Hereditary Defects Advice - Ceroid Lipofuscinosis (C.L.)

C.L. in the Border Collie is a rare disease which affects the nerve cells of the body. It is also known as Storage Disease. The disease also occurs in humans and is called Battens disease. C.L. has also been found in some other breeds of dog. It is an inherited disease; it is not contagious. Affected animals appear normal until approximately 18 months, the disease progresses rapidly once the initial signs appear and distressed animals have not been kept past 2 1/2 years.

The signs of the disease are:

Unreasonable apprehensive or fear of familar objects/surroundings, slight disturbance.
Abnormal gait, unsteady on feet, difficulty jumping or climbing, or placing feet.
Dementend behaviour, manic hyperactivity, rage.

To date there is no known test to identify which dogs have inherited the disease or are carriers of the disease. Cases can be confirmed by a brain biopsy. A post mortem examination will give an accurate diagnosis.

Both dog or bitch are equally susceptible. The Sire and Dam of an affected dog must be either carriers or affected themselves. Mating a carrier animal to a clear (non-carrier) animal can produce carrier offspring.

To control - don't breed from carriers or affected animals. The genetic inheritence of C.L. falls into three categories:

CLEAR has not inherited a defective gene.
CARRIER has inherited the defective gene from a parent.
AFFECTED has inherited the defective gene from both parents and has, or will develop, the disease.

The information, abridged above, is from documents supplied by the BCC of NSW Australia who regularly update and publish a proven carrier list.

DNA reasearch into the disease is currently proceeding in Australia, funded by the Border Collie Clubs of Australia and the Australia government.