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.

Originally thought to only be in Australian Dogs this has been proved false. This may have been due to the simple fact that the disease was identified in Australia. However, since then infected dogs have been identified in the USA, Japan and UK

The signs of the disease are:

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

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. .Further detailed information can normally be found at BCCNSW (At this time the particular pages are being redeveloped).

A DNA Test is now available and further information regarding testing can be found at OptiGen or GTG/GSS while general information regarding test results can be found at Border Collie Health