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Genetic Welfare Problems of Companion Animals

An information resource for prospective pet owners

Doberman Pinscher

Doberman Pinscher

Cervical Vertebral Malformation Syndrome

Related terms: cervical vertebral instability-malformation syndromes, wobbler syndrome, cervical vertebral instability, canine caudal cervical spondylomyelopathy (CCSM); cervical spondylomyopathy, cervical malformation-malarticulation, cervical spodylolisthesis, cervical spondylopathy, congenital osseous malformation

Outline: In cervical vertebral malformation syndrome, primary malformations of the neck bones along with secondary degenerative changes result in pressure on the spinal cord. This can cause chronic pain, which can be severe, and disrupts nerve signal transmission leading to loss of hind limb and, later, fore limb function. This may be difficult to treat and affected animals are likely to endure suffering prior to treatment and this may persist after treatment. It is quite common in Dobermans – one survey found that about 5% were affected - and there is no way to know which puppies will become affected by the disease. Some may feel that, unless the condition can be eliminated, perpetuating a breed in which a proportion of animals are likely to suffer chronic pain and/or disability is not justifiable.


Summary of Information

(for more information click on the links below)

1. Brief description

Cervical vertebral malformation syndrome is a multifactorial condition resulting from various primary developmental abnormalities of the vertebral neck bones which then predispose secondary degenerative changes that in turn narrow the spinal canal (spinal canal stenosis) and compress the spinal cord (VanGrundy 1989).

Cervical vertebral malformations are common in Great Danes and Doberman pinschers but they are also seen in other breeds of dogs, especially the larger breeds (which suggests that the disease may have arisen in association with selection for large size). Different breeds get different patterns of the disease.

Dobermans are typically middle-aged when they become affected. They most commonly get ventral compression of the spinal cord at the junction between cervical vertebral bones C5/ C6 or C6/C7. This compression is caused either by protrusions of the intervertebral discs that are found between the bones of the veterbrae, or by hypertrophy (thickening) of the annulus fibrosus (the outer layer of the intervertebral disc) (Dixon et al 1996, Cappello et al 1997, McKee et al 1999, Bagley 2002). The spinal canal stenosis that is also common in these animals compounds the condition by increasing the pressure on the spinal cord (Raffe & Knecht 1980, Seim and Withrow 1982, Lewis 1989). The Doberman’s head and neck conformation may cause increased stress in the caudal cervical vertebrae and this may be the reason for the prevalence of the condition in this breed (Lewis 1992, Sharp et al 1995). The disc degeneration and protrusion may result from vertebral instability or primary disc degeneration (Hansen type II disc protrusion) (Seim 2000). In this type of cervical malformation, male Dobermans are affected twice as often as females (Seim 2000).

In most Dobermans (65%) the condition is slowly progressive but, in some (20%), the disease starts slowly and then there is a sudden acute worsening of signs. A smaller proportion (15%), have sudden onset of signs with few previous problems (Seim & Withrow 1982).

Pressure on the spinal cord can result in dysfunction of the hind legs and this may progress to also affect the forelegs. Affected dogs often hold their head down as this tends to relieve pressure on the spinal cord and the dog may feel less pain in this position. Characteristically, the dogs start becoming ‘wobbly’ or ataxic on their hind legs, adopt a wide hind limb stance and scuff or drag the hind limbs when moving. Stiff abnormal gait in the forelegs may develop. The degree of nerve damage varies from none to severe, causing paraplegia (loss of use of the hind limbs) and progressing to tetraplegia (loss of use of all four legs).

2. Intensity of welfare impact    

The pain caused by compression of the spine can be severe and persistent, but not all affected Dobermans seem to suffer it. Relief of this pain can be difficult. The limb dysfunction affects locomotion and normal behaviours and significantly affects the quality of life. Interventions to try to treat the disease may also have adverse welfare consequences.

