NCL and Australian Cattle Dog                                                1. June 2007

I am a regular owner of an Australian Cattle Dog, I do not have any education relating to the subject. I will tell about the disease, as I myself understand it with my common sense. There may be errors in my text. Intensive research is done about this all the time, so there may be new information which I have not received.

What is NCL?

NCL is an abbreviation of the word Neural Ceroid Lipofuscinosis. The Ceroid Lipofuscinosis, CL, is met in many breeds including Border Collie, Cocker Spaniel, English Setter, Saluki, Chihuahua and Dachshund . A gene test has been developed to determine sick dogs and carriers of the disease for at least Border Collies. NCL is met in other species and even in humans. There are many mutations of the NCL, of which many are known.

CL is a rare variation of epilepsy. It is a metabolic disease which particularly affects nerve cells. Sick dogs suffer from enzyme shortage, which causes some of the body's lysosomal storage materials, ceroid and lipofuscin, to collect to cells of the tissue instead of dissolving at the enzyme's impact and being transported out of the system harmlessly. These substances are mainly accumulated in the nervous system, particularly neurons, which causes the cells to eventually explode and expire. The CL is not an infectious disease. Dogs which suffer from this disease are born ill, although they do not yet express symptoms. The effects of the enzyme deficiency may appear only after the dog has grown up.

Is there a cure for NCL?

Unfortunately, there is no cure known for the disease. It always leads to death, although dogs often need to be put to sleep due to severe behavioural problems even before this, often less than a year since the starting of the symptoms.

How common is NCL in cattle dogs?

I do not have confirmed information from other countries, but in Finland there has been only two confirmed cases to the date of this article. Thanks to those owners, who asked an autopsy for their dogs. Now we can fight this disease, because we know of its existence. The Finnish Australian Cattle Dog Club is working with gene researchers in order to develop a gene test for this disease. There are also statistics made on the basis of the two confirmed NCL-cases on possible inheritables, statistics are shown further below.

The beginning of NCL symptoms and signs

Unfortunately there is no one certain symptom which could be an identifier of the NCL. The symptoms can have many variations, depending on the breed, the dog and its original characteristics. Thus, even within the same breed there can be dogs with very different symptoms. The disease is believed to be very painful when progressing, which can be observed as varying, increasingly bad symptoms. The only certain thing is that the disease will get worse, and after the first symptoms are shown, the disease can progress rapidly toward the final destination, death.

Most of the dogs can be symptomless up to the age of 15-24 months, and in some breeds even up to 4-6 years. However, it has been claimed that the dogs which are put to sleep at 5-6 years for severe symptoms have most likely had some symptoms even before the age of 3 years, but they have not been identified until they've got worse.

One of the symptoms can be an increased, periodical restlessness and aimless wandering. As the disease progresses the symptoms get worse and the time between periods shorten. Eventually the dog wanders in a manic manner, being unable to relax. Abnormal fear of touch can be observed, for example putting on the collar can cause avoidance and aggressive protests as the dog fears the pain caused by the procedure. Also combing the dog can become impossible. The dog can have problems moving normally. Sitting normally, jumping or climbing can cause unreasonable problems. The dog might also have compulsive movements. Also symptoms of dementia can be present, and the dog might forget the house training he has already learned. The dog might show blind rage or stare at a certain spot increasingly long periods of time, even hours as the disease progresses. Unreasonable fear reactions can occur, and the dog can become afraid of things which have been safe and familiar to it. The dog can overreact to the owner's commands and gestures and even glances. The dog might have eyesight failures. The dog can become more and more aggressive, eventually biting with blind rage without being unable to control its own behaviour.

Inheritance of the NCL

The NCL is believed to be inherited recessively. It means that both parents need to be at least carriers of the disease, so that some of the puppies will become sick. The fact that a dog is a carrier means that the dog itself has no symptoms, but it carries one of the mutation gene copies which cause the disease. If one of the parents is completely healthy, without having the mutation gene, none of the puppies will become sick. There can be symptomless, healthy carriers of the disease in the litter, if one of the parents is either sick or a carrier of the disease.

Some examples of the inheritance of the CL:

Dog A is healthy. It doesn't posses the gene which causes the disease in its inheritance, so the offsprings can't become sick, even when the other parent is sick or a carrier, ie. possess the sick gene. When combined with dogs B or C, either half or all of the offsprings are healthy carriers of the disease.

