Syringomyelia (SM) MRI Screening Protocol
The British Veterinary Association (BVA) and the UK's Kennel Club (KC) issued in March 2012 a new syringomyelia MRI screening protocol, for complying with the BVA/KC's set of Chiari-like malformation and syringomyelia (CM/SM) breeding guidelines, with the aim of removing from breeding programs, cavalier King Charles spaniels with early-onset SM, and thereby reducing or even eliminating the incidence of CM/SM in future generations of cavaliers.
The MRI screening protocol is available in a downloadable pdf version at Dr. Clare Rusbridge's website by clicking here.
This MRI screening protocol should be followed everywhere in the world, to assure uniform application to the CM/SM Grading Scales, which are a set of uniform standards which any veterinary neurologist or other veterinary specialist should use to grade any cavalier's MRI scan.
4August 2011: Head position affects extent of cerebellar herniation in MRI scans. UK researchers examining 14 CKCS MRI scans (7 with CM/SM and 7 with just CM) comparing scans in both extended and flexed head positions. In their report, they have found the degree of cerebellar herniation was significantly worse in dogs with a flexed compared to an extended head position. When cerebrospinal fluid (CSF) space between the cerebellum and brainstem was compared in CKCS with and without SM, there was a significant increase in CSF space in CKCS with CM alone compared to those with CM/SM when head position was flexed. Based upon their findings, they state that it may be appropriate to position patients in a more flexed head position for optimal imaging in order to identify morphologic changes more accurately. They stated that this is important to consider for imaging CKCS with CM especially when studying the pathogenesis of CM/SM.
The researchers also found:
"When CSF space between the cerebellum and brainstem was compared in CKCS with and without SM, there was a significant increase in CSF space in CKCS with CM alone compared to those with CM/SM when head position was flexed. In their cine MR imaging study of CSF flow dynamics in CKCS with CM or CM/SM, Cerda-Gonzalez and others (2009a) found that turbulent CSF flow and jets are associated with SM presence and severity and CSF flow velocity at C2/3 is inversely related to the presence of SM. The reduced CSF space in CM/SM dogs reported in this study could explain this jet like CSF flow in dogs with CM/SM compared to those with CM alone."
This could mean that the severity of CM/SM, or even the presence or future presence of SM, could be predicted based upon the measure of cerebrospinal fluid space between the cerebellum and the brainstem. If so, this could go a long way to distinguishing whether a CM-only cavalier is either likely or unlikely to develop SM in the future. This distinction could be a way to fine-tune a breeding protocol, considering that we now know that over half of cavaliers with SM develop it after their 3rd birthday. If we could reliably count on this measurement of CSF space to tell us if a young dog will, or will not, develop SM in the future, then we could more reliably select SM-free breeding stock at a younger age than the present 2.5 years and the 3+ years the researchers may recommend in the future.
Dr. Rusbridge's Doctoral Thesis
Dr. Rusbridge's Syringomyelia News Winter 2007 Research Update
Dr. Rusbridge's Syringomyelia News Autumn 2007 Research Update
Dr. Rusbridge's Syringomyelia News 2007 Research Update
A SM support group is
A SM discussion group is http://groups.yahoo.com/group/CKCS-SM/
A website devoted to syringomyelia in Cavaliers is Karlin Lillington's SM.CavalierTalk.com
Neurological signs and results of magnetic resonance imaging in 40 cavalier King Charles spaniels with Chiari type 1-like malformations. Lu D, Lamb CR, Pfeiffer DU, Targett MP. Vet Rec. Aug 2003;153(9):260-3.
The Use of Ultrasonography to Diagnose “Caudal Occipital Malformation Syndrome” in Dogs – a Prospective Study in 12 Dogs. B Levitin, JJ McDonnell, D Faissler, AS Tidwell. J Vet Intern Med; May/June 2005;19(3) (ACVIM 23rd Ann. Vet. Med. Forum Abstract Program: Abstract 209).
Brain Stem Auditory Evoked Response (BAER) Testing in Cavalier King Charles Spaniels with Caudal Occipital Malformation Syndrome. CW Dewey, KS Bailey, G Barone, J Stefanacci. J Vet Intern Med; May/June 2006;20(3) (ACVIM 24th Ann. Vet. Med. Forum Abstract Program: Abstract 270).
Syringomyelia in cavalier King Charles spaniels: the relationship between syrinx dimensions and pain. C Rusbridge, H Carruthers. M-P Dubé, M Holmes, N D Jeffery. J Small Anim. Pract. 2007 Aug;48(8):432-6. Quote: "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."
