Abstract
Syringomyelia is an increasingly common diagnosis in veterinary medicine, especially in toy breed dogs where selections for small size and brachycephalic head shape are contributing factors. The most common cause is a condition analogous to Chiari I malformation in humans. This chapter details the pathophysiology, clinical signs, medical and surgical management, progression and prognosis of the canine condition. As a naturally occurring model of both syringomyelia and central neuropathic pain, observations that may have relevance to understanding of the pathophysiology of Chiari malformation and syringomyelia in humans are discussed. Finally, current knowledge of genetic factors and breeding advice is reviewed.
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Notes
- 1.
Written guidelines for the appearance, movement and temperament of a dog, which is the template used by breeders to produce typical specimens of the breed and the tool of the judge for assessing dogs in the show ring. In the UK the Kennel Club was founded in 1873 and took ownership of the breed standards drafted by the early dog breeders.
- 2.
This autosomal recessive-inherited paroxysmal exertion-induced dyskinesia is characterised by hypertonicity and abnormal posturing, usually occurring after exercise or periods of excitement. It is due to a 16 kb deletion encompassing the first three exons of the brevican gene (BCAN). Brevican is one of the central nervous system-specific members of the hyaluronan-binding chondroitin sulphate proteoglycan family. It is important in the organisation of the nodes of Ranvier in myelinated large diameter axons, and disruption of this region results in a delay in axonal conduction (Forman et al. 2012). Dogs can be screened for the disorder with a DNA test.
- 3.
- 4.
The terminal ventricle (ventriculus terminalis, or fifth ventricle) is an enlargement of spinal cord central canal within or near the filum terminale. In many species, and possibly some humans, there is a functional communication between this dilated central canal and the subarachnoid space. In humans the terminal ventricle is visible in the foetus and children but is usually absent in adults. There is evidence suggesting that the cerebrospinal fluid in the central canal may facilitate non-synaptic neural transmission or clear waste products and particulate matter from the parenchyma (so called ‘sink’ function) (Storer et al. 1998).
- 5.
Otitis media with effusion is often referred to as ‘glue ear’ and is characterised by thick or sticky fluid behind the eardrum in the middle ear but no ear infection. In the Cavalier King Charles spaniel, it is also sometimes referred to as primary secretary otitis media or PSOMs.
References
Adams VJ, Evans KM, Sampson J et al (2010) Methods and mortality results of a health survey of purebred dogs in the UK. J Small Anim Pract 51(10):512–524. doi:10.1111/j.1748-5827.2010.00974.x
Argoff CE (2011) Review of current guidelines on the care of postherpetic neuralgia. Postgrad Med 123(5):134–142. doi:10.3810/pgm.2011.09.2469
Armstrong WE, Sladek CD (1985) Evidence for excitatory actions of histamine on supraoptic neurons in vitro: mediation by an H1-type receptor. Neuroscience 16(2):307–322
Attar A, Kaptanoglu E, Aydin Z et al (2005) Electron microscopic study of the progeny of ependymal stem cells in the normal and injured spinal cord. Surg Neurol 64(Suppl 2):S28–S32. doi:10.1016/j.surneu.2005.07.057
Barnes PJ (1998) Anti-inflammatory actions of glucocorticoids: molecular mechanisms. Clin Sci (Lond) 94(6):557–572
Bynevelt M, Rusbridge C, Britton J (2000) Dorsal dens angulation and a Chiari type malformation in a Cavalier King Charles Spaniel. Vet Radiol Ultrasound 41(6):521–524
Cappello R, Rusbridge C (2007) Report from the Chiari-Like Malformation and Syringomyelia Working Group round table. Vet Surg 36(5):509–512. doi:10.1111/j.1532-950X.2007.00298.x
Carrera I, Dennis R, Mellor DJ et al (2009) Use of magnetic resonance imaging for morphometric analysis of the caudal cranial fossa in Cavalier King Charles Spaniels. Am J Vet Res 70(3):340–345. doi:10.2460/ajvr.70.3.340