3. Duration of welfare impact

Cervical malformation syndrome in Dobermans usually develops in middle age, but it may be seen in dogs as young as 18 months, and it is progressive (Tomlinson 2001). The syndrome causes disability and can cause long-lasting pain. Although veterinary interventions may be successful, such interventions themselves often involve significant welfare challenges: from repeated visits to the vets, repeated administration of anaesthetics, side effects from drug treatments, and the impact of major spinal surgery. Euthanasia may be required.

4. Number of animals affected

It has been reported that 80% of the cervical vertebral malformation cases seen in dogs are in Great Dane and Doberman pinscher breeds. It is recognised as a disease of Dobermans but there are few published data on its prevalence in the breed. Da Costa (2011) suggested, from a recent survey of the Veterinary Medical Database in the USA, that 5.5% of Dobermans were affected.

5. Diagnosis

Damage to the spine in the neck through other causes may cause identical signs to this disease so further tests are needed to confirm the condition and to assess its severity (and the likelihood of a response to treatment). These include radiography (x-rays), myelography (radiography after injecting a dye into the spinal canal), computer tomography (CT) and magnetic resonance imaging (MRI) scans.

6. Genetics

That this disease is highly breed specific shows that there is a genetic basis to it but currently the genetics are unknown.

7. How do you know if an animal is a carrier or likely to become affected?

The genetic basis of this syndrome has not yet been determined. Currently there is no way to know which Doberman puppies will become affected.

8. Methods and prospects for elimination of the problem

Affected individuals, or those that have produced affected offspring, should not be used for breeding. It has also been suggested that animals should not be used for breeding if any of their close relatives have been affected. Unfortunately dogs may have already been bred by the time of diagnosis.

Further progress in controlling this condition in Dobermans may come with elucidation of the genetics or other methods to identify animals at risk of developing the disease from an early age.


For further details about this condition, please click on the following:
(these link to items down this page)


1. Clinical and pathological effects

Cervical figure 1

Figure 1. The sections of the canine spine.

Dogs have seven cervical vertebrae bones in the neck (numbered 1-7 starting from the head). The spinal cord runs through these bones in the spinal canal and is protected by them. The spinal cord is made up of delicate nerve tissue which transmits sensory and motor information between the brain and other parts of the body (excluding the head). Between each pair of vertebrae, spinal nerves exit the spinal cord to innervate sections of the neck and body. Intervertebral discs are found between the vertebrae, starting between the second and third cervical vertebrae (C2-3) and extending throughout most of the spine. Each intervertebral disc is a highly specialised structure, designed to act as a mini shock absorber for the spinal column. Normal intervertebral discs consist of two parts: a central gelatinous part, called the nucleus pulposus (NP), which is contained within an outer fibrous layer, called the annulus fibrosus (AF). Intervertebral discs sit between the bodies of their adjacent vertebrae just ventral (below) the spinal canal. When surrounding tissues are damaged, spinal nerve tissue may easily become damaged also resulting in pain and sometimes spinal cord dysfunction (neurological disease).

Cervical figure 2

Figure 2. Diagram of the vertebral column. The intervertebral discs are located between the vertebrae. Each disc consists of an outer layer (the annulus fibrosus) and an inner core (the nucleus pulposus). (Image property of Southern Counties Veterinary Specialists, to whom we are grateful for permission to reproduce it here).

Cervical vertebral malformation syndrome is a multifactorial condition resulting from various primary developmental abnormalities which predispose affected animals to other, secondary degenerative changes that in turn narrow the spinal canal (ie causing spinal canal stenosis) and compress the spinal cord (VanGrundy 1989).

Cervical vertebral malformations are common in great Danes and Doberman pinschers but they are also seen in other breeds of dogs, especially the larger breeds (which suggests that the disease may have arisen in association with selection for large size). There are differences in the types of disease seen in various breeds regarding which vertebrae are typically affected and the types of malformation that occur.