Magnetic resonance imaging - unravelling the mystery. J. Fraser McConnell. J Small Anim. Pract. 2007 Aug;48(8): 421-422. Quote: "With evidence based medicine in vogue the study by Claire Rusbridge and colleagues (Rusbridge and others 2007) is to be congratulated and is of particular value as it contains a cohort of dogs with no clinical signs. Obtaining MR images of neurologically normal animals can be difficult as it is often not possible to justify anaesthesia in such animals. The use of MRI screening prior to breeding gives valuable information from a group of clinically normal animals. ... Reliance on statistical analyses alone can be misleading when it comes to determining the significance of MRI changes for an individual animal. While the diagnosis of syringohydromyelia in cavalier King Charles spaniels is straightforward with MRI, the problem is one of interpreting what the changes will mean for an individual. It has long been recognised that there is a poor correlation between severity of MRI abnormalities and whether the dog will show clinical signs with syringomyelia. This was shown by Lu and others (2003) who found no correlation between the severity of the syrinx and presence of neurological signs relating to the spine; some dogs with marked syringomyelia having no spinal signs (Lu and others 2003). The study by Rusbridge and colleagues in this issue reports different results and found, not surprisingly, that the larger the syrinx the more likely the individual will show clinical signs. While the study suggests that assessing syrinx size based on transverse plane images may be a more useful method than length of syrinx this does not give all the answers to the problem of interpretation. The data shows that while there is a good correlation between size of syrinx and pain there is an almost complete overlap of the range of syrinx size between the two groups. The significance of this is that it is still not possible to state with certainty, based on the MRI findings, whether a dog will develop clinical signs. It is likely that functional imaging such as CSF flow studies will be required to unpick the complexity of syringomyelia in cavalier King Charles spaniels. Similar findings have been reported in other areas of neuroimaging such as degenerative lumbosacral stenosis and intervertebral disk disease (Penning and others 2006, Mayhew and others 2002, Suwankong and others 2006) It is clear that with all the advances in imaging techniques there are still no easy answers. Imaging findings must be interpreted in light of the clinical findings and support rather than replace the neurologist or neuropathologist!"
Radiographic morphology of the cranial portion of the cervical vertebral column in Cavalier King Charles Spaniels and its relationship to syringomyelia. Stalin CE, Rusbridge C, Granger N, Jeffery ND. Am J Vet Res. 2008 Jan;69(1): 89-93. Quote: "Results suggested that radiographic morphology of the atlantoaxial region in CKCSs differs from morphology of that region in dogs of other breeds, but that these differences do not account for why some CKCSs develop syringomyelia and others do not."
Use of magnetic resonance imaging for morphometric analysis of the caudal cranial fossa in Cavalier King Charles Spaniels. Carrera I, Dennis R, Mellor DJ, Penderis J, Sullivan M. Am J Vet Res; 2009 Mar;70(3):340-5. Quote: "Objective-To perform morphometric analysis of the caudal cranial fossa in Cavalier King Charles Spaniels (CKCSs), to assess the relationship between caudal fossa dimensions and the frequency of magnetic resonance imaging (MRI) features of occipital abnormalities in CKCSs (with and without syringomyelia), and to compare caudal cranial fossa measurements in CKCSs with measurements of 2 groups of mesaticephalic dogs. Animals-70 CKCSs and 80 mesaticephalic (control) dogs. Procedures-Dogs were placed into 4 groups as follows: Labrador Retrievers (n = 40), spaniel-type dogs (40; English Springer Spaniels and Cocker Spaniels), CKCSs with syringomyelia (55), and CKCSs without syringomyelia (15). Multiple morphometric measurements (linear, angular, and area) were obtained from cranial midsagittalT2-weighted magnetic resonance images including the brain and cervical portion of the spinal cord. Several specific MRI findings were also recorded for CKCSs that appeared to affect the occipital bone and cervicomedullary junction. Results-No significant difference was identified among breeds in control groups and between sexes in any of the groups for all morphometric measurements. Significant differences were identified in CKCSs, compared with mesaticephalic dogs, in the area of the caudal cranial fossa and for several linear measurements that reflected the length of the ventral aspect of the occipital bone. These differences were greater in CKCSs with syringomyelia. All CKCSs had abnormalities in occipital bone shape. Conclusions and Clinical Relevance-CKCSs had a shallower caudal cranial fossa and abnormalities of the occipital bone, compared with those of mesaticephalic dogs. These changes were more severe in CKCSs with syringomyelia."
Growth of Clinical Veterinary Magnetic Resonance Imaging. Patrick R. Gavin. Vet.Rad. & Ultra.; Mar/Apr 2011; 52(s1):52-54. "In the spine, MR imaging reveals conditions which were previously difficult to diagnose ante-mortem, and may facilitate study of their pathophysiology. Examples include syringohydromyelia associated with Chiari-like malformation in the Cavalier King Charles spaniel... ."
