Vetrec-2011-100449.indd
PaperLong-term outcome of Cavalier King Charles spaniel dogs with clinical signs associated with Chiari-like malformation and syringomyelia
I. N. Plessas, C. Rusbridge, C. J. Driver, K. E. Chandler, A. Craig, I. M. McGonnell, D. C. Brodbelt, H. A. Volk
The disease complex Chiari-like malformation (CM) and syringomyelia (SM) has been
associated with the development of neuropathic pain (NeP), and commonly affects Cavalier
King Charles spaniels (CKCS). This prospective cohort study followed 48 CKCSs with
CM and/or SM and clinical signs suggestive of NeP for a period of 39 (±14.3) months from
diagnosis. At the end of the study, 36 dogs were still alive; five dogs died of an unrelated
or unknown cause, and seven were euthanased due to severe clinical signs suggestive
of NeP. During the follow-up period, the clinical signs of scratching, facial rubbing
behaviour, vocalisation and exercise ability were evaluated. Nine out of 48 dogs stopped
scratching (P<0.001), but there was no statistically significant change in the number of
dogs exhibiting exercise intolerance, vocalisation or facial rubbing behaviour. The overall
severity of clinical signs based on a visual analogue scale (VAS) (0 mm: no clinical signs
100 mm: severe clinical signs) increased (from median 75 mm (interquartile ranges (IQR)
68–84) to 84 mm (IQR 71.5–91), P<0.001). A quarter of the dogs were static or improved. In
general, the majority of the owners felt that the quality of life of their dogs was acceptable.
Medical treatments received were gabapentin or pregabalin and/or intermittently,
carprofen. The owner's perception of their animal's progress, and progress based on VAS,
had strong positive correlation (Spearman's rank correlation (s ) 0.74, P<0.001). Overall,
this study suggests that clinical signs suggestive of NeP progress in three-quarters of CKCSs
with CM and/or SM.
others 2003). SM refers to accumulation of fluid within the paren-
Chiari-like malformation (CM) and syringomyelia (SM) is an enfee-
chyma of the spinal cord, and is thought to result from CM and result-
bling disease complex most prevalent in Cavalier King Charles span-
ant changes in the dynamics of cerebrospinal fluid (CSF) flow through
iels (CKCS) (Rusbridge and others 2000). CM refers to the apparent
the foramen magnum and the cranial part of the cervical spinal cord
mismatch in volume between the caudal brain structures and the
(Pinna and others 2000, Iskandar and others 2004, March and others
caudal skull (Carrera and others 2009, Cross and others 2009, Driver
2005, Cerda-Gonzalez and others 2006, Rusbridge and others 2006,
and others 2010), and is associated with herniation of the cerebellum
Cerda-Gonzalez and others 2009).
through the foramen magnum (Rusbridge and others 2000, Lu and
The estimated prevalence of CM in this breed varies from 92 per
cent (Cerda- Gonzalez and others 2009) to 100 per cent (Couturier
and others 2008, Carrera and others 2009). SM affects almost half
Veterinary Record (2012)
doi: 10.1136/vr.100449
of asymptomatic (as perceived by their owners) young CKCS, which
I. N. Plessas, DVM, MRCVS,
A. Craig, BVSc; M Vet Clin Studies,
increases up to 70 per cent at the age of six years (Couturier and others
C. J. Driver, BSc, BVetMed (Hons),
2008, Parker and others 2011). The prevalence of CKCS with SM that
Department of Small Animal Medicine,
suffer clinical signs suggestive of NeP is unknown.
