This condition was first recognised in 1997 by a German veterinarian Diana Plange when a number of dogs bred by a single breeder were affected. It was first recognised in the US in 1999. Affected animals present with shaking and cramping episodes that last from a few seconds to minutes. Owners may report arching of back or bending of body during episode. These episodes occur with a variable frequency from weeks to months apart but animals are clinically normally between.
Typically affects animals between 2 and 6 years old for first episode. However has been seen in dogs as young as 4 months and first episode reported in dog over 10 years. Severity of episodes tends to remain constant throughout life ie no progression of signs.
Reported clinical signs include:
• Severe trembling, staggering – drunken appearance.
• Muscle cramping (as further episodes occur this becomes more obvious
- usually affects hind limbs and tail may curl up).
• Exaggerated stretching.
• Some dogs are unable to stand.
• Lip smacking or licking.
• Episodes last from a few seconds to 30 minutes.
• No loss of consciousness.
Causes and risk factors
No cause has been identified. There is apparently a higher incidence in colder climates and condition is more common in Europe than in US. CECS is inherited in Border terriers but occasionally isolated cases are reported in other breeds. Thought to exhibit autosomal recessive inheritance.
Episodes may reflect abnormal activity in the central nervous system (e.g. a seizure or episodic dyskinesia) or a primary muscle disease resulting in increased tone. The condition may be related to Hepatic Microvascular Dysplasia as affected dogs do have patches of HMVD - however, the degree of HMVD is not related to the severity of signs.
Diagnosis is usually based on history and clinical signs (it can be very useful for client to make video of an episode so that veterinarian can see the signs). Routine blood screens are usually all normal. A bile acid stimulation test should be performed to rule out microvascular disorders as these may have similar presentation and occur in similar breeds.
To accelerate the discovery of a causative gene(s), owners of all CECS dogs are encouraged to submit blood for DNA to the University of Missouri’s Canine Epilepsy Network to be used in research into the cause of the disease.
Differential diagnosis to be considered include:
• Microvascular disorders.
• Back pain.
• Irritable bowel syndrome (for abdominal form).
Diazepam or chlorazepate can be given to alleviate cramps and buscopam may help intestinal cramping. Some dogs respond well to hypoallergenic or gluten free diets and may become asymptomatic.
Investigation is being carried out at department of Clinical Sciences of Companion Animals at the Utrecht University, the Netherlands. If you have a dog with this condition a questionnaire can be completed at http://www.uu.nl/faculty/veterinarymedicine/nl/dierenklinieken/ukg/dierenarts/onderw_onderz/onderzoek/neurologie/krampaanvallen2/Pages/default.aspx
Information taken from article provided to Vetstream by Denis O’ Brien, University of Missour
Vets at Davies Veterinary Specialists are looking for Border terriers with suspected canine epileptoid cramping syndrome and Scottie dogs with suspected Scottie cramp to take part ina study to help our understanding of these diseases. Blood samples will be collected from these dogs for DNA typing. Identification of a DNA marker for these conditions may help us to identify the cause of these disorders, accurately diagnose them and advise breeders in their breeding programs.
A syndrome known as canine epileptoid cramping syndrome (also known as Spike’s disease) has been observed in Border Terriers. Episodes consist of gait abnormalities ranging from ataxia to an inability to stand, contractions of abdominal, neck and back muscles resulting in abnormal posturing and contractions/cramping of the appendicular muscle (extensor rigidity or flexion of the limbs). Duration of the episodes can vary from seconds to half an hour or longer, during which the dog remains aware of his surroundings. Increased intestinal motility is suspected based on hearing borborygmus. Affected dogs may experience pain during the episodes.
Scottie cramp is a syndrome observed in young adult Scottish Terriers consisting of involuntary sustained muscle contractions primarily affecting the hind limbs. With excitement, the hind limbs assume a hypertonic, extended position or they may occasionally display exaggerated flexion of the limbs. The forelimbs become abducted and develop increased extensor tone. Affected dogs progressively develop a stiff stilted gait over a few minutes. Severely affected dogs assume an arched posture over their back and may fall into lateral recumbency with their head and tail flexed.
If you have a case of suspected canine epileptoid cramping syndrome or of suspected Scottie cramp, please contact Dr Laurent Garosi at Davies Veterinary Specialists (email@example.com or telephone 01582 883950).
Dr L. Garosi DVM DipECVN MRCVS RCVS and European Recognised Specialist in Veterinary Neurology – from Davies Veterinary Specialists, Hertfordshire.
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