Canine Epileptoid Cramping Syndrome









history of Canine Epileptoid Cramping Syndrome (CECS):



In 1997, Diana Plange, a German veterinarian and Border Terrier Breeder (Malepartus) received several phone calls from anxious Border Terrier owners whose dogs Ms. Plange had bred. They were observing epileptic-like problems in their dogs. However, many of the presentations did not fit the classic form of epilepsy. So after taking a further health survey, she decided to give up on her own breeding program and started working to find the cause of this condition, which was epileptoid in character.

Ms. Plange arranged to have more than 100 Border Terriers examined thoroughly in her own surgery as well as in specialists' clinics. Many of the affected dogs showed abnormal liver function which resulted in much of the early focus implicating hepatic disorders as the cause of the newly discovered disease. However, after the number of symptomatic dogs with apparently normal livers increased, the concentration shifted to diet in early 1999, as through thorough investigation she found that the symptoms were responding to a nutritional change. It soon became clear that the condition must have a genetic (hereditary) background.

Ms. Plange wrote some articles discussing this disorder which were placed on her web site and published in different European dog magazines, as well as some veterinary magazines.  As a result, there were more responses from Border Terrier owners and some veterinarians who felt their dogs were exhibiting these symptoms, not only from Germany and other parts of Europe, but from all over the world as well.

Samples of liver tissues, blood and urine were sent to several laboratories including the U.S. Thereupon, a world wide cooperation among interested scientist started, and quite a number of people were involved. It was Erica Jabroer-ter Lüün from the Netherlands who stepped in at an early stage of Mrs. Plange`s investigations and in an enormous effort not only built up what can easily be recognized as the best Border Terrier database worldwide, but together with Mrs. Plange, coordinated research at several European universities.

Ms. Plange came to the U.S. Border Terrier list in early 1999 inquiring about the BT's in America and asking if any dogs had unusual epileptic-like symptoms, because a number of suspected carriers were imported from GB to the USA. Ms. Kris Blake contacted Ms. Plange and found that the symptoms of her dog ‘Breaker’ were identical to those of the dogs in Germany and that his pedigree contained dogs who also suffered from the disorder. Then, with Kris Blake struggling with this unusual medical problem in her dog, the problem became recognized in the United States, too.

"Breaker’s" death in early 2002, at the age of seven, brought early attention to the problem in the United States.  Connections were made between Ms. Blake and owners of other cramping dogs.  One of these was an internet support list started by Joke Miedema of the Netherlands and Kris Blake.  The purpose of this list was to provide support for owners of symptomatic dogs and to help determine what dietary changes and medications might help the affected dogs. These had all been collected from the experiences of German owners who had acted upon the advice given to them by Mrs.. Plange. It also became quite apparent by exchanging experiences on this list that dramatic remissions occurred when these dietary changes were made. Joke Miedema named the list “Spike's Disease", after her dog Spike who suffers from this disorder. 

In science it is customary for the person who first described a condition to also give it a name. So in the spring of 2003 Diana Plange decided to give the condition a descriptive name:  Canine Epileptoid Cramping Syndrome.

Dr. Leichty contacted another Border Terrier owner/veterinarian, Dr. Liz Whitney, who directed him to neuromuscular researcher, Dr. Diane Shelton in San Diego, California.  Dr. Shelton of the Comparative Neuromuscular Laboratory at UCSD graciously donated research, which also suggested that the disease was metabolic in nature because of elevated pyruvate and lactate levels in plasma and urine on organic acid screens.  These results were duplicated in urine samples sent to Philadelphia Childrens’ Hospital by Diana Plange and Prof. Urs Giger (Switzerland).

Genetic issues also became more apparent as documented family lines were carefully recorded. Initially pedigree research was coordinated by Erica Jabroer-ter Lüün of the Netherlands and aided by Ms. Blake of the United States. It was discovered that the first identifiable Border Terrier found to be symptomatic dated back to 1974. At this time the mode of inheritance has not been ascertained.  To accelerate the discovery of a gene(s), all owners of CECS dogs and those of often bred unaffected dogs are encouraged to submit blood for DNA testing to the University of Missouri’s Canine Epilepsy Network.  There is no charge for submission beyond postage.

Surveys have been conducted world wide throughout the past two years in the US, Great Britain, and Germany to assess the totality of the problem of cramping/epileptic disorders. It has been discovered that between 5 and 15% of the dogs were affected with cramping/epileptic disorders. In the Netherlands, Professor Jan Rothuizen at the University of Utrecht had done research on the cramping in Border Terriers, and Ms. Jabroer-ter Lüün is currently assisting with the collection of DNA at the University of Ultrecht. 


Mark Leichty DVM, in the United States, has been coordinating the efforts for testing, and assisting owners and veterinarians.  Kris Blake is collecting video and written documentation concerning affected dogs in the United States, creating extensive research pedigree's that will aide in determining the inheritance mode of CECS.






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