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.
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.
Information taken from article provided to Vetstream by Denis O’ Brien, University of Missour
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.
A paper in JSAP (Black et al, 2013) characterised the phenotype of Border terriers suspected to be affected by canine epileptoid cramping syndrome via an owner survey. There were 29 Border terriers in the study most of which had had their first episode before 3 years of age. The majority of episodes lasted between 2 and 30 minutes and affected animals had difficulty in walking (27 of 29), mild tremor (21 of 29) and dystonia (22 of 29). Episodes most frequently affected all four limbs (25 of 29) and the head and neck (21 of 29). Borborygmi occurred during episodes in 11 of 29 dogs. In just over half of cases a change in diet resulted in a reduction in the frequency of 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.
Black V, Garosi L, Lowrie M, Harvey RJ, Gale J (2013) Phenotypic characterisation of canine epileptoid cramping syndrome in the Border terrier. JSAP 2013 Dec 26 (e-pub before print). doi:10.1111/jsap.12170. PMID 24372194. - PubMed -
Dr L. Garosi DVM DipECVN MRCVS RCVS and European Recognised Specialist in Veterinary Neurology – from Davies Veterinary Specialists, Hertfordshire.
Laurent Garosi is a Diplomate of the European College of Veterinary Neurology (ECVN) and RCVS/European Specialist in Veterinary Neurology. He is currently company director and head of the neurology/neurosurgery service at Davies Veterinary Specialists, England. In 2012, Laurent opened a dedicated feline neurology service which is the first of its kind in Europe. His main clinical and research interests are cerebrovascular diseases, neuroimaging and feline neurology. Laurent has published widely in the field of neurology and is a regular speaker on the national and international continuing education circuit. He is currently vice-president of the ECVN, past chief examiner of the ECVN examination committee and co-editor with Simon Platt of a recently published textbook on Small Animal Neurological Emergencies.
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