The Scottish Terrier as a breed is reasonably healthy – it is important to realise this to avoid any undue worry, but every breeder will, sooner or later, be confronted with a problem of an hereditary nature. Scotty Cramp is one of these problems and I hope to give an impartial view, born of personal observation, of this illness in the following article.
What is Scotty Cramp? The experts cannot agree totally but technically it is an interference of the signal sent between the brain and the muscles of the hind quarters; caused either by a shortage (or an overdose) of serotonin, the nerve impulses are not transmitted correctly or are transmitted out of synch, causing the cramping effect of the rear legs (phase 1). In more serious cases, the cramping can extend into the back and topline (phase 2), causing arching similar to a roach back, even to such an extent that the dog loses it’s balance and can fall over (phase 3). Five to ten seconds later, the dog will jump up, shake itself (phase 4) and proceed to walk or run around as if nothing unusual has occurred! SC is in theory an innocent illness – the dog does not die from it, has no pain (as far as is known) and the quality of life of the dog does not have to suffer in any way as a result of the illness. Your Scottie can reach an age of 15 years or more. However, the illness is unwanted in our breed and all steps should be taken to prevent its occurrence. Note that it is not the same as epilepsy, has existed for at least fifty years and has become almost a sort of ‘breed-specific” illness, although it is not limited just to Scotties.
SC is considered to have an autosomal recessive form of inheritance (Willis - 1992, page 162) – this effectively means that the SC gene can be present as a single or even a pair of the dogs’ many normal genes, but that it is not visible or active in its singular form (recessive). Only when BOTH PARENTS carry this recessive SC gene AND both give this gene to their offspring is it possible that SC will become visible (the offspring would then be a sufferer). It is possible that only the sire or only the dam is a carrier of this SC gene, whereby the carrier parent is able only to transmit this defective gene but NOT the illness. In such an instance, the offspring would still run a risk of being a carrier but would not suffer from the illness. The Mendelian laws of inheritance show that, in theory, not all offspring in a litter would be sufferers anyway – 25% could be sufferers, 50% could be carriers and 25% could be clean.
An additional genetic problem makes it nevertheless extremely difficult to predict the behaviour and occurrence of the SC gene – the gene behaves differently in different dogs due to the influence of various factors, whereby the full effect of the SC gene is limited in its expression. Technically this is called ‘incomplete penetration’ and means in practical terms that you have no simple ‘black or white’ situation but many grey situations, whereby every sufferer of SC reacts differently to the presence of the two SC genes, causing various levels of clinical signs.
What causes the onset of SC? There is general consensus that one or more stimuli can trigger this signal-interference in a sufferer. My personal experience is that various types of stimuli such as playing with a ball, running after other dogs or children, fighting, extreme joy at, for example, the return of the owner from work, or even simply mealtime can trigger the attack. Due to the incomplete penetration of the gene into the dogs metabolism, every sufferer will react differently to the same stimuli, since every dog has received a different total genetic combination. I have seen cases of pup-sufferers at 12 weeks old (extremely young) as well as cases of dogs not exhibiting the clinical signs until almost two years of age. Furthermore, I would venture to state that if a dog has not shown SC symptoms by the age of two, then there is a good chance that the dog is not a sufferer; the uncertainty however, still remains. It is not a progressive illness, remaining at the same level during the animal’s whole life span.
A dog can be a sufferer but still not show the clinical signs. He never cramps, never does running somersaults, never falls over, etc. It is possible that the dog suffers from a mild form of the illness, whereby the dogs itself feels an attack coming on and thus decides to stop or avoid the activity (stimulus) that is causing the attack. He may refuse to chase after the ball, for example, or refuse to come when called.
The environment in which a dog lives has its influence as well. There are many Scotties living with older people – perhaps the circumstances never occur under which SC could show itself. The dog lives a very quiet, sedentary life, only making short outings and is not exposed to a high-pressure life. A sufferer Scot with a quiet character would have a relatively smaller chance of showing the clinical signs than a super-hyperactive dog would. A single Scottie sufferer living alone would generally have less chance of showing the illness than in a household where there are two dogs (more stimuli).