K. E. Chandler, BVetMed, PhD,
Faculty of Veterinary Science, University
Clinical signs typically associated with CM and/ or SM include
DipECVN, FHEA, MRCVS,
of Sydney, Australia
cervical scoliosis, thoracic and pelvic limb ataxia, thoracic limb paresis
D. C. Brodbelt, MA VetMB PhD DVA
I. M. McGonnell, PhD,
and signs suggestive of neuropathic pain (NeP) (Rusbridge and others
Department of Veterinary Basic
2006). NeP most often manifests as allodynia (pain arising from a
H. A. Volk, DVM, PhD, DipECVN,
Sciences, Royal Veterinary College,
non-noxious stimulus, i.e. gentle palpation) or dysaesthesia (sponta-
neous or evoked unpleasant sensation which manifests as phantom
Department of Veterinary Clinical
scratching, facial/ear rubbing) (Rusbridge and Jeffery 2008). One fre-
Sciences, Royal Veterinary College,
E-mail for correspondence:
quent sign of SM attributed to dysaesthesia and/or allodynia is phan-
tom scratching. Phantom scratching is characterised by a pelvic limb
C. Rusbridge, BVMS, PhD, DipECVN,
scratching action to the shoulder and neck area, often without making
Provenance: not commissioned;
skin contact, and typically on one side only. Unlike scratching associat-
externally peer reviewed
Goddard Veterinary Group, Stone Lion
ed with skin disease, dogs will often scratch whilst walking (Rusbridge
Veterinary Hospital, London, UK
Accepted September 21, 2012
and Jeffery 2008). It is not yet fully understood how CM/SM causes
10.1136/vr.100449
Veterinary Record
1 of 5
NeP. However, histopathological studies of SM in CKCS have found
the spinal cord parenchyma with a transverse diameter of greater than
that dogs which had expressed signs of NeP suffered an asymmetrical
or equal to 2 mm (Driver and others 2010).
syrinx with profound alteration of the structure of the dorsal horn
laminae, and had reduced expression of the pain-related neuropeptides
Assessment of clinical signs
substance P, and calcitonin gene-related peptide (Hu and others 2012a).
A questionnaire was used to assess the following factors in a face-to-
Glial and fibrous proliferation were also associated with expression
face interview at the initial visit, and then by telephone for the follow
of clinical signs (Hu and others 2012b). Chiari-I malformation/SM
up: name of the animal and owner, sex of the animal and neutering
causes clinical signs of NeP in up to 80 per cent of human beings with
status, date of birth and death, if applicable, whether it was euthana-
this disorder, and up to 35 per cent of affected dogs (Todor and oth-
sia or natural death, cause of death, general health status, history of
ers 2000, Rusbridge and others 2007). NeP has an important impact
brain or spinal cord diseases other than CM/SM, if the dog had evi-
on the affected person's quality of life (QOL) and neurobehaviour
dence of inflammation in the external ear canal, history and clinical
(Gustorff and others 2008), and a recent study in dogs (Rutherford and
signs of skin disease, seizures, exercise intolerance, whether there was
others 2010) confirmed an association between the degree of NeP and
another MR scan performed following their initial visit, if the animal
fear/anxiety-related behavioural changes.
underwent craniocervical decompression, if the animal had been diag-
Medical and surgical treatment options exist for dogs with CM/SM.
nosed with a heart murmur, and what grade it was. The owners were
Medical management includes the use of NSAIDs, drugs that reduce CSF
also asked to confirm the presence of the following signs: phantom
production (omeprazole, cimetidine), corticosteroids and antiepileptic
scratching of shoulder and/or neck, facial and ear rubbing, vocalisa-
drugs that have analgesic properties (gabapentin, pregabalin). However,
tion and spinal pain. At the initial consult, all dogs were examined by
there is no scientific evidence to prove the efficacy of these drugs in the
a board-certified neurologist, and had unremarkable complete blood
management of NeP associated with CM/SM in dogs (Rusbridge and
cell count and serum biochemistry profile. A VAS was used by the
others 2006, Rusbridge and Jeffery 2008, Wolfe and Poma 2010). Surgical
authors to assess the frequency and intensity of clinical signs sugges-
management (craniocervical decompression) is frequently performed in
tive of NeP. A 100 mm line ranging from 0 mm (asymptomatic dogs
people with Chiari-I malformation, with and without SM, to alleviate
exhibiting normal exercise ability, no scratching, no facial rubbing and
clinical signs (Tubbs and others 2003). Following surgery, 80 per cent of
no vocalisation) to 100 mm (dogs with severely compromised exercise
dogs improved, but there was no resolution of the syringes, and nearly al
activity, scratching more than five times a day and vocalising more
dogs continued to exhibit clinical signs suggestive of NeP postoperatively
than five times a week) was used for the VAS assessment by intersect-
(Vermeersch and others 2004, Dewey and others 2005, 2007, Rusbridge
ing the line with a second perpendicular line drawn by the observer,
2007). Additionally, 25–47 per cent of the operated dogs showed recur-
based on the subjective severity of the signs reported. To ensure con-
rence or deterioration of the clinical signs within 0.2–3 years after sur-
sistency as much as possible between scorers, two of the authors,i ii
gery (Dewey and others 2005, 2007, Rusbridge 2007).