Cerda-Gonzalez S, Dewey CW, Scrivani PV et al (2009a) Imaging features of atlanto-occipital overlapping in dogs. Vet Radiol Ultrasound 50(3):264–268
Cerda-Gonzalez S, Olby NJ, Broadstone R et al (2009b) Characteristics of cerebrospinal fluid flow in Cavalier King Charles Spaniels analyzed using phase velocity cine magnetic resonance imaging. Vet Radiol Ultrasound 50(5):467–476
Chapman DB, Rees CJ, Lippert D et al (2011) Adverse effects of long-term proton pump inhibitor use: a review for the otolaryngologist. J Voice 25(2):236–240. doi:10.1016/j.jvoice.2009.10.015
Chong MS, Hester J (2007) Diabetic painful neuropathy: current and future treatment options. Drugs 67(4):569–585
Cinalli G, Spennato P, Sainte-Rose C et al (2005) Chiari malformation in craniosynostosis. Childs Nerv Syst 21(10):889–901. doi:10.1007/s00381-004-1115-z
Connell JM, MacKenzie SM, Freel EM et al (2008) A lifetime of aldosterone excess: long-term consequences of altered regulation of aldosterone production for cardiovascular function. Endocr Rev 29(2):133–154. doi:10.1210/er.2007-0030
Cross HR, Cappello R, Rusbridge C (2009) Comparison of cerebral cranium volumes between cavalier King Charles spaniels with Chiari-like malformation, small breed dogs and Labradors. J Small Anim Pract 50(8):399–405. doi:10.1111/j.1748-5827.2009.00799.x
de Jong T, Toll MS, de Gier HH et al (2011) Audiological profile of children and young adults with syndromic and complex craniosynostosis. Arch Otolaryngol Head Neck Surg 137(8):775–778. doi:10.1001/archoto.2011.115
Dewey CW, Berg JM, Barone G (2005) Foramen magnum decompression for treatment of caudal occipital malformation syndrome in dogs. J Am Vet Med Assoc 227(8):1270–1275, 1250–1251
Dewey CW, Marino DJ, Bailey KS et al (2007) Foramen magnum decompression with cranioplasty for treatment of caudal occipital malformation syndrome in dogs. Vet Surg 36(5):406–415. doi:10.1111/j.1532-950X.2007.00286.x
Di Rocco C, Frassanito P, Massimi L et al (2011) Hydrocephalus and Chiari type I malformation. Childs Nerv Syst 27(10):1653–1664. doi:10.1007/s00381-011-1545-3
Driver CJ, Rusbridge C, Cross HR et al (2010a) Relationship of brain parenchyma within the caudal cranial fossa and ventricle size to syringomyelia in cavalier King Charles spaniels. J Small Anim Pract 51(7):382–386. doi:10.1111/j.1748-5827.2010.00952.x
Driver CJ, Rusbridge C, McGonnell IM et al (2010b) Morphometric assessment of cranial volumes in age-matched Cavalier King Charles spaniels with and without syringomyelia. Vet Rec 167(25):978–979. doi:10.1136/vr.c4109
Driver CJ, De Risio L, Hamilton S et al (2012) Changes over time in craniocerebral morphology and syringomyelia in cavalier King Charles spaniels with Chiari-like malformation. BMC Vet Res 8(1):215. doi:10.1186/1746-6148-8-215
Driver CJ, Chandler K, Walmsley G et al (2013) The association between Chiari-like malformation, ventriculomegaly and seizures in cavalier King Charles spaniels. Vet J 195:235–237. doi:10.1016/j.tvjl.2012.05.014
Elia M, Biondi R, Sofia V et al (1999) Seizures in Chiari I malformation: a clinical and electroencephalographic study. J Child Neurol 14(7):446–450
Faraci FM, Mayhan WG, Heistad DD (1990) Effect of vasopressin on production of cerebrospinal fluid: possible role of vasopressin (V1)-receptors. Am J Physiol 258(1 Pt 2):R94–R98
Finnerup NB (2008) A review of central neuropathic pain states. Curr Opin Anaesthesiol 21(5):586–589. doi:10.1097/ACO.0b013e32830a4c11
Forman OP, Penderis J, Hartley C et al (2012) Parallel mapping and simultaneous sequencing reveals deletions in BCAN and FAM83H associated with discrete inherited disorders in a domestic dog breed. PLoS Genet 8(1):e1002462. doi:10.1371/journal.pgen.100246210.1371/journal.pgen.1002462
Gellman H (2000) Reflex sympathetic dystrophy: alternative modalities for pain management. Instr Course Lect 49:549–557
German AJ, Holden SL, Wiseman-Orr ML et al (2012) Quality of life is reduced in obese dogs but improves after successful weight loss. Vet J 192(3):428–434. doi:10.1016/j.tvjl.2011.09.015
Giejda A, Veal J, Volk H et al (eds) (2012) Spinal cord analysis in the Neonatal Cavalier King Charles Spaniel 25th annual symposium of the European College of Neurology, Ghent, 14–15 Sept 2012.
