Chiari-like malformation and syringomyelia in normal cavalier King Charles spaniels: a multiple diagnostic imaging approach

Objectives : The objectives of the study were (1) to report the incidence of Chiari‐like malformation and syringomyelia in a normal French cavalier King Charles spaniel breeding population; (2) to precise the standard computed tomography dimensions of the caudal fossa and (3) to investigate the use of ultrasonography in diagnosis of this syndrome. Methods : Clinically normal adult cavalier King Charles spaniel underwent ultrasonographic examination of the spinal cord and caudal fossa. Computed tomography was used to measure the caudal fossa and magnetic resonance imaging allowed syringomyelia and cerebellar herniation identification. Results : Of the 16 dogs in the study, seven had syringomyelia (43·7 per cent). All dogs had cerebellar herniation, suggesting Chiari‐like malformation and also a tendency to occipital dysplasia. Computed tomography measurements of the caudal fossa are reported. In one dog, a syrinx was identified by ultrasonography. The only difference between dogs with or without syringomyelia was that dogs with Chiari‐like malformation/syringomyelia were statistically older. Clinical Significance : The incidence of Chiari‐like malformation and syringomyelia may be high in an asymptomatic population of cavalier King Charles spaniel . Computed tomography measurements reported in this study should now be compared with those of a symptomatic population to evaluate the hypothesis that dogs with Chiari‐like malformation/syringomyelia syndrome have a smaller caudal fossa. This study did not identify a smaller caudal fossa in an asymptomatic cavalier King Charles spaniel population with syringomyelia. Ultrasonography probably has a low sensitivity for diagnosis of Chiari‐like malformation/syringomyelia.     

Comparison of the relative occipital bone volume between Cavalier King Charles spaniels with and without syringohydromyelia and French bulldogs

Our aim was to determine the relative volume of the occipital bone of Cavalier King Charles spaniels with and without syringohydromyelia and normal French bulldogs to reappraise the role of a possible insufficiency of the paraxial mesoderm in the pathogenesis of the caudal occipital malformation syndrome. Analysis of the occipital bone volume of 43 dogs based on computed tomography datasets was performed. Volume was determined by means of three-dimensional models. Using manual segmentation of the occipital bone in sagittal, transverse, and dorsal images, the volume of the occipital bone and the rest of the skull was calculated. The absolute occipital bone volume was put in relation to the total skull volume, the occipital bone index. For the Cavalier King Charles spaniels without syringomyelia, the median occipital bone index was 0.0681; for Cavalier King Charles spaniels with syringomyelia, it was 0.0646 and for French bulldogs, it was 0.0676. There was no global difference of the occipital bone index between examined groups (P = 0.4331). A reduced volume of the occipital bone was not found in Cavalier King Charles spaniels in general in comparison to French bulldogs, or in Cavalier King Charles spaniels with syringomyelia compared to Cavalier King Charles spaniels without syringomyelia. These results do not support occipital hypoplasia as a cause for syringomyelia development, challenging the paraxial mesoderm insufficiency theory. This also suggests that the term Chiari-like malformation, a term derived from human studies, is not appropriate in the Cavalier King Charles spaniel.     

Inheritance of occipital bone hypoplasia (Chiari type I malformation) in Cavalier King Charles Spaniels

Occipital bone hypoplasia with foramen magnum obstruction and secondary syringomyelia (SM) is a common condition in the Cavalier King Charles Spaniel (CKCS) that is similar to human Chiari type I malformation. A worldwide family tree of more than 5,500 CKCSs spanning a maximum of 24 generations was established by obtaining pedigree information from 120 dogs diagnosed with SM secondary to occipital bone hypoplasia. The ongoing study showed 6 of 8 great grandparents of all affected dogs could be traced back to 2 female ancestors so that all 8 were descended from one or the other or both. The disease appears to be more severe and have an earlier onset with increased inbreeding, especially when breeding from affected dogs. The family tree of idiopathic epilepsy (IE) appears to be a different subset of the CKCS population, although some overlap was observed. Idiopathic epilepsy is more frequent in lines originating from whole-color dogs. Selection for coat color is believed to have influenced the development of both occipital hypoplasia with secondary SM and IE. In addition, breeding guidelines to reduce the incidence of mitral valve disease have placed further pressures on the gene pool.     