I have seen video-images of a young sufferer who was so excited at the return home of his owner or the children that several attacks followed – the dog was unable or unwilling to restrict his own activity, to the extent that this occurred several times a day and ultimately led to the young dog being euthanised at six months. In this situation, the quality of the dog’s life was so bad that this difficult decision was taken out of necessity, but this was indeed the worst case that I have ever seen.
Therefore, you should never breed with sufferers – this sounds like common-sense but as a breeder or owner you need to be able to recognise and diagnose the illness, and that is not as easy as it sounds. If you cannot recognise the illness, how can you know your dog is a sufferer?
It is difficult to help a sufferer – if they do not restrict their own activity, then the stimulus must be removed. Keep the dog calm and remain calm yourself. In America, serious cases have been treated with Vitamin E, diazepam (valium) and Prozac. There is no clear test yet which identifies the dogs as sufferer, carrier or clean. In the past, methysergide has been used to create hyperactivity in the dog and so artificially induce the clinical signs, but this was not 100% effective or accurate. The Dog Genome Project in America is currently working with the STC(America) in an attempt to develop a genetic test, but this takes a lot of time and money and there is no certainty that such a test will indeed be forthcoming in the near future.
Carriers are a totally different problem. You cannot recognise them from internal or external examination. They can only pass on the defective gene. They can be top winning show champions or normal domestic pets. Dog or bitch. Black, brindle or wheaten. English, Dutch, German or American. It doesn’t matter. They can only be identified as a carrier at the moment that their progeny is identified as a sufferer - thereby creating another problem. What do we do with these carriers? Ban them from breeding? Euthanasia? Use them more often? There is no simple answer to this problem. Purists will of course claim that exclusion is the best solution, but in a numerically small breed like the Scotties, you will almost certainly lose many good and desirable properties as well. Kill them? Unnecessary. They can live a completely normal life. Use them more often? Please, please NO! But are they totally useless for breeding purposes? Here lies a major dilemma for both the breeder and/or the stud-dog owner. All too often, the breeders know too little (or absolutely nothing) about the pedigree of their dog – which other, potential carriers there may be in their ancestry, which combinations carry a greater risk, etc. However, I propose that these carriers do not have to be discarded from the breed AS LONG AS a) they are used wisely and in limited amounts b) both breeder and stud-dog owner know of the risks involved and c) the carrier has something special and important to offer the breed.
Does SC occur very often? Apparently not in Holland, because the Breed Health Survey of 1994-1995 (carried out by the STC and the WKHF in 1997) discovered in a representative breed survey of 250 Scotties that only 2% of the replies received showed SC as a health issue. Personally, I find this figure too low. There have been cases in Holland of breeders whose first four litters have produced SC in at least three of these litters, but there are also breeders who have only produced one sufferer in 20 litters. From America, I have heard figures mentioned claiming that 90% of Scotties are carriers, but I think that the real figure is probably more around the 25-30%; the chance that these dogs will become breeding stock is also fairly small, so that a reasoned figure of sufferers in Holland would be more around 5% of the breed population.
There is an important role, no, an obligation for Scottie Breeders to learn more (and want to learn more) about this illness; to be able to deal with in a responsible way, to share information with each other and to aim at reducing the risks involved and the occurrence of this unwanted trait. Puppy buyers must ask the breeder what they know about this problem and how they approach it and attempt to avoid it. There is nothing in any of the old books about this illness, as if by not mentioning it, it ceased to exist. Fortunately, the last fifteen years have brought much more openness in this area – books, articles, surveys and more honesty amongst the breeders. It is no disgrace to admit that you have bred such a Scottie, is it? At least, that was never your intention, was it?
Practical Genetics for Dog Breeders, Malcolm Willis, 1992
Breed Health Survey (Dutch), W.K.Hirschfeld Foundation NL, 1997
Articles Cindy Cooke (Anstamm Kennels USA),
Vandra Huber (McVan Kennels USA)
Carole Fry Owen (The Bagpiper 1997)
Chris & Hanna Hunter
Pointchester Scottish Terriers – Holland
And for the curious amongst you, yes, we have bred a Scotty Cramp sufferer; we are not proud of the fact, it was never our intention but it is a reality of breeding, and we encourage others out there to be more honest about this as well.