responsible for the initial scoring underwent training, which involved
To the authors' knowledge there are currently no data regarding
independent scoring of clinical signs until their score varied less than
the long-term outcome of non-surgically managed dogs with clini-
three per cent. The follow-up information gathered by calling the
cal signs suggestive of NeP secondary to CM/SM. This prospective
owners was cross-referenced to the history from the referring veteri-
cohort study follows 48 CKCS dogs with clinical signs suggestive of
narian. The owners were asked what medication the animal received,
NeP due to CM and/ or SM for a period of 39 (±14.3) months.
and what was their perception of the progress of their animal's condi-
tion (worse, unchanged, better) and QOL. The follow-up information
Materials and methods
was subsequently given a second score (VAS) by one of the authors.ii
Study design
The author who assessed the VAS at the follow up was blinded to the
A prospective cohort study was performed following 48 CKCS dogs
initial score. In addition, the volume of the caudal cranial fossa, the
with CM and/ or SM disease complex for a mean period of 39 (±14.3)
parenchyma within the caudal cranial fossa, and the sizes of the ven-
months from treatment termination.
tricles and syringes were measured at the time of diagnosis using previ-
ously described methodology (Driver and others 2010) and compared
with the progression of the assessed clinical signs.
CKCSs between the ages of one and 13 years (median 46 months),
and bodyweight of 4–13 kg (median 9.5 kg), were recruited from the
general population in the UK by advertising through the veterinary
Statistical analysis was performed with a commercial software pack-
press and national CKCS health societies, into a two-week trial of
age (Prism 5 for Mac, Graphpad Software 2007). Paired nominal
a novel neuropathic pain medication, which was performed under
categorical data were compared using the McNemar's χ2 test. All
the Animals (Scientific Procedures) Act 1986, and was approved by
quantitative data were assessed for normality of distribution with
the institution's ethics committee. After the end of the drug trial,
the D'Agostino and Pearson omnibus normality test and graphically.
dogs were followed prospectively for a mean period of 39 (±14.3 SD,
Means and SDs were calculated for normally distributed continuous
4–107) months for this study.
data (means (±SD)), and medians and interquartile ranges (IQR) were
Dogs with other medical or neurological conditions that could
determined for non-parametric data (median (IQR)). A Wilcoxon's
have influenced the preceding pharmacological study were excluded,
signed rank test was used for statistical evaluation of paired, non-para-
such as brain or spinal cord diseases, other than CM/SM; CKCS
metric data. The unpaired
t test (parametric data) or Mann-Whitney U
which had undergone craniocervical decompression; those with evi-
test (non-parametric data) were used for comparing the morphometric
dence of inflammation in the external ear canal (erythema, discharge,
values between dogs with and without deterioration of clinical signs
lichenification); with a history and clinical signs of skin disease; sei-
assessed by VAS as appropriate. The association between owner's per-
zures at the time of diagnosis; or with a systolic heart murmur of
ception of their animal's progress (worse, unchanged, better) and pro-
greater than grade II/VI. Thus, owners were questioned about gen-
gress based on VAS, was evaluated with Spearman's rank correlation.
eral health status, exercise intolerance and clinical signs suggestive
A P value of 0.05 or below was considered significant.