Association between cervical and intracranial dimensions and syringomyelia in the cavalier King Charles spaniel

O bjectives : To investigate the possible association between caudal fossa area and cervical vertebral dimensions and the presence of syringomyelia in cavalier King Charles spaniels.
M ethods : From magnetic resonance imaging scans of 78 cavalier King Charles spaniels, measurements were made of the widest vertical spinal width at C1/C2, C2, C2/C3 and C3; angulation of the C2/C3 spine; and estimated caudal fossa area. A correlation between these measurements and syringomyelia was sought.
R esults : A total of 59 dogs with and 19 without syringomyelia were compared. Older dogs had a significantly higher incidence of syringo-myelia. No difference in incidence was noted between genders. There was no significant difference in vertebral canal width at C1/C2 and C2, or angulation of C2/C3 between syringomyelia and non-syringomyelia groups. The width of the canal at C2/C3 and C3 was significantly increased in syringomyelia dogs. There was no significant difference in the caudal fossa area between groups.
C linical S ignificance : Although syringomyelia was shown to be more prevalent in older dogs, the age beyond which dogs were considered at greater risk was not deducible from the dataset. The association identified between wider spinal canal at C3, and C2/C3 and syringomyelia presence is of questionable clinical significance, as the difference between syringomyelia and non-syringomyelia groups is too small to be measured in a clinical setting.     

Syringomyelia in cavalier King Charles spaniels: the relationship between syrinx dimensions and pain

OBJECTIVES: This study was designed to test the hypothesis that pain associated with syringomyelia in dogs is dependent upon size and involvement of the dorsal part of the spinal cord. METHODS: Masked observers determined syrinx dimensions and precise location within the spinal cord on magnetic resonance images of 55 cavalier King Charles spaniels with syringomyelia. After removal of masking, syrinx size and location were compared between the cohorts of dogs that exhibited pain with those that did not. RESULTS: Maximum syrinx width was the strongest predictor of pain, scratching behaviour and scoliosis in dogs with syringomyelia. Both pain and syrinx size were positively correlated with syrinxes located in the dorsal half of the spinal cord. CLINICAL SIGNIFICANCE: Large syrinxes associated with damage to the dorsal part of the spinal cord are associated with persistent pain suggesting that the pain behaviour expressed by this group of patients is likely to be "neuropathic pain," resulting from disordered neural processing in the damaged dorsal horn. As such it is likely that conventional analgesic medication may be ineffective.     

Chronic valvular disease in the cavalier King Charles spaniel in Sweden.

The prevalence of chronic valvular disease was studied in 494 cavalier King Charles spaniels with a mean (+/- sd) age of 3.0 +/- 2.7 years. Cardiac murmurs were detected in 65 (13.2 per cent) of the dogs. Among 61 cavalier King Charles spaniels with a mean age of 6.4 +/- 2.8 years, cardiac murmurs were detected in 32 (52 per cent). In both groups of dogs the prevalence of cardiac murmurs was low among dogs younger than three years (1.9 per cent) but increased with age (P < 0.001). The estimated ages at which 50 per cent of the dogs had developed murmurs were 7.5 and 6.2 years, respectively. When 39 of the 61 dogs were re-examined three years later, cardiac murmurs were detected in 28 (72 per cent), and the intensities of the murmurs had generally increased (P < 0.05). Nine (28 per cent) of the dogs which had previously had murmurs had been euthanased for signs of congestive heart failure whereas none of the dogs which had been free of murmurs had died from congestive heart failure. Animal insurance statistics from 1982 to 1990 (1983 excluded) for dogs less than 10 years old showed that claims for veterinary care or death or euthanasia were five times more common in the cavalier King Charles spaniel than in dachshunds (P < 0.001) and eight times more common than the mean for all other insured breeds (P < 0.001).     

Coexistence of occipital dysplasia and occipital hypoplasia/syringomyelia in the cavalier King Charles spaniel

Concurrent occurrence of occipital dysplasia and occipital hypoplasia in two dogs is described in this report. Occipital hypoplasia results in reduced volume of the caudal fossa, leading to overcrowding of the neural structures and, in severe cases, development of syringomyelia. In occipital dysplasia, there is a failure of complete ossification of the supraoccipital bone. When the two conditions occur concurrently, it is possible that syringomyelia may develop more slowly, resulting in presentation with clinical signs in middle to old age. This has implications for screening tests for early detection of syringomyelia, with a view to using the dog for breeding purposes, as dogs with an apparently mild phenotype for occipital hypoplasia/syringomyelia may actually have a more severe genotype.     

