Storage Disease in Border Collies

Ceroid Lipofuscinosis (C.L) is a disease occurring in Border Collies which affects the nerve cells of the body. It is mirrored in children and is known as Batten’s Disease.
It is INHERITED. It is NOT CONTAGIOUS.
The first Australian case was diagnosed in 1980 and there have been in excess of 25 proven cases since that time.
The names of PROVEN carriers and their pedigrees have been published (with permission) to improve knowledge of mode of inheritance and to encourage sensible breeding patterns.
The disease does exist overseas in other breeds of dogs (Salukis, English setters, Cocker Spaniels & Dachsunds), Siamese Cats, Devon Cattle and South Hampshire Sheep. Affected Chihauhuas have been found in Australia.
Affected dogs may display no signs until about 15 - 18 months, all affected animals have died before 2 1/2 years of age.
Carrier dogs are not affected and may be kept as NON-BREEDING companions.

Clinical Signs of C.L (Storage Disease)

Although appearing normal at a young age the affected dog carries the disease from birth. Essentially the disease creates a metabolic defect which allows waste protein (Ceroid lipofuscin) to accumulate (thus, storage) in brain cells. This accumulation begins to depress and destroy healthy brain cells resulting in the following symptoms....

* Unreasonable apprehension of familiar objects and surroundings
* Unsteady gait, difficulty jumping, tends to prop or goose-step
* Demented behaviour - mania, unprecedented hyperactivity and rage

The disease progresses rapidly once initial signs are apparent. A dog showing these signs should be clinically examined. There is no clinical assessment which can categorically determine C.L. (pre - symptomatic) but obviously a dog in distress with these behaviours should be euthanased. Post-mortem examination is the only confirmation available of the disease although the veterinary research being carried out is getting closer all the time.

Veterinary Aspects and Inheritance of C.L.

C.L. is a metabolic storage disease - simply , this means that excess lipid and protein waste is not removed from the body by the action of enzymes. In C.L, although we know that a missing enzyme is the catalyst, the specific enzyme has not yet been pinpointed.

Current research and historical evidence indicates that C.L. has a simple autosomal recessive inheritance pattern. (See diagrams for clear detail) This means that....

* Dog and bitch are equally susceptible
* Sire and dam of affected pups MUST BE either carrier or affected.

Genetic Inheritance of C.L. (Mendel’s mathematical ratios)

Clear Has not inherited the defective gene
Carrier Has inherited the defective gene from ONE parent
Affected Has inherited the defective gene from BOTH parents and will develop the disease

History and Known carriers to date...

As previously mentioned, the disease in our dogs is also a rare disease occurring in children. It is hoped that through joint efforts to discover the exact causes of the disease both canine and human will benefit. The life span of children suffering Batten’s Disease is approximately seven years.

Several cases in 1980 were suspected and then confirmed by the University of Melbourne and the Eye and Ear hospital in Melbourne. Throughout the 1980’s there were several more confirmed cases. Unfortunately, there are still confirmed cases arising occassionally which only confirms the need for careful breeding. Don’t be lulled into a false sense of security, this is not a past health problem; the problem has not disappeared.

Many of the lines behind these affected animals are strikingly familiar and you may draw your own conclusions from this. I will gladly supply any interested people with as much pedigree information as I have. Only confirmed carriers have been published and then only those whose owners have given permission.

Listed below are the known carriers to date, these are animals whose names and pedigrees have been released for the benefit of the Border Collie breeders....... (I have not bothered placing titles next to names as this is irrelevant information)

CARRIER DOGS                                                                                            CARRIER BITCHES
Bundaliere Jazz Amatazz                                                                                  Blackbeck Black Mistic
Byrnbala Rhyl Solette                                                                                     Crestvale Bonnie Lass
Crestvale Gay Roger                                                                                      Crestvale Gay Serena
Finestyle Sundancer                                                                                        Crestvale Natasha
Gotrah Lancelot Lad                                                                                       Finestyle Fancy Free
Kennoway Bill Bailey                                                                                     Jenlehton Kirrie Bay
Margian Skipper Too                                                                                     Kelsey Lady In Blue
Milangimbi Bronzed Gizmo                                                                            Kennoway Our Wee Maggie
Nahrof No Comment                                                                                     Lakemount Autumn Dew
Rosebrook Shar Moss                                                                                   Liric Opal Jayde
Tallain Mengarvi                                                                                             Marglo Blu Rhapsody
Tullaview Sono Spade                                                                                    Monsalvat Amazing Grace
Tullaview Trailblazer                                                                                       Rantara Shantelle Lace
Wallanguma Jock                                                                                           Rosebrook Corindi
Werlak Beau Jade                                                                                         Tallain Tessera
                                                                                                                      Tullacrest Town Tart

PLEASE FEEL FREE TO CONTACT ME REGARDING THIS INFORMATION - PARTICULARLY IF YOU ARE CONSIDERING MATING AND NEED CONFIRMATION ABOUT LINES AND POSSIBILITIES.

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