of pruritus and pain. Recruitment and inclusion criteria were: CKCS
with clinical signs suggestive of NeP (such as spinal hyperaesthesia
on palpation, facial rubbing, vocalisation and/or phantom scratching)
Sixty-one CKCS were initially considered for this study. Of these,
(Rusbridge and others 2006, Rusbridge and Jeffery 2008) which scored
eight were excluded due to the absence of clinical signs attribut-
50 or more on the visual analogue scale (VAS), underwent MRI of the
able to CM/SM (n=3), craniocervical decompression (n=1), grade
brain and spinal cord (Driver and others 2010, Loderstedt and others
III/VI systolic heart murmur (n=2), generalised pyoderma (n=1), and
2011), and were subsequently diagnosed with CM and/or SM by a
board-certified neurologist. CM was defined as evidence of cerebellar
herniation or indentation by the supraoccipital bone (Lu and others
2003), and a syrinx was defined as a fluid-containing cavity within
2 of 5 Veterinary Record 10.1136/vr.100449
otitis externa (n=1). Fifty-three dogs underwent MRI of the brain
and the whole spinal cord. Of these, three were excluded because
To the authors' knowledge, this is the first report of clinical signs
MRI studies were incomplete and two were excluded because of
suggestive of NeP associated with CM/SM being progressive in the
evidence of disc extrusion at C2–C3. Therefore, 48 dogs with MRI
majority of dogs when treated non-surgically. In this study, morpho-
confirmed CM and clinical signs suggestive of NeP were included
metric values did not seem to play a significant role in the progres-
and followed up for a period of 39 (±14.3) (mean (±SD)) months
sion or improvement of the clinical signs. Three-quarters of the dogs
from diagnosis, prospectively. There were 25 male dogs (17 neu-
displayed progression of clinical signs, whereas, one-quarter remained
tered) and 23 female (16 neutered). Thirty-nine dogs (81 per cent,
static or improved. Despite the deterioration of the clinical signs in the
95 per cent CI 69.9 per cent to 92.1 per cent) had SM at the time
majority of these dogs, 75 per cent were still alive 39 (±14.3) months
of diagnosis.
later, with an acceptable QOL for the owners. All the owners of these
At the end of the study, 36 dogs (75 per cent, 95 per cent CI 62.75
dogs indicated that if their dogs' QOL was severely compromised,
per cent to 87.25 per cent) were still alive, four dogs died naturally at
then they would opt for euthanasia. This study shows that non-
home with signs of suspected congestive heart failure (a postmortem
surgical management of this condition can be an acceptable option
examination was not performed), one dog was euthanased due to an
considering that there is no evidence for better management in dogs.
aggressive ovarian tumour that had metastasised to other organs, and
The clinical course of the clinical signs seen in our study is similar
seven were euthanased due to severe signs of NeP. The mean age at
to what has been reported in human beings with unoperated SM asso-
diagnosis was 53.2 (±33.1) months, and the mean age at the end of the
ciated with type I Chiari malformation, and/or underdevelopment of
study, or death, 92.3 (±30.5) months.
posterior fossa (Bogdanov and Mendelevich 2002). It has been suggest-
Overall, 39 dogs were treated medically with gabapentin
ed that the natural history of symptomatic SM associated with type
(Neurontin, 10 mg /kg every 8–12 hours), pregabalin (Lyrica,
I Chiari malformation, and/or signs of posterior fossa underdevelop-
2–4 mg /kg every 8 hours) and/or intermittently carprofen ment, is characterised by an initial relatively rapid clinical progression
(Rimadyl, 2 mg/kg every 24 hours) and nine dogs were not on
accompanied with distended cavities. If surgery is not performed, then
any medication for the management of NeP. Nine out of 48 dogs
a state of syrinx equilibrium may be reached, that is, no further expan-
stopped scratching (P < 0.001), but there was no statistically signifi-
sion of the syrinx, although this may be associated with irreversible
cant change in the number of dogs exhibiting compromised exercise
spinal cord damage. Eventually, in some cases, there may be MRI signs
ability, vocalisation or facial rubbing behaviour. The median VAS
of cavity collapse (Vaquero and others 2011). A possible explanation is
of the clinical signs increased in the study population significantly
that there was destruction of spinothalamic and lemniscal tracts by the
(P < 0.001) from the initial median VAS of 75 mm (IQR 68–84 mm)
syringes, so that pain could no longer be perceived (Hatem and others
to follow up VAS 84 mm (IQR 71.5–91 mm) (Fig 1). The severity
2010). A further possibility is that the caudal cranial fossa and brain
of clinical signs based on VAS deteriorated in 36 (75 per cent, 95 per
parenchyma adjust to compensate for the overcrowding of the caudal
cent CI 62.75 per cent to 87.25 per cent) dogs. From these dogs, 31
cranial fossa (Hamilton and others 2011).