MORPHOLOGY OF THE CAUDAL FOSSA IN CAVALIER KING CHARLES SPANIELS

Chiari malformations and syringohydromyelia are an important disease complex in Cavalier King Charles Spaniels. Although abnormalities in caudal fossa morphology are considered major contributors to the development of this disease, limited information exists on the range of morphologies in Cavalier King Charles Spaniels and on the relationship of these to clinically evident disease. Sixty-four Cavalier King Charles Spaniels were studied. Each underwent a neurologic examination and magnetic resonance imaging of the cervical spine and brain. T2-weighted sagittal images were used to determine both the morphologic characteristics and volume of the caudal fossa in each dog. This volume was also analyzed as a percentage of total cranial cavity volume. Each attribute was correlated with neurological grade and presence of syringohydromyelia. Fifteen dogs had neurologic signs, and 59 had morphologic abnormalities of the craniocervical junction. While 27 dogs had syringohydromyelia, 13 of these were clinically normal. Cerebellar herniation and occipital dysplasia were common findings but were not associated with syringohydromyelia. Dorsal compressive lesions were noted at the first and second cervical vertebral junction. Factors associated with the presence of neurologic signs included syringohydromyelia and the ratio of caudal fossa/total cranial cavity volume; dogs with signs had significantly larger syringohydromyelia than asymptomatic dogs. Caudal fossa size was not associated with syringohydromyelia. A positive association was identified between foramen magnum size and length of cerebellar herniation. The prevalence of craniocervical junction abnormalities is high in Cavalier King Charles Spaniels. While several factors are associated with neurologic signs, occipital hypoplasia appears to be the most important factor.     

CT findings in Pneumocystis carinii pneumonia in five dogs

Pneumocystis carinii pneumonia is a rare disease in dogs. It is primarily reported in cavalier King Charles spaniels and miniature dachshunds with suspected underlying immunodeficiency. This case series reports the findings in five dogs (four cavalier King Charles spaniels and one Bedlington terrier) with confirmed P. carinii pneumonia. Thoracic (CT) revealed ground glass opacity of the pulmonary parenchyma with a diffuse or multifocal distribution. The severity of this pattern was variable. Less consistent imaging findings included parenchymal bands, bronchial dilation and signs consistent with pulmonary hypertension. Four dogs recovered well with treatment and there was resolution of CT abnormalities in all dogs with follow‐up.     

Neurological signs and results of magnetic resonance imaging in 40 cavalier King Charles spaniels with Chiari type 1-like malformations

In human beings a Chiari type 1 malformation is a developmental condition characterised by cerebellar herniation and syringohydromyelia. Abnormalities compatible with such a malformation were identified by magnetic resonance imaging in 39 cavalier King Charles spaniels with neurological signs and in one neurologically normal cavalier King Charles spaniel that was examined postmortem. The dogs with these abnormalities had a wide variety of neurological signs, but there was no apparent correlation between the neurological signs and the severity of cerebellar herniation, syringohydromyelia or hydrocephalus.     

CHARACTERISTICS OF CEREBROSPINAL FLUID FLOW IN CAVALIER KING CHARLES SPANIELS ANALYZED USING PHASE VELOCITY CINE MAGNETIC RESONANCE IMAGING

Syringomyelia is an important morbidity source in Cavalier King Charles Spaniels. Although abnormal cerebrospinal fluid (CSF) flow secondary to Chiari malformations is thought to cause syringomyelia in humans, this relationship is unknown in dogs. We used phase-contrast magnetic resonance (MR) imaging to evaluate CSF flow in dogs. Fifty-nine Cavalier King Charles Spaniels were assigned a neurologic grade reflecting their neurologic status. Five normal control dogs of other breeding were imaged for comparison. The presence of syringomyelia was noted from sagittal MR images. The pattern and velocity of CSF flow were assessed using phase-contrast cine MRI at the foramen magnum, C2–C3 disc space, and within syrinxes. Flow was measured most easily with the neck flexed to mimic standing. CSF flow velocity in the dorsal aspect of the subarachnoid space at the foramen magnum was significantly higher in control dogs than Cavalier King Charles Spaniels ( P =0.035). Flow was obstructed at the foramen magnum in 41 of 59 Cavalier King Charles Spaniels. Turbulent flow and jets were associated with syringomyelia presence and severity, and CSF flow velocity at C2/3 dorsally was inversely related to the presence of syringomyelia ( P =0.0197). Peak dorsal subarachnoid space CSF flow velocity at the foramen magnum and C2–C3 were together highly predictive of syringomyelia. CSF flow can be assessed in dogs using phase-contrast cine MRI. Obstruction to flow at the foramen magnum is common in Cavalier King Charles Spaniels and CSF flow pattern and velocity are related to the presence of syringomyelia.     