Granata T, Valentini LG (2011) Epilepsy in type 1 Chiari malformation. Neurol Sci 32(Suppl 3):S303–S306. doi:10.1007/s10072-011-0697-y
Gustorff B, Dorner T, Likar R et al (2008) Prevalence of self-reported neuropathic pain and impact on quality of life: a prospective representative survey. Acta Anaesthesiol Scand 52(1):132–136. doi:10.1111/j.1399-6576.2007.01486.x
Hagiwara T, Mukaisho K, Nakayama T et al (2011) Long-term proton pump inhibitor administration worsens atrophic corpus gastritis and promotes adenocarcinoma development in Mongolian gerbils infected with Helicobacter pylori. Gut 60(5):624–630. doi:10.1136/gut.2010.207662
Hannerz J, Ericson K (2009) The relationship between idiopathic intracranial hypertension and obesity. Headache 49(2):178–184. doi:10.1111/j.1526-4610.2008.01240.x
Harcourt-Brown TR, Parker JE, Granger N et al (2011) Effect of middle ear effusion on the brain-stem auditory evoked response of Cavalier King Charles Spaniels. Vet J 188(3):341–345. doi:10.1016/j.tvjl.2010.05.018
Hayes GM, Friend EJ, Jeffery ND (2010) Relationship between pharyngeal conformation and otitis media with effusion in Cavalier King Charles spaniels. Vet Rec 167(2):55–58. doi:10.1136/vr.b4886
Hu HZ, Rusbridge C, Constantino-Casas F et al (2011a) Distribution of substance P and calcitonin gene-related peptide in the spinal cord of Cavalier King Charles Spaniels affected by symptomatic syringomyelia. Res Vet Sci 93(1):318–320. doi:10.1016/j.rvsc.2011.08.012
Hu HZ, Rusbridge C, Constantino-Casas F et al (2011b) Histopathological investigation of syringomyelia in the Cavalier King Charles spaniel. J Comp Pathol 146(2–3):192–201. doi:10.1016/j.jcpa.2011.07.002
Hugnot JP, Franzen R (2011) The spinal cord ependymal region: a stem cell niche in the caudal central nervous system. Front Biosci 16:1044–1059
Javaheri S, Corbett WS, Simbartl LA et al (1997) Different effects of omeprazole and Sch 28080 on canine cerebrospinal fluid production. Brain Res 754(1–2):321–324
Kawabata A (2011) Prostaglandin E2 and pain–an update. Biol Pharm Bull 34(8):1170–1173
Knowler SP, McFadyen AK, Rusbridge C (2011) Effectiveness of breeding guidelines for reducing the prevalence of syringomyelia. Vet Rec 169(26):681. doi:10.1136/vr.100062
Lal R, Sukbuntherng J, Luo W et al (2010) Clinical pharmacokinetic drug interaction studies of gabapentin enacarbil, a novel transported prodrug of gabapentin, with naproxen and cimetidine. Br J Clin Pharmacol 69(5):498–507. doi:10.1111/j.1365-2125.2010.03616.x
Levine DN (2004) The pathogenesis of syringomyelia associated with lesions at the foramen magnum: a critical review of existing theories and proposal of a new hypothesis. J Neurol Sci 220(1–2):3–21. doi:10.1016/j.jns.2004.01.014
Lewis T, Rusbridge C, Knowler P et al (2010) Heritability of syringomyelia in Cavalier King Charles spaniels. Vet J 183(3):345–347. doi:10.1016/j.tvjl.2009.10.022
Lewis T, Swift S, Woolliams JA et al (2011) Heritability of premature mitral valve disease in Cavalier King Charles spaniels. Vet J 188(1):73–76. doi:10.1016/j.tvjl.2010.02.016
Lindvall-Axelsson M, Nilsson C, Owman C et al (1992) Inhibition of cerebrospinal fluid formation by omeprazole. Exp Neurol 115(3):394–399
Loderstedt S, Benigni L, Chandler K et al (2011) Distribution of syringomyelia along the entire spinal cord in clinically affected Cavalier King Charles Spaniels. Vet J 190(3):359–363. doi:10.1016/j.tvjl.2010.12.002
Lu D, Lamb CR, Pfeiffer DU et al (2003) Neurological signs and results of magnetic resonance imaging in 40 cavalier King Charles spaniels with Chiari type 1-like malformations. Vet Rec 153(9):260–263
Marin-Garcia P, Gonzalez-Soriano J, Martinez-Sainz P et al (1995) Spinal cord central canal of the German shepherd dog: morphological, histological, and ultrastructural considerations. J Morphol 224(2):205–212. doi:10.1002/jmor.1052240209