(86 per cent, 95 per cent CI 76.18 per cent to 95.82 per cent) were
Rusbridge (2007) followed up 15 CKCSs that underwent crani-
treated with gabapentin or pregabalin, and carprofen. The remain-
ocervical decompression for 0.2–2.3 years after surgery, and despite
ing five dogs did not receive any treatment. A quarter of the dogs
an initial clinical improvement in 80 per cent of the dogs, 47 per cent
(95 per cent CI 12.75 to 37.25) were static (n=7, 14.5 per cent) or
deteriorated in a mean time of 1.3 years. Ten out of these 15 dogs
improved (n=5, 10.5 per cent).
had severe signs of NeP, and medical treatment was not successful.
The owner's perception of their animal's progress, and progress
Despite the initial improvement though, all dogs continued to exhib-
based on VAS, was strongly positively correlated (Spearman's rank
it signs of NeP, and surgery did not change the size of the cervical
correlation (s )=0.74, P<0.001). All the owners of the dogs that were
syringes. Similar findings were described by Vermeersch and others
alive at the end of the study reported that their dog's QOL was not
(2004) and Dewey and others (2005, 2007). One cause of deteriora-
severely compromised, and had that been the case, they would have
tion was attributed to scar tissue adhering to exposed neural tissue and
opted for euthanasia. There was no significant association between
preventing adequate CSF flow. Further studies that follow up cases
morphometric values (volume of the caudal cranial fossa, the paren-
for a longer period of time are needed to be able to compare the long-
chyma within the caudal cranial fossa, and the sizes of the ventricles
term outcome of conservative and surgical treatment, but it seems that
and syringes) at the time of diagnosis between dogs with and without
craniocervical decompression may not have more favourable long-
deteriorating clinical signs.
term outcomes in the management of neuropathic pain.
In three out of eight female entire bitches, the owners reported
Nine of the 48 dogs had CM only. Traditionally, clinical signs sug-
periodical aggravation of the CM/SM-related clinical signs during oes-
gestive of NeP in CKCS have been associated with CM and SM, but
trus. Six dogs developed seizures during the study.
from our study, we found that CM only may contribute to these signs,
too. The pathophysiology of these clinical signs in dogs with CM
is not well understood, but the overcrowding of the foramen mag-
num might be applying pressure onto brainstem nuclei causing signs
suggestive of NeP (Rusbridge and Jeffery 2008). Unfortunately, there
is no evidence for this theory, but in the human literature, there are
several reports associating Chiari malformation with trigeminal neu-
ralgia that resolves after craniooccipital decompression or placement
of vetriculoperitoneal shunt (Gnanalingham and others 2005, Vince
and others 2010).
Interestingly, there was a significant improvement in scratching
(nine dogs stopped scratching), whereas, the VAS got significantly
worse. New research findings in the area of pruritus may give an
explanation for this. Pruritus, traditionally, has been associated with
a submodality or subquality of pain (Sun and others 2009). Advances
in this area have elucidated differences between pruritus and pain, but
have also obscured the distinction between them. Pruritus and pain
appear to be independent sensations because nociceptive and pruri-
ceptive stimuli each elicit unique behavioural responses (Davidson
FIG 1: Scatter plots showing the distribution of Visual Analogue
Scale (mm) in Cavalier King Charles spaniel dogs with clinical signs
and others 2010). Sun and Chen (2007) reported that they have iden-
at the beginning of the study (initial) and 39±14.3 months later
tified the first gene in the spinal cord of mice, linked with pruritus,
(follow up) (bar represents median; Wilcoxon's signed rank test,
and that it is responsible for the expression of gastrin-releasing pep-
*represents P < 0.001)
tide receptors (GRPR). These GRP receptors were found in a group
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Veterinary Record
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of spinal cord cells called lamina-I neurones that relay both itch and
shared ethical dilemma with human sufferers of CM/SM complex.