Dobermans are typically middle-aged when they become affected. They most commonly get ventral compression of the spinal cord at the junction between cervical vertebral bones C5/ C6 or C6/C7. This compression is caused either by protrusions of the intervertebral discs that are found between the bones of the veterbrae, or by hypertrophy (thickening) of the annulus fibrosus (the outer layer of the intervertebral disc) (Dixon et al 1996, Cappello et al 1997, McKee et al 1999, Bagley 2002). The spinal canal stenosis that is also common in these animals compounds the condition by increasing the pressure on the spinal cord (Raffe & Knecht 1980, Seim and Withrow 1982, Lewis 1989). The Doberman’s head and neck conformation may cause increased stress in the caudal cervical vertebrae and this may be the reason for the prevalence of the condition in this breed (Lewis 1992, Sharp et al 1995). The disc degeneration and protrusion may result from vertebral instability or primary disc degeneration (Hansen type II disc protrusion) (Seim 2000). Dobermans are typically middle-aged when they become affected, in contrast to the situation in great Danes in which younger animals are affected. IN Dobermans, ventral compression of the spinal cord typically occurs at C5/C6 or C6/C7, as a result of disc protrusion or hypertrophy (thickening) of the annulus fibrosus (Dixon et al 1996, Cappello et al 1997, McKee et al 1999, Bagley 2002). Additional spinal canal stenosis is common in these animals which compounds the condition (Raffe & Knecht 1980, Seim & Withrow 1982, Lewis 1989). The Doberman’s head and neck conformation may cause increased stress in the caudal cervical vertebrae and the high prevalence in the breed may be because of this (Lewis 1992, Sharp et al 1995). The disc degeneration and protrusion may result from vertebral instability or from primary disc degeneration (Hansen type II disc protrusion) (Seim 2000). With disc protrusion, the AP of the disc gets protruded through the AF into the spinal canal causing narrowing of the space and compression of the spinal cord. In this type of cervical malformation male Dobermans are affected twice as often as females (Seim 2000).

Cervical figure 3

Figure 3. Vertebral disc protrusion can result in pressure on and constriction of the spinal cord as the inner core (red) of the disc protrudes through the outer layer in to the spinal canal. This can occur in Dobermans because of the head and neck conformation of these dogs.  (Image property of Dr Phil Witte, to whom we are grateful for permission to reproduce it).

Other forms of cervical malformation seen in the breed include congenital osseus malformation and vertebral tipping (Seim 2000). Congenital osseus malformation can occur anywhere in the neck and in this condition the shape of the affected vertebra, or vertebrae (usually more than one bone is affected), is abnormal from birth resulting in narrowing of the spinal canal and compression of the spinal cord. The pressure on the cord may occur only when the neck is held in particular positions and the compression may be from any direction (eg side to side or top to bottom).

Cervical figure 4

Figure 4. An MRI scan of a dog with deformed vertebrae and intervertebral disc protrusion (blue arrow) causing severe spinal cord compression.

 Cervical figure 5

Figure 5. A CT-myelogram showing how deformities of the vertebrae, collapse of the intervertebral disc space and disc protrusion all contribute to the narrowing of the spinal canal and onset of the condition.

(Figures 4 & 5 are the property of Willows Veterinary Centre and Referral service, to whom we are grateful for permission to reproduce them here).

Vertebral tipping is characterised by the cranial dorsal (front top) part of the vertebral body tipping upwards causing narrowing of the spinal canal and, in some cases, compression of the spinal cord. This may occur secondary to instability created by degenerative disc disease or may be a primary problem that itself predisposes to disc displacement (Seim 2000).

The pressure on the spinal cord that occurs in these conditions can cause pain, nerve dysfunction or both. About 40% of affected dogs show obvious signs of pain (Seim 2000) but others may show changes in behaviour and the way in which they hold their bodies in order to avoid pain. The effects of pressure on transmission of nerve signals in the spine usually shows as dysfunction of the hind legs. This may progress to also affect the fore legs. Usually the dysfunction starts insidiously but they can appear suddenly, sometimes after minor trauma of a sort that would not cause a problem to a normal dog (Seim 2000).

