Genetic Welfare Problems of Companion Animals

An information resource for prospective pet owners

Border Terrier

Border Terrier

Canine Epileptoid Cramping Syndrome

Related terms: partial motor seizures, Spike’s disease

Outline: Dogs with canine epileptoid cramping syndrome have episodes of painful muscle and intestinal cramps. Episodes last from a few seconds to up to about 30 minutes, and may be rare or occur frequently. Such cramping typically first starts to occur in dogs aged between 2 and 6 years and will then persist throughout the affected animal’s life. It appears to be a genetic disease but rather little is known about it.


Summary of Information

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1. Brief description

Canine epileptoid cramping syndrome or CECS is a recently recognised condition of Border terriers. Currently the cause is unclear, although it is thought to be hereditary. Theories as to possible causes of the syndrome include that it may be due to:

  • partial motor seizures;
  • metabolic disease;
  • a muscular disorder episodically affecting muscle function and tone; or
  • hepatic microvascular dysplasia (O’Brien no date).

Signs shown by affected individuals vary widely. All appear normal between episodes. Some experience only a few mild episodes, during which they show loss of muscle co-ordination (ataxia), stagger and tremble (Leitchty and Blake 2005). Others may show exaggerated stretching and lip licking. More severely affected dogs experience cramping of the muscles of the body and limbs. Ripples of muscle contractions may be seen. Such dogs may fall over and remain recumbent. Some dogs appear to suffer cramping of the intestinal muscles, causing signs of abdominal pain and excessive abdominal noises (Leitchty and Blake 2005, O’Brien no date). The animals always remain conscious and responsive throughout the episodes in this disease. In most cases, the episodes are self-limiting and last from a few seconds to 30 minutes.

O’Brien (no date) suggested that the condition is non-progressive whilst others consider that, in some dogs, the frequency and duration of the episodes tend to increase (Leitchty and Blake 2005). Treatment with low protein or hypoallergenic diets and various drugs has been suggested and have anecdotally been found to be useful (www.borderterriers-CECS.com).

2. Intensity of welfare impact

During episodes of cramping, it seems reasonable to assume that the cramps cause significant pain (as muscle and intestinal cramps can cause severe pain and discomfort in humans). It is possible that the pain might lead to confusion and fear, but this is more speculative. Treatment can help decrease the incidence and severity of episodes but the drugs used have potential side effects and welfare implications.

3. Duration of welfare impact

Episodes generally start occurring at between two to six years of age, but sometimes as early as 4 months of age (O’Brien no date). An episode lasts from a few seconds to 30 minutes. Some individuals only ever have one or two episodes during their life, others have recurrent episodes every few days, weeks or months that persist throughout life.

4. Number of animals affected

Surveys in the UK, Germany and the USA, have indicated that between 5 and 15% of border terriers are affected (Leitchty and Blake 2005).

5. Diagnosis

A definitive diagnosis of CECS involves ruling out all other possible causes of the signs. A wide range of diagnostic tests may be needed.

6. Genetics

It has been proposed that CECS has an autosomal recessive mode of inheritance but this has yet to be confirmed (O’Brien no date).

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

It is not possible to identify individuals that will later develop the condition. It is not known whether the abnormal genes that cause the condition can be carried by dogs which themselves are unaffected by the disease.

8. Methods and prospects for elimination of the problem

In conditions such as this, where the mode of inheritance is unknown, animals with affected relatives (including siblings and siblings of parents), ideally, should not be used for breeding (Bell 2010). With the current state of knowledge it is difficult to give further advice.

Affected dogs should not be used for breeding, but the condition may not show until after breeding age has been reached. Research into the genetics of CECS, and towards developing a genetic test for the disease, is currently underway (see below for details).

 

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1. Clinical and pathological effects

Canine epileptoid cramping syndrome or CECS has only recently been recognised as a distinct clinical entity. In the past, cases may have been diagnosed as canine epilepsy. It has been recognised in Border terriers in the UK, mainland Europe and the USA.

The cause of the disease is currently unclear, but it is thought to be a hereditary condition. There are various theories about the cause and these are outlined below.

1. Partial motor seizure. Rusbridge (no date) suggested that CECS is caused bypartial motor seizures. Seizures are uncontrolled, random, over excitation of parts of the brain, with excessive firing (depolarisation) of the nerve cells (neurons). They usually last between a few seconds and a few minutes. They are commonly called fits or convulsions when there is widespread abnormal excitation in the brain and in these cases the animal becomes unconscious.  In partial or focal seizures only one specific part of the brain is affected and the animal remains conscious. Seizures which just affect the animal’s movements are called partial motor seizures and it may be that these are the cause of CECS.

2. Metabolic disease. It has been suggested that CECS may have a metabolic cause because elevated pyruvate and lactate levels (products of metabolism) have been reported in the blood and urine of affected dogs (http://www.borderterrier-cecs.com).

3. It has been suggested that CECS may be a muscular disorder which episodically affects muscle function and tone.

4. Another possible cause is liver dysfunction caused by hepatic microvascular dysplasia (O’Brien no date). In this disease, small blood vessels of the liver do not develop correctly and the liver is unable to function properly. There may be a build up of harmful toxins which can affect brain function leading to seizures and other signs of brain disease (Tobias 2006). This condition is seen in terriers of various breeds. It can only be diagnosed by examination of a liver biopsy. Dogs with CECS may have patches of hepatic microvascular dysplasia in their livers but the severity of CECS was not found to be linked to the severity of the hepatic microvascular dysplasia (O’Brien no date), so it does not seem likely that this is generally the cause of CECS.