Neurological diseases of the Cavalier King Charles spaniel

Several neurological syndromes have been described in Cavalier King Charles spaniels and many of the conditions have similar clinical signs. The current knowledge of these syndromes is reviewed in this article, with the aim of enabling the general practitioner to formulate a differential diagnosis and plan for diagnostic tests and treatment. Specifically, the article discusses and contrasts the most common conditions seen, Including occipital hypoplasia/syringomyelia, episodic collapse, epilepsy and vestibular disorders.     

Prevalence of asymptomatic syringomyelia in Cavalier King Charles spaniels

The prevalence of syringomyelia was investigated in a sample population of 555 Cavalier King Charles spaniels. All dogs, which were declared by their owners to be showing no clinical signs of syringomyelia, underwent MRI to determine the presence or absence of the condition. Data were analysed by logistic regression to determine the effects of sex and age on the prevalence of syringomyelia. Only increased age was found to have a significant effect. The prevalence of syringomyelia was 25 per cent in dogs aged 12 months, increasing to a peak of 70 per cent in dogs aged 72 months or more.     

COMPARISON OF CLOSURE TIMES FOR CRANIAL BASE SYNCHONDROSES IN MESATICEPHALIC,BRACHYCEPHALIC, AND CAVALIER KING CHARLES SPANIEL DOGS

Premature closure of cranial base synchondroses has been proposed as the mechanism for brachycephaly in dogs and caudal occipital malformation syndrome (COMS) in Cavalier King Charles Spaniels. The purpose of this retrospective study was to compare times of closure for cranial base synchondroses in mesaticephalic, brachycephalic, and Cavalier King Charles Spaniel dogs. Cranial magnetic resonance imaging studies were retrieved for client‐owned dogs less than 18 months of age. Breed, age, skull conformation, and the open or closed state of cranial base synchondroses were independently recorded by two observers. For dogs with a unanimous observer agreement, regression analysis was used to test effects of age and gender on the open or closed status of synchondroses and differences between groups. A total of 174 dogs were included in MRI interpretations and 165 dogs were included in the regression analysis. Statistically significant differences in closure time of the spheno‐occipital synchondrosis were identified between brachycephalic and mesaticephalic dogs (P = 0.016), Cavalier King Charles Spaniels and mesaticephalic dogs (P < 0.0001), and Cavalier King Charles Spaniels and brachycephalic dogs (P = 0.014). Findings from the current study supported the theory that morphological changes leading to the skull phenotype of the Cavalier King Charles Spaniels could be due to an earlier closure of the spheno‐occipital synchondrosis.     

Use of breed-specific ranges for the vertebral heart scale as an aid to the radiographic diagnosis of cardiac disease in dogs.

The vertebral heart scale was measured on right lateral recumbent thoracic radiographs of 320 dogs of six popular breeds, including for each breed at least 20 dogs with no clinical signs of cardiovascular or respiratory disease and at least 19 dogs with cardiac or respiratory disease. There were significant differences between the mean values of the scale for the different breeds; the normal boxer dogs had a significantly higher mean value than the normal dogs of all the other breeds, and the labrador retrievers had a significantly higher mean value than all the other breeds except the boxer and the cavalier King Charles spaniel. For all the breeds except the boxer, there was a trend for dogs with cardiac disease (but not respiratory disease) to have higher mean values on the scale than normal dogs of the same breed; however, at the optimal value of the scale for distinguishing between dogs of each breed with and without cardiac disease, the sensitivity and specificity were relatively low, in the range 58 to 83 per cent. The scale was most accurate for the diagnosis of cardiac disease in the Yorkshire terrier and the cavalier King Charles spaniel, breeds affected by predominantly dilative forms of cardiac disease. In contrast, it was very inaccurate in the boxer, a breed that has a higher incidence of cardiac diseases associated with concentric hypertrophy.     