Affected dogs often hold their heads low as this tends to relieve pressure on the spinal cord and lessen the pain (Seim 2000).

The degree of nerve damage varies from none to severe, causing paraplegia (loss of use of the hind limbs) and progressing to tetraplegia (loss of use of all four legs). Affected dogs tend to have a wide stance, particularly of the hindlimbs, and to have problems turning and getting into position for toileting. They may move in an ‘exaggerated’ way, seeming wobbly (hence the name wobbler’s syndrome) and ataxic on their hind limbs with a stiff or choppy movement of the forelimbs. Their claws may be worn unevenly due to gait abnormalities (Lewis 1992). Muscle atrophy (decrease in size) often occurs in affected limbs (McKee 2007).

Cervical figure 6

Figure 6.  A Doberman with a moderate-severe case of cervical malformation syndrome, holding the head low to relieve pain.
(Image property of
www.vetneurochesapeake.com to whom we are grateful for permission to reproduce it here).

 

Cervical figure 7

Figure 7. A wide stance and lack of control over the hind legs (and in some cases of the fore legs also) are also characteristic signs of cervical pain and cervical vertebral malformation syndrome.
(Image property of Willows Veterinary Centre and Referral service, to whom we are grateful for the permission to reproduce them here).

In most Dobermans (65%) the condition is slowly progressive but, in some (20%), the disease starts slowly and then there is a sudden acute worsening of signs. In a smaller proportion (15%) there is a sudden onset of signs with few previous problems (Seim & Withrow 1982).

Surgical treatments to attempt stabilisation of the vertebral column or to decompress the spinal cord and eliminate the narrowing are often recommended. There are various techniques employed and some controversy as to what the best treatments options are for each of the different forms of this syndrome. It is recommended that specialist advice is sort from a veterinary orthopaedic surgeon. The prognosis for each affected individual varies with the severity of their condition, the underlying cause and the treatment offered. Those animals which do not have surgery generally have a guarded prognosis; surgery can improve the prognosis but is not without its complications and may not bring about a cure.

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2. Intensity of welfare impact

The pain caused by compression of the spine can be severe and persistent but this is not present in all affected Dobermans. Relief of the pain can be difficult. The limb dysfunction affects locomotion and behaviour and may significantly decrease the dog’s quality of life.

Interventions to try to treat the disease may also have adverse welfare consequences due to the stress of repeat veterinary visits, handling, procedures and medications. Recurrence of the problems can occur following surgery.

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3. Duration of welfare impact

The syndrome can cause long-lasting pain and disability and is usually progressive. In Dobermans it often appears when they are middle-aged to older - most are between four and eight years of age at presentation (Tomlinson 2001, Bagley 2002), but they may be as young as eighteen months (Tomlinson 2001). Although veterinary interventions may be successful, such interventions themselves may involve significant welfare challenges. The outlook for dogs having only conservative (non-surgical) treatments is considered guarded to poor (Seim 2000, Jeffery & McKee 2001), though Jeffery & McKee (2001) suggest that actually there is a lack of information on the natural progression of the disease as typically seen in Dobermans.

Some dogs may experience multiple and major interventions and still require euthanasia.

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4. Number of animals affected

Eighty percent of cases of cervical vertebral malformations in dogs occur in Great Danes and Doberman pinschers (Bruecker et al 1989, Lewis 1992). It is recognised as a disease of Doberman but currently there is little published data on the prevalence within the breed. Da Costa (2011) suggested, from a recent survey of the Veterinary Medical Database in the USA, that 5.5 % of this population of Dobermans were affected.

