First recognized in 1997 by German veterinarian Diana Plange after a number of dogs bred by a single breeder were affected. Recognized in 1999 in the US.
Cause : unknown.
Signs : cramping episodes with normal attitude between episodes.
Treatment : symptomatic, although some dogs respond to hypoallergenic diets.
Diagnosis : usually based on history, signs and ruling out other conditions.
Prognosis : generally good as not life-threatening and episodes do not appear to progress.
Presenting signs
Shaking, cramping episodes lasting seconds to minutes.
Variable frequency weeks to months apart.
Geographic incidence
Apparently higher incidence in colder climates.
More common in Europe than in US.
Age predisposition
Typically 2-6 years for first episode.
Has been seen in dogs as young as 4 months and first episode reported in dog over 10 years.
Breed predisposition
Border terriers (inherited) .
Occasionally isolated cases reported in other breeds.
Trigger factors may include ingestion of unusual food items, changes in temperature, excitement.
Pathophysiology
Unknown. Episodes could reflect abnormal activity in the central nervous system (eg a seizure or episodic dyskinesia) or a primary muscle disease resulting in increased tone.
May be related to Hepatic Microvascular Dysplasia - affected dogs do have patches of HMVD. However, degree of HMVD is not related to the severity of signs.
?Autosomal recessive inheritance.
Timecourse (incubation, duration)
Episodes start around 2-6 years of age.
Frequency varies from weeks to months between episodes.
Arching of back or bending of body may be reported in episode.
Normal between episodes.
Clinical signs
Severe trembling, staggering - drunken appearance.
As further episodes occur muscle cramping more obvious - usually affects hind limbs and tail may curl up.
Exaggerated stretching.
Some dogs unable to stand.
Borborygmi.
Lip smacking or licking.
Episodes last seconds to 30 minutes.
No loss of consciousness.
Diagnostic investigation
Routine blood screens - all normal.
Bile acid stimulation test to rule out microvascular disorders. To accelerate the discovery of a gene(s), all owners of 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.
Confirmation of diagnosis Discriminatory diagnostic features
Diagnosis usually based on history and clinical signs. Can be very useful for client to make video of episode so that veterinarian can see the signs.
Histopathology findings
Affected dogs often do have patches of HMVD. However, degree of HMVD is not related to the severity of signs.
To accelerate the discovery of a gene(s), all owners of 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.