pain sensation to the brain. However, Sun and others (2009) believe
For each owner, this will be an individual decision, and based on the
that there is a specific subpopulation of GRPR neurones, located in
bond they have with their pets, and also the potential economic deci-
the superficial dorsal horn within the lamina-I, that are specific only
sions. Finally, it is important to mention that this study is document-
for the pruritic sensation (labelled-line hypothesis). Destruction of the
ing the progression of a specific set of clinical signs in a group of dogs
described neurones in mice and stimulation afterwards with various
with CM and/or SM, and not the progression of the disease itself.
pruritic agents showed up to an 85 per cent reduction of pruritus com-
Radiological progression of the disease has not yet been reported.
pared with the controlled group, but nociception and motor function
Fifteen per cent (95 per cent CI 4.9 to 25.1) of the study population
appeared to be unaffected (Sun and others 2009). Many attempts to
was euthanased at the request of the owner due to the severity of the
localise the pathway of these GRPR neurones all the way up to the
clinical signs. However, in the remaining surviving dogs, despite the
thalamus (where they finally project) failed, but it is still believed
progression of the clinical signs, the majority of the owners felt that
that the pruriceptive pathway is different to the nociceptive path-
QOL of their dogs was acceptable.
way in the spinothalamic tract neurones that project to the posterior
and ventral posterior region of the thalamus (Sun and others 2009).
Further research is needed to elucidate the difference between these
The authors would like to acknowledge the owners of the patients
two pathways.
who participated in this study, and the veterinarians who referred
Another interesting finding of this study is that despite the fact
them to us. The authors would like to thank the various CKCS health
that history of seizures at the time of diagnosis was an exclusion cri-
clubs for their continuous support for the investigation of these con-
terion, six dogs (12.2 per cent) developed seizures in the investigated
ditions. Finally, the authors also would like to thank the Clinical
period of time. It was suggested that seizures may be related to ven-
Investigation Centre of the Royal Veterinary College and its research
triculomegaly secondary to overcrowding of the caudal cranial fossa
office for assessing the manuscript according to the Royal Veterinary
(Rusbridge and others 2006), however, a recent study could not show
College's code of good research practice (authorisation number:
a relationship between ventroculomegaly and seizures (Driver and
others 2012). This finding is more likely related to idiopathic epilepsy
which is common in this breed (Rusbridge and Knowler 2004) or
other unknown pathologies. In human beings, epilepsy, in conjunc-
BOGDANOV, E. I. & MENDELEVICH, E. G. (2002) Syrinx size and duration of symp-
tion with type I Chiari malformation, is occasionally reported. Two
toms predict the pace of progressive myelopathy: retrospective analysis of 103 unop-
subtypes are described: the first as an incidental finding in the diag-
erated cases with craniocervical junction malformations and syringomyelia.
Clinical
nostic work-up of patients with idiopathic epilepsies, and the second
Neurology and Neurosurgery 104, 90–7
where both type I Chiari malformation and epilepsy occur as part of
BOND, A., SHINE, P. & BRUCE, M. (1995) Validation of visual analogue scales in
anxiety.
International Journal of Methods in Psychiatric Research 5, 1–9
a more widespread developmental disorder (Granata and Valentini
BOONSTRA, A. M., RENEMAN, M. F., POSTHUMUS, J. B., STEWART, R. E. &
SCHIPHORST PREUPER, H. R. (2008) Reliability and validity of the visual analogue
In three out of eight female entire bitches, the owners reported
scale for disability in patients with chronic musculoskeletal pain.
International Journal of
periodical aggravation of the CM/SM-related clinical signs during oes-
Rehabilitation Research 31(2), 165–169
CARRERA, I., DENNIS, R., MELLOR, D. J., PENDERIS, J. & SULLIVAN, M. (2009)
trus. Hubscher and others (2010) reported that oestrogen (17b-estradi-
Use of magnetic resonance imaging for morphometric analysis of the caudal cranial
ol) administration reduces experimentally induced allodynia in rats,
fossa in Cavalier King Charles spaniels.
American Journal of Veterinary Research 70, 340–345
but there is no literature to support that oestrogens can deteriorate the
CERDA-GONZALEZ, S., OLBY, N. J., BROADSTONE, R., MCCULLOUGH, S. &
signs of NeP. To the authors' knowledge, there is no involvement of
OSBORNE, J. A. (2009) Characteristics of cerebrospinal fluid flow in Cavalier King
oestrogens in the pathophysiology of NeP, and this may be an inter-
Charles Spaniels analyzed using phase velocity cine magnetic resonance imaging.