Marino DJ, Loughin CA, Dewey CW et al (2012) Morphometric features of the craniocervical junction region in dogs with suspected Chiari-like malformation determined by combined use of magnetic resonance imaging and computed tomography. Am J Vet Res 73(1):105–111. doi:10.2460/ajvr.73.1.105
Matiasek LA, Platt SR, Shaw S et al (2007) Clinical and magnetic resonance imaging characteristics of quadrigeminal cysts in dogs. J Vet Intern Med 21(5):1021–1026
Moritani T, Aihara T, Oguma E et al (2006) Magnetic resonance venography of achondroplasia: correlation of venous narrowing at the jugular foramen with hydrocephalus. Clin Imaging 30(3):195–200. doi:10.1016/j.clinical.2005.10.004
Motta L, Skerritt GC (2012) Syringosubarachnoid shunt as a management for syringohydromyelia in dogs. J Small Anim Pract 53(4):205–212. doi:10.1111/j.1748-5827.2011.01185.x
Naveh Y, Kitzes R, Lemberger A et al (1992) Effect of histamine H2 receptor antagonists on the secretion of cerebrospinal fluid in the cat. J Neurochem 58(4):1347–1352
Nolan AM (2000) Pharmacology of analgesic drugs. In: Flecknell PA, Waterman-Pearson A (eds) Pain management in animals. W.B. Saunders, London, pp 21–52
Parker JE, Knowler SP, Rusbridge C et al (2011) Prevalence of asymptomatic syringomyelia in Cavalier King Charles spaniels. Vet Rec 168(25):667. doi:10.1136/vr.d1726
Parrinello G, Torres D, Paterna S (2011) Salt and water imbalance in chronic heart failure. Intern Emerg Med 6(Suppl 1):29–36. doi:10.1007/s11739-011-0674-8
Pedersen HD (1996) Effects of mild mitral valve insufficiency, sodium intake, and place of blood sampling on the renin-angiotensin system in dogs. Acta Vet Scand 37(1):109–118
Pedersen HD, Haggstrom J, Olsen LH et al (2002) Idiopathic asymptomatic thrombocytopenia in Cavalier King Charles Spaniels is an autosomal recessive trait. J Vet Intern Med 16(2):169–173
Plessas IN, Rusbridge C, Driver CJ et al (2012) Long-term outcome of Cavalier King Charles spaniel dogs with clinical signs associated with Chiari-like malformation and syringomyelia. Vet Rec 171(20):501. doi:10.1136/vr.100449
Radojicic M, Nistor G, Keirstead HS (2007) Ascending central canal dilation and progressive ependymal disruption in a contusion model of rodent chronic spinal cord injury. BMC Neurol 7:30. doi:10.1186/1471-2377-7-30
Roberts T, McGreevy P, Valenzuela M (2010) Human induced rotation and reorganization of the brain of domestic dogs. PloS ONE 5(7):e11946. doi:10.1371/journal.pone.0011946
Rusbridge C (2007) Chiari-like malformation with syringomyelia in the Cavalier King Charles spaniel: long-term outcome after surgical management. Vet Surg 36(5):396–405. doi:10.1111/j.1532-950X.2007.00285.x
Rusbridge C, Jeffery ND (2008) Pathophysiology and treatment of neuropathic pain associated with syringomyelia. Vet J 175(2):164–172. doi:10.1016/j.tvjl.2006.12.007
Rusbridge C, Knowler SP (2006) Coexistence of occipital dysplasia and occipital hypoplasia/syringomyelia in the cavalier King Charles spaniel. J Small Anim Pract 47(10):603–606. doi:10.1111/j.1748-5827.2006.00048.x
Rusbridge C, MacSweeny JE, Davies JV et al (2000) Syringohydromyelia in Cavalier King Charles spaniels. J Am Anim Hosp Assoc 36(1):34–41
Rusbridge C, Greitz D, Iskandar BJ (2006) Syringomyelia: current concepts in pathogenesis, diagnosis, and treatment. J Vet Intern Med 20(3):469–479
Rusbridge C, Carruthers H, Dube MP et al (2007) Syringomyelia in cavalier King Charles spaniels: the relationship between syrinx dimensions and pain. J Small Anim Pract 48(8):432–436. doi:10.1111/j.1748-5827.2007.00344.x
Rusbridge C, Knowler SP, Pieterse L et al (2009) Chiari-like malformation in the Griffon Bruxellois. J Small Anim Pract 50(8):386–393. doi:10.1111/j.1748-5827.2009.00744.x
Rutherford L, Wessmann A, Rusbridge C et al (2012) Questionnaire-based behaviour analysis of Cavalier King Charles spaniels with neuropathic pain due to Chiari-like malformation and syringomyelia. Vet J 194(3):294–298. doi:10.1016/j.tvjl.2012.05.018
Schmidt MJ, Neumann AC, Amort KH et al (2011) Cephalometric measurements and determination of general skull type of Cavalier King Charles Spaniels. Vet Radiol Ultrasound 52(4):436–440. doi:10.1111/j.1740-8261.2011.01825.x