Canine keratoconjunctivitis sicca: disease trends in a review of 229 cases

OBJECTIVES: To investigate the disease patterns in dogs with keratoconjunctivitis sicca referred to the University of Glasgow Small Animal Hospital. METHODS: A retrospective study of 229 cases was carried out. RESULTS: There were 44 breeds in the study, with four breeds, English cocker spaniels, cavalier King Charles spaniels, West Highland white terriers and shih-tzus, making up 58 per cent of the cases. Among these four breeds, two breed-dependent disease patterns, one chronic and one acute, were identified. English cocker spaniels and West Highland white terriers had a mean age at onset of clinical signs of five years and one month and five years and six months, respectively, with more females affected than males. Clinical signs consisted predominantly of conjunctival hyperaemia and mucopurulent discharge, with a relatively low incidence of ulcerative keratitis. In contrast, cavalier King Charles spaniels and shih-tzus showed a more acute disease pattern with a biphasic age distribution at 0 to less than two years of age, and four to less than six and six to less than eight years of age, respectively, with more males affected than females and a significantly higher incidence of ulcerative keratitis in some cases resulting in corneal perforation. CLINICAL SIGNIFICANCE: The study reveals interbreed differences with respect to sex, age and risk of ulcerative keratitis which have not been detailed previously in a referral population.     

Effectiveness of breeding guidelines for reducing the prevalence of syringomyelia

Several toy breed dogs are predisposed to syringomyelia (SM), a spinal cord disorder, characterised by fluid-filled cavitation. SM is a complex trait with a moderately high heritability. Selective breeding against SM is confounded by its complex inheritance, its late onset nature and high prevalence in some breeds. This study investigated the early outcome of existing SM breeding guidelines. Six hundred and forty-three dogs, 550 Cavalier King Charles spaniels (CKCS) and 93 Griffon Bruxellois (GB), were identified as having either one (454 dogs) or both parents (189 dogs) with MRI-determined SM status. Offspring without SM were more common when the parents were both clear of SM (SM-free; CKCS 70 per cent, GB 73 per cent). Conversely, offspring with SM were more likely when both parents had SM (SM-affected; CKCS 92 per cent, GB 100 per cent). A mating of one SM-free parent with an SM-affected parent was risky for SM affectedness with 77 per cent of CKCS and 46 per cent of GB offspring being SM-affected. It is recommended that all breeding dogs from breeds susceptible to SM be MRI screened; that the SM status at five years old is established; and all results submitted to a central database that can be used by dog breeders to better enable mate selection based on estimated breeding values.     

Differences between breeds of dog in a measure of heart rate variability

The vasovagal tonus index (VVTI), a time-domain indicator of heart rate variability, was measured in 92 dogs of six breeds (German shepherd dogs, labrador retrievers, cocker spaniels, boxers, bulldogs and cavalier King Charles spaniels). There was a significant difference in VVTI between the six breeds (P = 0.003). Brachycephalic dogs had a higher VVTI than other types of dog (P < 0.005), and when comparing individual breeds brachycephalic breeds tended to have a higher VVTI than non-brachycephalic breeds, although the difference was not always significant. The VVTI was negatively correlated with heart rate (P < 0.01) and dogs suffering from congestive heart failure had a lower VVTI than other dogs, whether compared within or between breeds (P < 0.01).     

Rate of change of heart size before congestive heart failure in dogs with mitral regurgitation

Objectives : The objective of the study was to examine the changes in vertebral heart scale, and left atrial and ventricular dimensions before and at onset of congestive heart failure in cavalier King Charles spaniels with mitral regurgitation. Methods : Records and radiographs from 24 cavalier King Charles spaniels with mitral regurgitation were used. Vertebral heart scale (24 dogs), and left atrial dimension and left ventricular end diastolic and end systolic diameters (18 dogs) and their rate of increase were measured at intervals over years to the onset of congestive heart failure. They were plotted against time to onset of congestive heart failure. Results : Dimensions and rates of change of all parameters were highest at onset of congestive heart failure, the difference between observed and chance outcome being highly significant using a two-tailed chi-square test (P<0·001). Clinical significance : The left heart chambers increase in size rapidly only in the last year before the onset of congestive heart failure. Increasing left ventricular end systolic dimension is suggestive of myocardial failure before the onset of congestive heart failure. Rate of increase of heart dimensions may be a useful indicator of impending congestive heart failure.     

Atypical masticatory muscle myositis in three cavalier King Charles spaniel littermates

This case report describes a novel manifestation of the immune-mediated disease, masticatory muscle myositis. Clinical signs, including difficulty in opening the mouth (trismus), were seen in three of four 12-week-old cavalier King Charles spaniel littermates. Diagnosis was established by 2M immunohistochemistry, supported by characteristic histopathological changes in affected temporal muscle. Treatment using corticosteroids at immune-modifying doses resulted in resolution of clinical signs in all the affected animals. Masticatory muscle myositis should be considered as a differential diagnosis in groups of young dogs with clinical signs of myositis localised to the head.