Veterinary Radiology and Ultrasound 50, 467–76
esting finding that requires further investigation. Perhaps the stress
CERDA-GONZALEZ, S., OLBY, N. J., PEASE, T. P., MCCULLOUGH, S., MASSOUD,
associated with the oestrus can aggravate the perception of NeP, in a
N. & BROADSTONE, R. (2006) Morphology of the caudal fossa in cavalier King
similar way that mood affects the pain perception in human beings
Charles spaniels.
Journal of Veterinary Internal Medicine 20, 736
(Gustorff and others 2008).
COUTURIER, J., RAULT, D. & CAUZINILLE, L. (2008) Chiari-like malformation and
syringomyelia in normal Cavalier King Charles spaniels: a multiple diagnostic imaging
There are limitations to this study, considering the selection cri-
approach.
Journal of Small Animal Practice 49, 438–443
teria. Only dogs with VAS of 50 or more underwent MRI, and were
COX, J. & DAVISON, A. (2005) The visual analogue scale as a tool for self-reporting of
included in the study. However, we do not feel that selecting for dogs
subjective phenomena in the medical radiation sciences.
Radiographer 52(5), 22
with prominent clinical signs is a significant limitation of the study
CROSS, H. R., CAPPELLO, R. & RUSBRIDGE, C. (2009) Comparison of cerebral cra-
nium volumes between Cavalier King Charles spaniels with Chiari-like malformation,
as they reflect the general population of dogs that are presented to a
small breed dogs and Labradors.
Journal of Small Animal Practice 50, 399–405
neurologist for investigation of this condition. There is possible bias
DAVIDSON, S. & GIESLER, G. (2010) The multiple pathways for itch and their interac-
from the owners and the veterinary surgeons when assessing the
tions with pain.
Trends in Neurosciences 33, 550–558
clinical signs. Also, one of the most important problems is that pain
DEWEY, C., BERG, J., BARONE, G., MARINO, D. J. & STEFANACCI, J. D. (2005)
Foramen magnum decompression for treatment of caudal occipital malformation syn-
is a subjective variable and may have been inappropriately assessed
drome in dogs.
Journal of the American Veterinary Medical Association 227, 1270–1275
considering that the dogs cannot verbally communicate their level of
DEWEY, C. W., MARINO, D. J., BAILEY, K. S., LOUGHIN, C. A., BARONE,
discomfort. Moreover, we can only assume that CM/SM is the cause
G., BOLOGNESE, P., MILHORAT, T. H., & POPPE, D. J. (2007) Foramen magnum
of the described clinical signs and that these signs are suggestive of
decompression with cranioplasty for treatment of caudal occipital malformation syn-
neuropathic pain. A follow-up MRI was not performed, so we cannot
drome in dogs.
Veterinary Surgery 36, 406–415
DRIVER, C. J., CHANDLER, K., WALMSLEY, G., SHIHAB, N. & VOLK, H. A. (2012)
exclude the possibility of development of other spinal diseases in the
The association between Chiari-like malformation, ventriculomegaly and seizures in
study period that may affect the progression of the clinical signs. The
cavalier King Charles spaniels.
The Veterinary Journal (In press) http://dx.doi.org/10.1016/j.
VAS itself, as with other scoring systems, is subject to bias, and its
reproducibility can be questioned; however, the scorers in this study
DRIVER, C. J., RUSBRIDGE, C., CROSS, H. R., MCGONNELL, I. & VOLK,
H. A. (2010) Relationship of brain parenchyma within the caudal cranial fossa and
were trained to be as consistent as possible in their observations. The
ventricle size to syringomyelia in Cavalier King Charles spaniels.
Journal of Small Animal
VAS is a recognised tool for measuring subjective phenomena, such
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10.1136/vr.100449
Veterinary Record
5 of 5
Long-term outcome of Cavalier King Charles
spaniel dogs with clinical signs associated
with Chiari-like malformation and
syringomyelia
I. N. Plessas, C. Rusbridge, C. J. Driver, et al.
published online October 25, 2012
Veterinary Recorddoi: 10.1136/vr.100449
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