagnosed between the ages of five and 12 years. The majority of the dogs were receiving insulin therapy once a day, but in the dogs receiving insulin injections twice a day there was a trend for lower serum fructosamine concentrations, suggesting better glycaemic control. The proportion of female dogs with diabetes was lower than in previous surveys. The disease was diagnosed more commonly in the winter months, a seasonal pattern also observed in human beings with diabetes, suggesting that similar environmental factors might be involved in the disease.     

Autoimmune form of myasthenia gravis in a juvenile Yorkshire Terrier x Jack Russell Terrier hybrid contrasted with congenital (non-autoimmune) myasthenia gravis of the Jack Russell

A case of juvenile onset myasthenia gravis is described in a Yorkshire Terrier x Jack Russell bitch in which there were serum autoantibodies to acetylcholine receptors and reduction in the muscle's content of acetylcholine receptors. Approximately 25% of the receptors remaining were complexed with antibody. After 8 months of treatment with the anticholinesterase pyridostigmin, the animal made a complete recovery and the serum level of antibody decreased. The condition of this dog in many respects resembled acquired myasthenia gravis of adult onset and is contrasted with the seven cases of congenital myasthenia gravis which we have studied in the Jack Russell Terrier. In the congenital form of myasthenia gravis, reduction of acetylcholine receptors in muscle is found without any demonstrable autoantibodies to acetylcholine receptors, either in serum or muscle.