Ceroid Lipofuscinosis (CL)
This is a rare disease, which is inherited. It is not
contagious, but it is fatal and cannot be treated. It affects the nervous
system including the brain. The likelihood of a Border Collie developing the
disease is very small as can be seen by the number of cases identified since
1980, when the first case was diagnosed. There have been less than 30 cases
notified, indicating that the incidence of CL is less than 1 in 1800 pure
bred Border Collies.
DNA Research has identified the gene for the identical
disease, which occurs in humans and is known as Battens Disease. When this
gene is identified in Border Collies, we will be able to eliminate the
disease from the breed in one or two generations.
The CL Sub-Committee of The Border Collie Club of NSW Inc investigates the incidence of the disease and sister clubs are informed when affected cases are notified.
The names and pedigrees of proven identified carriers are
periodically published (with permission from the owners of the animals) to
improve the knowledge of inheritance.
CL has been found in other breeds of dogs ie Cocker
Spaniels, Dachschunds, English Setters, Miniature Schnauzers, Rough Collies,
and Salukis as well as in Devon Cattle, South Hampshire Sheep and in Siamese
The occurrence of CL in Border Collies is not the fault of any one person or group. The defective gene was carried by an imported dog at a time when the disease was unidentified. Since then, CL has occurred sporadically, as most Australian bred Border Collies are descended from that dog. Therefore, no breeder can be 100% sure that their stock does not carry the defective gene.
Line breeding increases the risk of producing genetic defects and diseases.
Symptoms and Diagnosis of CL (Storage Disease)
Affected animals appear normal until aged approx 15 months. From that age any or all of the following signs may be noted:
The progress and effect of the symptoms will steadily continue to deteriorate and medication cannot improve the condition. Affected animals have all been euthanased by the age of 3½ years. CL symptoms can be confused with other brain disorders.
To date, there is no test available to identify carriers of the defective gene. Suspected cases can be confirmed by brain biopsy from the age of 7 months. A post mortem will also give an accurate diagnosis.
If you are concerned that your dog may have CL, please contact the CL Sub Committee or Dr Alan Wilton, University of NSW School of Biochemistry.
GENETIC INHERITANCE of CL
Genetically the dog/bitch falls into one of three categories:
Important points to remember are:-
The following diagrams show the mating possibilities using the mathematical ratios of Mendel.
These indicate the probable inheritance of CL for any individual puppy from such matings
What Do We Do About It?
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