Correlating Magnetic Resonance Findings with Neuropathology and Clinical Signs in Dogs and Cats. Charles H. Vite, Johnny R. Cross. Vet.Rad. & Ultra. Mar/Apr 2011; 52(1),Supp. 1:S23–S31.
Influence of head positioning on the assessment of Chiari-like malformation in Cavalier King Charles spaniels. J. J. Upchurch, I. M. McGonnell, C. J. Driver, L. Butler, H. A. Volk. Vet Rec Aug2011; doi:10.1136/vr.d4395. Quote: "Chiari-like malformation (CM) is almost omnipresent in the Cavalier King Charles spaniels (CKCS), often leading to syringomyelia (SM). Morphometric studies have produced variable results concerning relationship between the brain parenchyma within the caudal cranial fossa (CCF) and SM. The present study assesses the effect of head position, one potential confounder. Magnetic resonance images of CKCS with CM were reviewed in extended and flexed head positions. ... Fourteen CKCS were included into the study, seven dogs with CM and seven with CM/SM. ... The degree of cerebellar herniation was significantly worse in dogs with a flexed compared to an extended head position. ... Cerebellar herniation did not differ significantly between CKCS with and without SM in the extended head position. However, in the flexed head position the cerebellum herniated more caudally in CKCS with CM/SM compared to CKCS with CM alone. This highlights that the non-flexed head position may underestimate the true extent of cerebellar herniation and could explain why previous studies have missed an association with SM. ... It is well documented that flexing the head opens the CSF space of the cisterna magna and this could explain the morphometric measurements. The brainstem reduces in size to accommodate this increase in CSF space. ... When CSF space between the cerebellum and brainstem was compared in CKCS with and without SM, there was a significant increase in CSF space in CKCS with CM alone compared to those with CM/SM when head position was flexed. ... The reduced CSF space in CM/SM dogs reported in this study could explain this jet like CSF flow in dogs with CM/SM compared to those with CM alone. ... Furthermore, there is an association between head position and level of foramen magnum overcrowding. Based on these findings it may be appropriate to position patients in a more flexed head position for optimal imaging in order to identify morphologic changes more accurately. This is important to consider for imaging CKCS with CM especially when studying the pathogenesis of CM/SM. It should also be considered when taking a cisternal CSF sample as the spinal needle might be traumatic to the herniated cerebellum."
Distribution of syringomyelia along the entire spinal cord in clinically affected Cavalier King Charles Spaniels. Shenja Loderstedt, Livia Benigni, Kate Chandler, Jacqueline M. Cardwell, Clare Rusbridge, Christopher R. Lamb, Holger A. Volk. Vet J Dec 2011;190:359–363. Quote: "The objective of the present study was to define the anatomic distribution of SM in CKCS clinically affected by CM/SM. Our hypotheses were that in dogs with SM, (1)SM is present at multiple locations throughout the whole spinal cord; (2) the prevalence of SM is greatest in the cervical spine versus other regions; (3) the presence of SM in the cervical spinal cord is associated with the presence of SM in further caudal spinal cord regions; (4) the maximal syrinx size in each patient is in the cervical spine, and (5) there is a positive association between patient age and total syrinx size. ... 49 dogs were included in the present study. The median age of the dogs included in the study was 5 years (1.2–10.8 years). CM was present in all patients. ... All dogs in the present study were clinically affected. ... However, in our study SM was absent in 25% of dogs with clinical signs. ... There was no evidence of SM on MRI in 12/49 (25%) dogs. In all dogs with MRI evidence of SM (37/49; 75%), SM was present within the C1–C4 region (Figs. 2 and 3). Of those dogs with SM within C1–C4 region, 76% (28/37) also had SM within the C5–T1 and/ or T2–L2 regions, but only 49% (18/37) had SM within the L3–L7 region. ... The results of our investigation show that imaging only the cervical spine in clinically affected CKCSs is likely to underestimate the total syrinx size and the anatomical distribution of SM. The presence of cervical SM in all dogs with MRI evidence of SM could however justify the restricted imaging under screening purposes. ... The results of our study showed syrinx formation in all regions of the spinal cord with no significant differences of Sx between the cervical, cervicothoracic and thoracolumbar regions, but significantly smaller Sx in the caudolumbar spinal region. ... In our study Smax was most often seen within the C1–C4 region and, interestingly, also over T2–L2 vertebral bodies. ... We found that the severity of SM was positively correlated with patient age. This is consistent with previous studies indicating that CKCS with SM were significantly older than dogs without SM (Couturier et al., 2008). It seems likely therefore, that SM is a progressive disease in dogs. ... Conclusions: There is a very high potential for CKCS with clinical signs of CM/SM to develop SM in more than one spinal cord region. Diagnostic imaging limited to the cervical spine in clinically affected CKCS is likely to underestimate the degree and severity of SM. It can be hypothesised that SM has a progressive nature in CKCS, which requires further characterisation."