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5. Diagnosis

A veterinary surgeon will suspect cervical vertebral malformation syndrome when presented with a Doberman showing the characteristic signs of the disease. Care is needed, because these signs can also be caused by other diseases or injuries (McKee 2007), and veterinary manipulation of the head and neck can lead to collapse (Lewis 1992). Further diagnostic tests are needed to diagnose these conditions and their severity (and the likelihood of response to treatment (Seim 2000). These include radiography (x-rays), myelography, computer tomography (CT) and magnetic resonance imaging (MRI) scans. Some of these diagnostic techniques are only available at referral centres.

Radiographs are only able to show the size and position of the bones. Because the disease is a result of instability in the positions of the neck vertebrae, and their positions alter with the posture of the head and body, it is often necessary to take a series of radiographs with the dog in different postures (Lewis 1992).

Myelography has been the standard technique used for diagnosis (Lewis 1992). This technique involves injection of a dye into the spinal canal that is visible on radiographs to reveal areas of compression of the spinal cord. This is potentially dangerous but necessary in some cases, although the technique has been partially replaced by magnetic resonance imaging (MRI) scans (Lipsitz et al 2001, Penderis & Dennis 2004, McKee 2007).

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6. Genetics

Cervical vertebral malformation is highly breed specific and this indicates that it has a genetic basis (Bruecker et al 1989, Lewis 1992, Drost et al 2002, Berry 2003). However the genetics of this disease have not been fully investigated. It has been suggested that environmental factors may also play a part (Hazewinkel et al 1985, Lewis 1992, Seim and Briecker 1993, Olby 2003). Currently, studies are underway at the College of Veterinary Medicine, Ohio State University, to try and elucidate the genetics of this condition in the Doberman (da Costa 2011).

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7. How do you know if an animal is a carrier or likely to become affected?

The genetic basis of this syndrome has not yet been determined. Currently there is no way to know which Doberman puppies will become affected. However, as genes are considered to play a role, choosing a puppy whose parents and other ancestors are free of the disease seems logical.

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8. Methods and prospects for elimination of the problem

It has been suggested that diseases with an unknown mode of inheritance may be controlled using the approaches employed in tackling polygenic (multiple gene) disorders (Bell 2010). Affected animals, and those that have produced affected offspring, should not be used for breeding (Shell 2003). Avoiding breeding from animals with affected close relatives is also likely to help eliminate this disease. However, many Dobermans do not show signs until after breeding age.

This breed is predisposed to a number of other major genetic conditions with significant welfare impacts such as von Willibrand’s disease, hypothyroidism and dilated cardiomyopathy, so any breeding programme aimed at reducing the prevalence of cervical vertebral malformation needs to take account of these and other genetic conditions so that the general health of the breed is improved and that efforts to tackle one problem do not risk exacerbating others.

Advances in understanding of the genetics of the disease are likely to help in tackling the problem, especially if these lead to methods for detection, prior to breeding, of animals that may have abnormal genes.

Some may consider that perpetuating a breed in which a proportion of animals are likely to suffer chronic pain and/or disability, which can be severe, is not justifiable.

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9. Acknowledgements

UFAW is grateful to Rosie Godfrey BVetMed MRCVS and David Godfrey BVetMed FRCVS for their work in compiling this section and to Stephanie Kaufman for assistance in illustrating it.

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10. References

Bagley R (2002) Spinal Instability In Dogs. AmericanCollege of Veterinary Internal Medicine Forum proceedings 24th May 2002

Bell JS (2010) Genetic Testing and Genetic Counseling in Pet and Breeding Dogs. World Small Animal Veterinary Association World Congress Proceedings

Berry WL (2003) Cervical Myelopathy: Large Breed Dogs. Western Veterinary Conference

Bruecker KA, Seim HB and Blass CE (1989) Caudal cervical spondylomyopathy: decompression by linear traction and stabilization with Steinmann pins and polymethyl methacrylate. Journal of the American Animal Hospital Association 25: 677-682