In between episodes of cramping affected dogs behave and appear normal. During episodes, the signs shown by affected dogs varies widely. Some experience only a few mild episodes, during which they show loss of muscle co-ordination (ataxia), stagger and tremble (Leitchty and Blake 2005). Others may show exaggerated stretching and lip licking. More severely affected dogs experience cramping of the muscles of the body and limbs. Ripples of muscle contractions may be seen. Such dogs may fall over and remain recumbent. Some dogs appear to suffer cramping of the intestinal muscles, causing signs of abdominal pain and excessive abdominal noises (Leitchty and Blake 2005, O’Brien no date). The animals always remain conscious and responsive throughout the episodes in this disease. In most cases, the episodes are self-limiting and last from a few seconds to 30 minutes. Only occasionally will veterinary intervention will need to be sought during an episode (www.borderterriers-CECS.com).

Certain “trigger” factors may play a role in the induction of episodes. Certain foods and other substances eaten by the dogs have been blamed, as have excitement and changes in environmental temperature (O’Brien no date). Stress inducing events such as rehoming have been implicated, as has a sudden increase in exercise (Leitchty and Blake 2005). There seems to be a higher incidence in colder climates and in Europe compared to the USA (O’Brien no date).

O’Brien (no date) suggested that the condition is non-progressive but others consider that, in some dogs, the frequency and duration of the episodes tend to increase (Leitchty and Blake 2005). Treatment with low protein or hypoallergenic diets and various drugs has been suggested and have anecdotally been found to be useful (www.borderterriers-CECS.com).

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2. Intensity of welfare impact

During episodes of cramping, it seems reasonable to assume that the cramps cause significant pain (as muscle and intestinal cramps can cause severe pain and discomfort in humans). It is possible that the pain might lead to confusion and fear, but this is more speculative. Treatment can help decrease the incidence and severity of episodes but the drugs used have potential side effects and welfare implications.  

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3. Duration of welfare impact

Episodes generally start occurring at between two to six years of age, but sometimes as early as 4 months of age (O’Brien no date). An episode lasts from a few seconds to 30 minutes. Some individuals only ever have one or two episodes during their life, others have recurrent episodes every few days, weeks or months that persist throughout life.

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4. Number of animals affected

Surveys in the UK, Germany and the USA, have indicated that between 5 and 15% of border terriers are affected (Leitchty and Blake 2005).

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5. Diagnosis

The diagnosis of CECS is currently one of exclusion of other causes so the attending vet may wish to run blood and urine tests, carry out a full neurological examination and possibly do further tests to check for causes of epilepsy including electric recordings of the brain or MRI scans. A range of tests such as x-rays, endoscopy etc. may be needed to assess for intestinal and back disease. The condition is easily confused with epilepsy, back problems and even gut problems such as irritable bowel disease (Leitchty and Blake 2005).

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6. Genetics

It is thought that this disease has a genetic basis because of its occurrence in certain bloodlines of this breed (Leitchy and Blake 2005), and it has been suggested that it may be due to an autosomal recessive gene (Leitchty and Blake 2005). However, this has not been confirmed and the mode of inheritance is not currently known.

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

There is no test to identify individuals which will develop the condition when older. It is recommended not to purchase puppies from affected parents or from parents that have previously produced affected offspring. It seems likely that carrier animals exist, that is, that some animals may carry the abnormal gene without developing the disease themselves. 

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8. Methods and prospects for elimination of the problem

In conditions such as this, where the mode of inheritance is unknown, animals with affected relatives (including siblings and siblings of parents) should ideally not be used for breeding (Bell 2010). With the current state of knowledge it is difficult to give further advice.

Affected dogs should not be used for breeding, but the condition may not show until after breeding age has been reached. Research into this condition is being carried out at the University of Utrecht, in the Netherlands and the University of Missouri, USA.  Owners of affected dogs can help in this research by filling in questionnaires available at http://www.vet.uu.nl/site/viavet/html/enquete_en_voor or submitting blood samples to the University of Missouri see www.borderterriers-CECS.com for details. Hopefully these research projects will help shed light on how this condition can be eliminated.

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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

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10. References

Bell JS (2010) Genetic Testing and Genetic Counselling in Pet and Breeding Dogs. Proceedings of World Small Animal Veterinary Association World Congress 2nd - 5th June, 2010 Geneva. WSAVA: Ontario, Canada

Leitchty M and Blake K (2005) Canine epileptoid cramping syndrome in border terriers. National Border Terrier Specialty CECS Seminar. 6th June 2005 Available at http://www.borderterrier-cecs.com/cecs_handout.htm. Accessed 28.2.11.

O’Brien D no date. Canine epileptoid cramping syndrome. (On-line) Available at http://www.vetstreamcanis.com/ACI/November08/VMD1/dis02955.asp. Accessed 28.2.11

Rusbridge C nodate Seizures and epilepsy. (On-line) Available at http://www.veterinary-neurologist.co.uk/seizures_epilepsy.htm. Accessed 24.2.11

Shell L (2003) Epilepsy and seizures, general. Available from VIN Associate. Accessed 18.1.11.

Tobias K (2006) Hepatic portal dysplasia or portal atresia. (On-line) Available at http://www.acvs.org/AnimalOwners/HealthConditions/SmallAnimalTopics/HMD/. Accessed 28.2.11

www.borderterriers-CECS.com. Accessed 28.2.11

© UFAW 2011


Credit for main photo above:

http://depositphotos.com/33769317/stock-photo-border-terrier-on-a-green-grass-lawn.html ©Depositphotos.com/CaptureLight