Dog B is a healthy carrier of the gene. It will not get sick, but it still carriers one copy of the gene wich causes the disease in its inheritance. If this dog is combined with dog A, the offsprings can not be sick, but about a half of them will be carriers. If the dog is combined with either B or even C, even half of the offsprings can be sick.

Dog C carries two copies of the gene which causes the disease and is thus born sick, although it will only start presenting symptoms approximately at 6 to 18 months. If this dog would be used with dog A, all of the offsprings will be healthy carriers of the disease. If this dog is combined with dog B, half of he offsprings would be healthy carriers and the other half sick dogs presenting symptoms.

What happens when two healthy carriers are combined: Statistically half of the offsprings are healthy carriers, ¼ are healthy and ¼ are sick, as presented in the picture below:

When a healthy carrier is combined with a sick dog, half of the offsprings are healthy carriers and half are born sick, as presented in the picture below:

When a carrier dog is combined with a healthy dog, none of the puppies are sick and half are carriers, as presented in the picture below. This way the carriers do not need to be counted out of breeding, when they are combined with a genetically healthy individual.

A list of the dogs who possibly possess the NCL-gene, based on the 2 dogs which have been diagnosed sick:

Note! Healthy carriers of the disease can be used in breeding as soon as the gene test is developed. Healthy partners can be chosen for the carriers!!

MPBC* calculated relatives, making lines to them (= both parents possess the same
grandparent) should preferably be avoided until we get the gene test for NCL:

NCL-confirmed dog(1):
Cattlefarm's Blue Rock Boots 25,00 % 
Turrella Blue Dynamite 25,00 % 
Turrella Blue Pirate 18,75 % 
Dalotek Blue Dynamite 18,75 % 
Taits Glen Red Sonny 15,82 % 
Reddenblu's Navaho Rug 15,63 % 
Turrella Blue Stetson 12,50 % 
Reddenblu's North of the Law 11,72 % 
Dreamtime Fallen Angel 11,72 % 
Turrella Blue Pebbles 9,38 % 
Turrella Blue Arrow 9,38 % 
Jandanee Blue Mist 9,38 % 

NCL-confirmed dog(2):
Cattlefarm's Blue Rock Boots 37,50 % 
Reddenblu's The Proclaimer 25,00 % 
Reddenblu's Navaho Rug 21,88 % 
Turrella Blue Stetson 18,75 % 
Reddenblu's North of the Law 17,97 % 
Dreamtime Fallen Angel 17,97 % 
Turrella Blue Pirate 15,63 % 
Dalotek Blue Dynamite 15,63 % 
Reddenblu's Angel Luvs Badmen 14,06 % 
Taits Glen Red Sonny 13,18 % 
HGF A-bit-O Blu Jk's Cowchip 12,78 % 
Hanging Grove Farm Kit 9,38 % 

*Major percentage of blood contributors aka percentage of blood. Calculated with DalPedigree program from 10 generations (Willis, 1989).

How do I find out if my dog has NCL?

At the moment it is hard to determine if a living dog has NCL. When the dog already presents symptoms, the disease can be seen in the official eye examination. At this point, the disease can also possibly be noticed in the magnetic resonance imaging. The certain diagnosis can be given only with an autopsy. If the dog's behaviour is abnormal, the owner should contact a vet who is specialised in neurology. If the dog is presenting symptoms of the NCL, the symptoms will certainly get worse over time. If a dog is put to sleep because of suspected NCL symptoms, an autopsy should be done. The autopsy doesn't cost much, but can bring much more information about the causes of the dog's behaviour.

NCL in the future of the ACDs

When the gene test for the NCL in ACDs is developed, the disease will hopefully eventually disappears. The breeders can select the dogs used in breeding so that sick dogs are no longer born. The carriers can be used in breeding to ensure that the gene pool remains broad enough. ACD people all around Finland have been active in gathering blood samples for researchers in order to develop the gene test. Still more blood samples are needed and all dogs who are put to sleep because of suspected NCL symptoms should be done an autopsy. Until the gene test is developed, examining the pedigrees is the best way to breed healthy dogs. In the future there is hope even regarding this fairly rare disease.

Original text by: Anna Elvilä-Moksi
Translated by: Elli Kinnunen, Anna Elvilä-Moksi

The Australian Cattle Dog club of Finland breeding goalprogram 2006(, Vet Pathol 37:488-490 (2000), Hannes Lohi, Academy Research Fellow (