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
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
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.