Schmidt MJ, Ondreka N, Rummel C et al (2012a) Volume reduction of the jugular foramina in Cavalier King Charles Spaniels with syringomyelia. BMC Vet Res 8(1):158. doi:10.1186/1746-6148-8-158
Schmidt MJ, Volk H, Klingler M, Failing K, Kramer M, et al (2013) Comparison of closure times for cranial base synchondroses in mesaticephalic, brachycephalic, and Cavalier King Charles Spaniel dogs. Vet Radiol Ultrasound 54:497–503
Sekula RF Jr, Jannetta PJ, Casey KF et al (2005) Dimensions of the posterior fossa in patients symptomatic for Chiari I malformation but without cerebellar tonsillar descent. Cerebrospinal Fluid Res 2:11. doi:10.1186/1743-8454-2-11
Serfass P, Chetboul V, Sampedrano CC et al (2006) Retrospective study of 942 small-sized dogs: prevalence of left apical systolic heart murmur and left-sided heart failure, critical effects of breed and sex. J Vet Cardiol 8(1):11–18. doi:10.1016/j.jvc.2005.10.001
Shaw TA, McGonnell IM, Driver CJ et al (2012) Increase in cerebellar volume in Cavalier King Charles Spaniels with Chiari-like malformation and its role in the development of syringomyelia. PloS ONE 7(4):e33660. doi:10.1371/journal.pone.0033660
Shaw TA, McGonnell IM, Driver CJ et al (2013) Caudal cranial fossa partitioning in Cavalier King Charles spaniels. HA Volk Veterinary Record 172(13):341–341
Stalin CE, Rusbridge C, Granger N et al (2008) Radiographic morphology of the cranial portion of the cervical vertebral column in Cavalier King Charles Spaniels and its relationship to syringomyelia. Am J Vet Res 69(1):89–93. doi:10.2460/ajvr.69.1.89
Stockyard CR (1941) The genetic and endocrinic basis for differences in form and behaviour. In: Anatomical memoirs. Wistar Institute of Anatomy and Biology, Philadelphia
Storer KP, Toh J, Stoodley MA et al (1998) The central canal of the human spinal cord: a computerised 3-D study. J Anat 192(Pt 4):565–572
Thompson DN, Harkness W, Jones BM et al (1997) Aetiology of herniation of the hindbrain in craniosynostosis. An investigation incorporating intracranial pressure monitoring and magnetic resonance imaging. Pediatr Neurosurg 26(6):288–295
Tremont-Lukats IW, Megeff C, Backonja MM (2000) Anticonvulsants for neuropathic pain syndromes: mechanisms of action and place in therapy. Drugs 60(5):1029–1052
Van Biervliet J, de Lahunta A, Ennulat D et al (2004) Acquired cervical scoliosis in six horses associated with dorsal grey column chronic myelitis. Equine Vet J 36(1):86–92
Vermeersch K, Van Ham L, Caemaert J et al (2004) Suboccipital craniectomy, dorsal laminectomy of C1, durotomy and dural graft placement as a treatment for syringohydromyelia with cerebellar tonsil herniation in Cavalier King Charles spaniels. Vet Surg 33(4):355–360. doi:10.1111/j.1532-950X.2004.04051.x
Watson PJ, Roulois AJ, Scase T et al (2007) Prevalence and breed distribution of chronic pancreatitis at post-mortem examination in first-opinion dogs. J Small Anim Pract 48(11):609–618. doi:10.1111/j.1748-5827.2007.00448.x
Wetering EJ, Lemmens KM, Nieboer AP et al (2010) Cognitive and behavioral interventions for the management of chronic neuropathic pain in adults–a systematic review. Eur J Pain 14(7):670–681. doi:10.1016/j.ejpain.2009.11.010
Williams B, Bentley J (1980) Experimental communicating syringomyelia in dogs after cisternal kaolin injection. Part 1. Morphology. J Neurol Sci 48(1):93–107
Williams B, Weller RO (1973) Syringomyelia produced by intramedullary fluid injection in dogs. J Neurol Neurosurg Psychiatry 36(3):467–477
Wong HK, Tan KJ (2002) Effects of corticosteroids on nerve root recovery after spinal nerve root compression. Clin Orthop Relat Res 403:248–252
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Rusbridge, C. (2014). Veterinary Aspects. In: Flint, G., Rusbridge, C. (eds) Syringomyelia. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-13706-8_14
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