Cappello R, McEvoy FE, Wheeler SJ and Rusbridge C (1997) Clinical and radiographical findings in caudal cervical spondylomyelopathy in Dobermann pinschers: 42 cases. Proceedings of the WSAVA, BSAVA 8, FECAVA World Congress, Birmingham, April. P 234

Da Costa R (2011) Wobbler’s syndrome, College of Veterinary Medicine, Ohio State university. On-line. http://vet.osu.edu/wobbler-syndrome. Accessed 23.5.11

Dixon BC, Tomlinson JL and Kraus KH (1996) Modified distraction-stabilisation technique using an interbody polymethyl methacrylate plug in dogs with caudal cervical spondylomyelopathy. Journal of the American Veterinary Medical Association 208: 61-68

Drost WT, Lehenbauer TW and Reeves J (2002) Mensuration of cervical vertebral ratios in Doberman pinschers and Great Danes. Veterinary Radiology and Ultrasound 43: 124–131

Hazewinkel HAW, Goedegebuure SA, Poulos PW and WTC Wolvekamp (1985) Influences of chronic calcium excess on growing Great Danes. Journal of the American Animal Hospital Association 21: 377-91

Jeffery ND and McKee WM (2001) Surgery for disc-associated wobbler syndrome in the dog-an examination of the controversy. Journal of Small Animal Practice 42: 574-581

Lewis DG (1989) Cervical spondylomyelopathy (‘wobbler’ syndrome) in the dog: a study based on 224 cases. Journal of Small Animal Practice 30: 657-66

Lewis D (1992) Cervical spondylomyelopathy (`wobbler syndrome') in dogs. In Practice 14: 125-130

Lipsitz D, Levitski RE, Chauvet AE and Berry WL (2001) Magnetic resonance imaging features of cervical stenotic myelopathy in 21 dogs. Veterinary Radiology and Ultrasound 42: 20–27

McKee M (2007) Lameness and weakness in dogs: is it orthopaedic or neurological? In Practice 29: 434-444

McKee WM, Butterworth SJ and Scon HW (1999) Management of cervical spondylopathy-associated intervertebral disc protrusions using 7.5 mm and 6.0 mm intervertebral metal washers in 78 dogs. Journal of Small Animal Practice 40: 465-472

Olby NJ (2003) Update on Canine Wobbler Surgery. Proceeding of the American College of Veterinary Internal Medicine

Penderis J and Dennis R (2004) Use Of Traction During Magnetic Resonance Imaging Of Caudal Cervical Spondylomyelopathy (“Wobbler Syndrome”) In The Dog. Veterinary Radiology and Ultrasound 45: 216–219

Raffe MR and Knecht CD (1980) Cervical vertebral malformation - a review of 36 cases. Journal of the American Animal Hospital Association 16: 881- 883

Sampson J (2006) What is required for breeding programmes or molecular technologies to make impact on the prevalence and incidence of elbow dysplasia in dogs? Proceedings of the British Veterinary Orthopaedics Association Autumn Meeting 2006: 4-5

Seim HB (2000) Diagnosis and treatment of cervical vertebral instability-malformation syndrome. In Bongura JD ed Kirk’s Current Veterinary Therapy XIII. pp992 W.B. Saunders: Philadelphia

Seim HB and Withrow SJ (1982) Pathophysiology and diagnosis of caudal cervical spondylomyelopathy with emphasis on the dobermann pinscher. Journal of the American Animal Hospital Association 18: 241-251

Sharp NJH, Cofone M, Robertson ID, Decarlo A, Smith GK and Thrall DE (1995) Computed

tomography in the evaluation of caudal cervical spondylomyelopathy of the Dobermann. Veterinary Radiology and Ultrasound 36: 100-108

Shell L (2003) Cervical Instability. On-line VIN Associate accessed 14.11.2011

VanGrundy TE (1989) Canine wobbler syndrome Part I.  pathophysiology and diagnosis. Compendium on Continuing Education for the Practicing Veterinarian 12: 144-157

© UFAW 2011